PLP1

Summary

Gene Symbol: PLP1
Description: proteolipid protein 1
Alias: GPM6C, HLD1, MMPL, PLP, PLP/DM20, PMD, SPG2, myelin proteolipid protein, lipophilin, major myelin proteolipid protein
Species: human

Top Publications

  1. ncbi Expression of soma-restricted proteolipid/DM20 proteins in lymphoid cells
    Ji Ming Feng
    Developmental Biology Group, Mental Retardation Research Center, Neuropsychiatric Institute, University of California, Los Angeles, Medical School, Room 47 448, 760 Westwood Plaza, Los Angeles, CA 90024, USA
    J Neuroimmunol 144:9-15. 2003
  2. pmc Genomic rearrangements resulting in PLP1 deletion occur by nonhomologous end joining and cause different dysmyelinating phenotypes in males and females
    Ken Inoue
    Department of Molecular and Human Genetics, Baylor College of Medicine, Houston, TX 77030, USA
    Am J Hum Genet 71:838-53. 2002
  3. doi The myelin proteolipid DMα in fishes
    Christian Brösamle
    Institute of Biochemistry, Ludwig Maximilians Universitat Munchen, Germany
    Neuron Glia Biol 6:109-12. 2010
  4. doi PLP1 gene duplication causes overexpression and alteration of the PLP/DM20 splicing balance in fibroblasts from Pelizaeus-Merzbacher disease patients
    Stefano Regis
    Laboratorio Diagnosi Pre Postnatale Malattie Metaboliche, Istituto G Gaslini, Largo G Gaslini 5, 16147 Genova, Italy
    Biochim Biophys Acta 1792:548-54. 2009
  5. doi Genotype-phenotype correlation in five Pelizaeus-Merzbacher disease patients with PLP1 gene duplications
    S Regis
    Diagnosi Pre Postnatale Malattie Metaboliche Laboratory, Children Hospital IRCCS G Gaslini, Genoa, Italy
    Clin Genet 73:279-87. 2008
  6. ncbi The gene encoding for the major brain proteolipid (PLP) maps on the q-22 band of the human X chromosome
    M G Mattei
    Hum Genet 72:352-3. 1986
  7. ncbi A cellular mechanism governing the severity of Pelizaeus-Merzbacher disease
    A Gow
    Brookdale Center for Molecular Biology, Mount Sinai School of Medicine, New York, New York 10029 6574, USA
    Nat Genet 13:422-8. 1996
  8. ncbi A de novo mutation (C755T; Ser252Phe) in exon 6 of the proteolipid protein gene responsible for Pelizaeus-Merzbacher disease
    M E Hodes
    Clin Genet 54:248-9. 1998
  9. pmc Proteolipoprotein gene analysis in 82 patients with sporadic Pelizaeus-Merzbacher Disease: duplications, the major cause of the disease, originate more frequently in male germ cells, but point mutations do not. The Clinical European Network on Brain Dysmy
    C Mimault
    INSERM U 384 Faculté de Médecine, Clermont Ferrand Cedex, France
    Am J Hum Genet 65:360-9. 1999
  10. ncbi Patients lacking the major CNS myelin protein, proteolipid protein 1, develop length-dependent axonal degeneration in the absence of demyelination and inflammation
    James Y Garbern
    Department of Neurology and Center for Molecular Medicine and Genetics, Wayne State University School of Medicine, Detroit, Michigan, USA
    Brain 125:551-61. 2002

Research Grants

  1. Shirley ShiDu Yan; Fiscal Year: 2014
  2. MYELIN PROTEIN GENE EXPRESSION IN DYSMYELINATING MUTANTS
    Wendy B Macklin; Fiscal Year: 2013
  3. Vitamin B6 Effects on One-Carbon Metabolism
    Jesse F Gregory; Fiscal Year: 2013
  4. A Gene therapy Approach for the treatment of EAE
    Yacov Ron; Fiscal Year: 2007
  5. REACTION SPECIFICITY OF PYRIDOXAL PHOSPHATE ENZYMES
    MICHAEL TONEY; Fiscal Year: 2011
  6. REGULATION OF PYRIDOXAL PHOSPHATE BIOSYNTHESIS IN E COLI
    Malcolm Winkler; Fiscal Year: 1999
  7. VITAMIN B6 ADEQUACY, SMOKING AND DNA DAMAGE
    Terry Shultz; Fiscal Year: 2002
  8. PERTURBATIONS IN VITAMIN B6 METABOLISM IN CANCER
    LOUISE NUTTER; Fiscal Year: 2000
  9. VITAMIN B-6 STATUS IN PREGNANCY, LACTATION AND INFANCY
    PHYLIS MOSER VEILLON; Fiscal Year: 1993
  10. Richard B Silverman; Fiscal Year: 2016

Detail Information

Publications234 found, 100 shown here

  1. ncbi Expression of soma-restricted proteolipid/DM20 proteins in lymphoid cells
    Ji Ming Feng
    Developmental Biology Group, Mental Retardation Research Center, Neuropsychiatric Institute, University of California, Los Angeles, Medical School, Room 47 448, 760 Westwood Plaza, Los Angeles, CA 90024, USA
    J Neuroimmunol 144:9-15. 2003
    A new family of the myelin proteolipid protein (PLP/DM20) gene products, srPLP/DM20, has been identified recently in thymus and brain...
  2. pmc Genomic rearrangements resulting in PLP1 deletion occur by nonhomologous end joining and cause different dysmyelinating phenotypes in males and females
    Ken Inoue
    Department of Molecular and Human Genetics, Baylor College of Medicine, Houston, TX 77030, USA
    Am J Hum Genet 71:838-53. 2002
    In the majority of patients with Pelizaeus-Merzbacher disease, duplication of the proteolipid protein gene PLP1 is responsible, whereas deletion of PLP1 is infrequent...
  3. doi The myelin proteolipid DMα in fishes
    Christian Brösamle
    Institute of Biochemistry, Ludwig Maximilians Universitat Munchen, Germany
    Neuron Glia Biol 6:109-12. 2010
    ..apposition by three structural proteins of myelin, myelin basic protein, myelin protein zero (MPZ) and myelin proteolipid protein (PLP1/DMalpha)...
  4. doi PLP1 gene duplication causes overexpression and alteration of the PLP/DM20 splicing balance in fibroblasts from Pelizaeus-Merzbacher disease patients
    Stefano Regis
    Laboratorio Diagnosi Pre Postnatale Malattie Metaboliche, Istituto G Gaslini, Largo G Gaslini 5, 16147 Genova, Italy
    Biochim Biophys Acta 1792:548-54. 2009
    ..Defects of the PLP1 gene cause Pelizaeus-Merzbacher disease (PMD) or X-linked spastic paraplegia type 2 (SPG2). Duplication of the PLP1 gene is the most frequent gene defect, usually responsible for the classic form of PMD...
  5. doi Genotype-phenotype correlation in five Pelizaeus-Merzbacher disease patients with PLP1 gene duplications
    S Regis
    Diagnosi Pre Postnatale Malattie Metaboliche Laboratory, Children Hospital IRCCS G Gaslini, Genoa, Italy
    Clin Genet 73:279-87. 2008
    ..myelination disorder most frequently caused by duplication of a genomic segment of variable length containing the PLP1 gene. We studied five PMD male patients affected by the classic PMD form carrying a PLP1 gene duplication...
  6. ncbi The gene encoding for the major brain proteolipid (PLP) maps on the q-22 band of the human X chromosome
    M G Mattei
    Hum Genet 72:352-3. 1986
    Recombinant plasmid clone p23 containing the cDNA proteolipid (PLP) sequence was localized by in situ hybridization on band q22 of the human X chromosome...
  7. ncbi A cellular mechanism governing the severity of Pelizaeus-Merzbacher disease
    A Gow
    Brookdale Center for Molecular Biology, Mount Sinai School of Medicine, New York, New York 10029 6574, USA
    Nat Genet 13:422-8. 1996
    Pelizaeus-Merzbacher disease (PMD) is a leukodystrophy linked to the proteolipid protein gene (PLP)...
  8. ncbi A de novo mutation (C755T; Ser252Phe) in exon 6 of the proteolipid protein gene responsible for Pelizaeus-Merzbacher disease
    M E Hodes
    Clin Genet 54:248-9. 1998
  9. pmc Proteolipoprotein gene analysis in 82 patients with sporadic Pelizaeus-Merzbacher Disease: duplications, the major cause of the disease, originate more frequently in male germ cells, but point mutations do not. The Clinical European Network on Brain Dysmy
    C Mimault
    INSERM U 384 Faculté de Médecine, Clermont Ferrand Cedex, France
    Am J Hum Genet 65:360-9. 1999
    Pelizaeus-Merzbacher Disease (PMD) is an X-linked developmental defect of myelination affecting the central nervous system and segregating with the proteolipoprotein (PLP) locus...
  10. ncbi Patients lacking the major CNS myelin protein, proteolipid protein 1, develop length-dependent axonal degeneration in the absence of demyelination and inflammation
    James Y Garbern
    Department of Neurology and Center for Molecular Medicine and Genetics, Wayne State University School of Medicine, Detroit, Michigan, USA
    Brain 125:551-61. 2002
    ..of rodents and humans with a null mutation in the gene encoding the major CNS myelin protein, proteolipid protein (PLP1, previously PLP)...
  11. ncbi A PLP splicing abnormality is associated with an unusual presentation of PMD
    Grace M Hobson
    Department of Research, Alfred I duPont Hospital for Children, Wilmington, DE, USA
    Ann Neurol 52:477-88. 2002
    We report that a deletion of 19 base pairs (bp) in intron 3 of the proteolipid protein (PLP/DM20) gene causes a neurological disease characterized by mild developmental delay, followed by progressive decline of acquired motor and ..
  12. pmc Schwann cell expression of PLP1 but not DM20 is necessary to prevent neuropathy
    Michael E Shy
    Department of Neurology and Center for Molecular Medicine and Genetics, Wayne State University School of Medicine, Detroit, MI 48201, USA
    Ann Neurol 53:354-65. 2003
    Proteolipid protein (PLP1) and its alternatively spliced isoform, DM20, are the major myelin proteins in the CNS, but are also expressed in the PNS...
  13. ncbi PLP1-related inherited dysmyelinating disorders: Pelizaeus-Merzbacher disease and spastic paraplegia type 2
    Ken Inoue
    Department of Mental Retardation and Birth Defect Research, National Institute of Neuroscience, National Center of Neurology and Psychiatry NCNP, 4 1 1 Ogawahigashi, Kodaira, Tokyo 187 8502, Japan
    Neurogenetics 6:1-16. 2005
    ..Both PMD and SPG2 are caused by mutations in the proteolipid protein 1 (PLP1) gene, which encodes a major component of CNS myelin proteins...
  14. ncbi Spastic paraplegia type 2 associated with axonal neuropathy and apparent PLP1 position effect
    Jennifer A Lee
    Department of Molecular and Human Genetics, Baylor College of Medicine, Houston, TX, USA
    Ann Neurol 59:398-403. 2006
    To report an association between spastic paraplegia type 2 with axonal peripheral neuropathy and apparent proteolipid protein gene (PLP1) silencing in a family.
  15. pmc Heterogeneous duplications in patients with Pelizaeus-Merzbacher disease suggest a mechanism of coupled homologous and nonhomologous recombination
    Karen J Woodward
    Clinical and Molecular Genetics, Institute of Child Health, London
    Am J Hum Genet 77:966-87. 2005
    ..We describe genomic structures of 59 X-chromosome segmental duplications that include the proteolipid protein 1 gene (PLP1) in patients with Pelizaeus-Merzbacher disease...
  16. ncbi Role of genomic architecture in PLP1 duplication causing Pelizaeus-Merzbacher disease
    Jennifer A Lee
    Department of Molecular and Human Genetics, Baylor College of Medicine, One Baylor Plaza, Room 604B, Houston, TX 77030, USA
    Hum Mol Genet 15:2250-65. 2006
    ..genomic disorder most commonly arising from genomic duplications of the dosage-sensitive proteolipid protein gene (PLP1)...
  17. pmc Complete deletion of the proteolipid protein gene (PLP) in a family with X-linked Pelizaeus-Merzbacher disease
    W H Raskind
    Department of Medicine, University of Washington School of Medicine, Seattle 98195
    Am J Hum Genet 49:1355-60. 1991
    ..Proteolipid protein (PLP), a major structural protein of myelin, is coded on the X chromosome...
  18. pmc Individual exons encode the integral membrane domains of human myelin proteolipid protein
    H J Diehl
    Proc Natl Acad Sci U S A 83:9807-11. 1986
    The gene encoding human proteolipid protein (PLP) was isolated from a human genomic library by hybridization with labeled DNA of a PLP-specific cDNA clone. The entire PLP gene spans approximately 17 kilobases...
  19. ncbi Amino-acid sequence of human and bovine brain myelin proteolipid protein (lipophilin) is completely conserved
    W Stoffel
    Biol Chem Hoppe Seyler 366:627-35. 1985
    Proteolipid protein (PLP) was isolated from white matter of human brain by chloroform/methanol extraction and further purified by chromatography...
  20. ncbi X-linked spastic paraplegia and Pelizaeus-Merzbacher disease are allelic disorders at the proteolipid protein locus
    P Saugier-Veber
    Service de Genetique, Unite de Recherches sur les Handicaps Genetiques de l Enfant, INSERM U 393 Paris, France
    Nat Genet 6:257-62. 1994
    ..One locus (SPG 1) maps to Xq28 while two clinically distinct forms map to Xq22 (SPG2)...
  21. pmc A duplicated PLP gene causing Pelizaeus-Merzbacher disease detected by comparative multiplex PCR
    K Inoue
    Department of Psychiatry, Yokohama City University, School of Medicine, Yokohama
    Am J Hum Genet 59:32-9. 1996
    ..disease (PMD) is an X-linked dysmyelinating disorder caused by abnormalities in the proteolipid protein (PLP) gene, which is essential for oligodendrocyte differentiation and CNS myelin formation...
  22. ncbi Duplication of the proteolipid protein gene is the major cause of Pelizaeus-Merzbacher disease
    E A Sistermans
    Department of Human Genetics, University Hospital Nijmegen, The Netherlands
    Neurology 50:1749-54. 1998
    Pelizaeus-Merzbacher disease (PMD), an X-linked recessive dysmyelination disorder, is caused by mutations in the proteolipid protein (PLP) gene...
  23. pmc Pelizaeus-Merzbacher disease: identification of Xq22 proteolipid-protein duplications and characterization of breakpoints by interphase FISH
    K Woodward
    Molecular Genetics Unit, Institute of Child Health, Guy s Hosptial, London, United Kingdom
    Am J Hum Genet 63:207-17. 1998
    Pelizaeus-Merzbacher disease (PMD) is an X-linked, dysmyelinating disorder of the CNS...
  24. ncbi Novel exon 3B proteolipid protein gene mutation causing late-onset spastic paraplegia type 2 with variable penetrance in female family members
    K Sivakumar
    Medical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD 20892 1361, USA
    Ann Neurol 45:680-3. 1999
    Spastic paraplegia type 2 (SPG2) is allelic to Pelizaeus-Merzbacher disease (PMD), with both conditions resulting from mutations in the proteolipid protein gene (PLP)...
  25. ncbi The molecular pathogenesis of Pelizaeus-Merzbacher disease
    J Garbern
    Department of Neurology, Center for Molecular Medicine and Genetics, Wayne State University School of Medicine, Detroit, Mich, USA
    Arch Neurol 56:1210-4. 1999
    ..description of this family provides the clinical, genetic, and pathological basis for Pelizaeus-Merzbacher disease (PMD): an X-linked disorder of myelination classically characterized by nystagmus, spastic quadriparesis, ataxia, and ..
  26. ncbi X inactivation phenotype in carriers of Pelizaeus-Merzbacher disease: skewed in carriers of a duplication and random in carriers of point mutations
    K Woodward
    Molecular Genetics Unit, Institute of Child Health, London, UK
    Eur J Hum Genet 8:449-54. 2000
    Pelizaeus-Merzbacher disease (PMD) is an X-linked recessive disease caused by coding sequence mutations in the PLP gene, sub-microscopic duplications of variable sizes including the PLP gene or very rarely deletions of the PLP gene...
  27. ncbi Genotype-phenotype correlation in inherited brain myelination defects due to proteolipid protein gene mutations. Clinical European Network on Brain Dysmyelinating Disease
    F Cailloux
    INSERM U384, Faculte de Medecine, Clermont Ferrand, France
    Eur J Hum Genet 8:837-45. 2000
    Pelizaeus-Merzbacher disease (PMD) and spastic paraplegia type 2 (SPG2) are X-linked developmental defects of myelin formation affecting the central nervous system (CNS)...
  28. ncbi Myelin proteolipid protein forms a complex with integrins and may participate in integrin receptor signaling in oligodendrocytes
    Tatyana I Gudz
    Department of Neurosciences, Lerner Research Institute, Cleveland Clinic Foundation, Cleveland, Ohio 44195, USA
    J Neurosci 22:7398-407. 2002
    ..A new role for the myelin proteolipid protein (PLP), the most abundant protein of CNS myelin, has been identified, in studies showing PLP interaction ..
  29. pmc Role of calnexin in the glycan-independent quality control of proteolipid protein
    Eileithyia Swanton
    School of Biological Sciences, University of Manchester, 2 205 Stopford Building, Oxford Road, Manchester M13 9PT, UK
    EMBO J 22:2948-58. 2003
    ..We have used a folding mutant of a non- glycosylated membrane protein, proteolipid protein (PLP), to examine the quality control of this class of polypeptide...
  30. ncbi Splice-site contribution in alternative splicing of PLP1 and DM20: molecular studies in oligodendrocytes
    Grace M Hobson
    Nemours Biomedical Research, Alfred I duPont Hospital for Children, Nemours Children s Clinic, Wilmington, Delaware, USA
    Hum Mutat 27:69-77. 2006
    ..PLP1) gene cause the X-linked dysmyelinating diseases Pelizaeus-Merzbacher disease (PMD) and spastic paraplegia 2 (SPG2)...
  31. ncbi PLP1 and GPM6B intragenic copy number analysis by MAPH in 262 patients with hypomyelinating leukodystrophies: Identification of one partial triplication and two partial deletions of PLP1
    Patricia Combes
    INSERM U 384, Faculte de Medecine, Place Henri Dunant, 63000 Clermont Ferrand, France
    Neurogenetics 7:31-7. 2006
    The proteolipid protein 1 (PLP1) gene is known to be mutated in the X-linked disorders of myelin formation Pelizaeus-Merzbacher disease (PMD) and spastic paraplegia type 2...
  32. pmc Novel alternatively spliced endoplasmic reticulum retention signal in the cytoplasmic loop of Proteolipid Protein-1
    Cherie Southwood
    Center for Molecular Medicine and Genetics, Wayne State University School of Medicine, Detroit, Michigan 48201, USA
    J Neurosci Res 85:471-8. 2007
    ..aspects of ER retention in transfected fibroblasts expressing missense mutations in the Proteolipid Protein-1 (PLP1) gene that cause mild or severe forms of neurodegenerative disease in humans...
  33. ncbi Steroid-responsive neurologic relapses in a child with a proteolipid protein-1 mutation
    M P Gorman
    Department of Neurology, Children s Hospital, Boston, MA 02115, USA
    Neurology 68:1305-7. 2007
    ..Although the patient met criteria for multiple sclerosis (MS), the proteolipid protein-1 gene (PLP1) contained a mutation in exon 3B (c.409C>T), predicting a tryptophan-for-arginine substitution...
  34. pmc A common mechanism of PLP/DM20 misfolding causes cysteine-mediated endoplasmic reticulum retention in oligodendrocytes and Pelizaeus-Merzbacher disease
    Ajit Singh Dhaunchak
    Department of Neurogenetics, Max Planck Institute of Experimental Medicine, D 37075 Gottingen, Germany
    Proc Natl Acad Sci U S A 104:17813-8. 2007
    A large number of mutations in the human PLP1 gene lead to abnormal myelination and oligodendrocyte death in Pelizaeus-Merzbacher disease (PMD)...
  35. pmc Molecular genetic analysis of the PLP1 gene in 38 families with PLP1-related disorders: identification and functional characterization of 11 novel PLP1 mutations
    Serena Grossi
    SSD Lab, Diagnosi Pre Postnatale Malattie Metaboliche, IRCCS G, Gaslini, Genova, Italy
    Orphanet J Rare Dis 6:40. 2011
    ..paraplegia type 2 is due to the extensive allelic heterogeneity in the X-linked PLP1 gene encoding myelin proteolipid protein (PLP)...
  36. ncbi A case of complicated spastic paraplegia 2 due to a point mutation in the proteolipid protein 1 gene
    Eun Sil Lee
    Department of Pediatrics, Yeungnam University Hospital, Daegu, South Korea
    J Neurol Sci 224:83-7. 2004
    ..In addition, spastic paraplegia 2 (SPG2) is allelic to PMD and typically caused by missense mutations in the second extracellular domain of PLP1 or in the ..
  37. ncbi [Connatal type of Pelizaeus-Merzbacher disease: a case report]
    K Kagitani
    Department of Pediatrics, Osaka University
    No To Hattatsu 31:171-6. 1999
    Pelizaeus-Merzbacher disease (PMD) is a hereditary disorder with myelin dysplasia in the central nervous system...
  38. ncbi [Clinical symptoms and characteristic MR spectroscopic findings in Pelizaeus-Merzbacher disease]
    K Kurata
    Department of Neuropediatrics, Fuchu Metropolitan Medical Center of Severe Motor and Intellectual Disabilities, Fuchu, Tokyo
    No To Hattatsu 32:503-8. 2000
    ..Genetical diagnosis revealed in 2 cases, duplication of proteolipid protein (PLP) and deletion in 1, whereas one case had no abnormality of PLP gene...
  39. ncbi [Serial MR imaging of possible Pelizaeus-Merzbacher disease]
    M Ishii
    Department of Pediatrics, Chiba University School of Medicine
    No To Hattatsu 25:9-15. 1993
    ..serial magnetic resonance imaging (MRI) toward 4 male children with possible Pelizaeus-Merzbacher disease (PMD)...
  40. ncbi Identification of a new exon in the myelin proteolipid protein gene encoding novel protein isoforms that are restricted to the somata of oligodendrocytes and neurons
    E R Bongarzone
    Neuropsychiatric Institute, University of California, Los Angeles, Medical School, Los Angeles, California 90024 1759, USA
    J Neurosci 19:8349-57. 1999
    The myelin proteolipid protein (PLP) gene (i.e., the PLP/DM20 gene) has been of some interest because of its role in certain human demyelinating diseases, such as Pelizaeus-Merzbacher disease...
  41. pmc Overexpression of the myelin proteolipid protein leads to accumulation of cholesterol and proteolipid protein in endosomes/lysosomes: implications for Pelizaeus-Merzbacher disease
    Mikael Simons
    Department of Neurology, University of Tubingen, 72076 Tubingen, Germany
    J Cell Biol 157:327-36. 2002
    ..of the proteolipid protein (PLP) gene are known to cause the dysmyelinating disorder Pelizaeus-Merzbacher disease (PMD)...
  42. ncbi Myelin proteolipid protein (Plp) intron 1 DNA is required to temporally regulate Plp gene expression in the brain
    Shenyang Li
    Department of Physiology and Biophysics, University of Arkansas for Medical Sciences, 4301 W Markham Street, Little Rock, AR 72205, USA
    J Neurochem 83:193-201. 2002
    The myelin proteolipid protein (Plp) gene encodes the most abundant protein found in mature CNS myelin...
  43. ncbi Glial cell degeneration and hypomyelination caused by overexpression of myelin proteolipid protein gene
    T Kagawa
    Laboratory of Neural Information, Okazaki National Research Institutes, Aichi, Japan
    Neuron 13:427-42. 1994
    b>Myelin proteolipid protein (PLP), the major myelin protein in the CNS, has been thought to function in myelin assembly...
  44. ncbi Processing of PLP in a model of Pelizaeus-Merzbacher disease/SPG2 due to the rumpshaker mutation
    Mark McLaughlin
    Applied Neurobiology Group, Institute of Comparative Medicine, University of Glasgow, Bearsden Glasgow G61 1QH, Scotland
    Glia 53:715-22. 2006
    The rumpshaker mutation of the X-linked myelin proteolipid protein (PLP1) gene causes spastic paraplegia type 2 or a mild form of Pelizaeus-Merzbacher disease in man. The identical mutation occurs spontaneously in mice...
  45. doi Modulation of the oligomerization of myelin proteolipid protein by transmembrane helix interaction motifs
    Derek P Ng
    Division of Molecular Structure and Function, Research Institute, Hospital for Sick Children, Toronto, Ontario, Canada
    Biochemistry 49:6896-902. 2010
    Proteolipid protein (PLP) is a highly hydrophobic 276-residue integral membrane protein that constitutes more than 50% of the total protein in central nervous system myelin...
  46. ncbi Immunolocalization of the electrogenic Na+-HCO-3 cotransporter in mammalian and amphibian kidney
    B M Schmitt
    Department of Cellular and Molecular Physiology, Section of Nephrology, Yale University School of Medicine, New Haven, Connecticut 06520, USA
    Am J Physiol 276:F27-38. 1999
    ..5-micrometer cryosections of rat and rabbit kidneys fixed in paraformaldehyde-lysine-periodate (PLP), the pAbs produced a strong and exclusively basolateral staining of the PT...
  47. ncbi Differential sensitivity in the survival of oligodendrocyte cell lines to overexpression of myelin proteolipid protein gene products
    E R Bongarzone
    Mental Retardation Research Center, School of Medicine, University of California, Los Angeles, California 90095, USA
    J Neurosci Res 65:485-92. 2001
    The proteolipid (PLP) gene encodes at least four proteins, including the classic PLP and DM20, which are important components of the myelin sheath, and the recently identified soma-restricted (sr) isoforms, srPLP and srDM20...
  48. ncbi A combination of PLP and DM20 transgenes promotes partial myelination in the jimpy mouse
    N L Nadon
    Biology Department, University of Tulsa, OK 74104 3189
    J Neurochem 63:822-33. 1994
    Mutations in the myelin proteolipid protein (PLP) gene, such as that found in the jimpy mouse, result in an abnormal structure of the myelin, severe dysmyelination, and a reduction in the number of mature oligodendrocytes...
  49. ncbi Perturbed interactions of mutant proteolipid protein/DM20 with cholesterol and lipid rafts in oligodendroglia: implications for dysmyelination in spastic paraplegia
    Eva Maria Krämer-Albers
    Department of Biology, Unit of Molecular Cell Biology, University of Mainz, 55099 Mainz, Germany
    J Neurosci 26:11743-52. 2006
    Missense mutations in the human PLP1 gene lead to dysmyelinating diseases with a broad range of clinical severity, ranging from severe Pelizaeus-Merzbacher disease (PMD) to milder spastic paraplegia type 2 (SPG-2)...
  50. pmc Cisplatin-induced toxicity is associated with platinum deposition in mouse kidney mitochondria in vivo and with selective inactivation of the alpha-ketoglutarate dehydrogenase complex in LLC-PK1 cells
    Lei Zhang
    Department of Cell Biology, Biomedical Research Center, Room 264, 975 N E 10th Street, University of Oklahoma Health Sciences Center, Oklahoma City, Oklahoma 73104, USA
    Biochemistry 45:8959-71. 2006
    ..proposed bioactivation is the formation of a platinum-cysteine S-conjugate followed by a pyridoxal 5'-phosphate (PLP)-dependent cysteine S-conjugate beta-lyase reaction...
  51. ncbi Myelin proteolipid protein (PLP) as a marker antigen of central nervous system contaminations for routine food control
    Carmen Villmann
    Institut fur Biochemie, Emil Fischer Zentrum, Friedrich Alexander Universitat Erlangen Nurnberg, Fahrstrasse 17, 91054 Erlangen, Germany
    J Agric Food Chem 55:7114-23. 2007
    ..Here, myelin proteolipid protein (PLP) is identified as a specific and largely heat-resistant marker for detection of food contaminations ..
  52. pmc Leydig cells express the myelin proteolipid protein gene and incorporate a new alternatively spliced exon
    Shenyang Li
    Department of Physiology and Biophysics, University of Arkansas for Medical Sciences, Little Rock, AR 72205, USA
    Gene 436:30-6. 2009
    Although the myelin proteolipid protein gene (Plp1) is highly expressed in the central nervous system encoding the most abundant myelin protein in oligodendrocytes, it is also expressed in other tissues, including testis...
  53. ncbi Analysis of myelin proteolipid protein and F0 ATPase subunit 9 in normal and jimpy CNS
    J A Benjamins
    Department of Neurology, Wayne State University School of Medicine, Detroit, Michigan 48201
    Neurochem Res 19:1013-22. 1994
    ..jp) and normal CNS at 17-20 days were examined by immunoblot and sequence analysis to determine whether myelin proteolipid protein (FLP) or DM-20 could be detected in jp CNS...
  54. doi Transport of the major myelin proteolipid protein is directed by VAMP3 and VAMP7
    Anke Feldmann
    Department of Biology, Molecular Cell Biology, University of Mainz, D 55128 Mainz, Germany
    J Neurosci 31:5659-72. 2011
    ..VAMP3 and VAMP7 colocalize with the major myelin proteolipid protein (PLP) in recycling endosomes and late endosomes/lysosomes, respectively...
  55. ncbi Developmental expression of myelin protein genes in dysmyelinating mutant mice: analysis by nuclear run-off transcription assay, in situ hybridization, and immunohistochemistry
    C Shiota
    Division of Regulation of Macromolecular Function, Osaka University, Japan
    J Neurochem 56:818-26. 1991
    Gene expression for myelin proteolipid protein (PLP) and myelin basic protein (MBP) in the dysmyelinating mutant mice shiverer and jimpy was analyzed by nuclear run-off transcription assay, in situ hybridization, and immunohistochemistry...
  56. doi Modulation of rumpshaker phenotype with wild-type PLP/DM20 suggests several pathogenic mechanisms
    Jennifer A Barrie
    Applied Neurobiology Group, Institute of Comparative Medicine, University of Glasgow, Bearsden, Glasgow, Scotland
    J Neurosci Res 88:2135-45. 2010
    The rumpshaker mutation of the murine myelin proteolipid protein 1 (Plp1) gene generates misfolded PLP/DM20 protein, resulting in dysmyelination, increased oligodendrocyte apoptosis, and death prior to P40 when expressed on the C57 BL/6 ..
  57. ncbi Enhancement of EAE and induction of autoantibodies to T-cell epitopes in mice infected with a recombinant vaccinia virus encoding myelin proteolipid protein
    L Y Wang
    Department of Neurology, University of Utah School of Medicine, Salt Lake City 84132, USA
    J Neuroimmunol 75:75-83. 1997
    SJL/J mice were infected with a recombinant vaccinia virus encoding myelin proteolipid protein (PLP) (VVplp)...
  58. ncbi Myelin proteolipid protein gene structure and its regulation of expression in normal and jimpy mutant mice
    K Ikenaka
    Institute for Protein Research, Osaka University, Japan
    J Mol Biol 199:587-96. 1988
    The mouse proteolipid protein (PLP) gene was cloned into the lambda bacteriophage Charon 4A...
  59. ncbi Many naturally occurring mutations of myelin proteolipid protein impair its intracellular transport
    A Gow
    Brookdale Center for Molecular Biology, Mount Sinai School of Medicine, New York, New York 10029 6574
    J Neurosci Res 37:574-83. 1994
    The primary structure of the proteolipid protein (PLP) from the central nervous system (CNS) myelin of mammals is highly conserved with only three amino acid differences between the mouse, rat, dog, bovine, and human proteins...
  60. ncbi Expression of members of the proteolipid protein gene family in the developing murine central nervous system
    Y Yan
    Department of Neurobiology, University of Pittsburgh School of Medicine, Pennsylvania 15261, USA
    J Comp Neurol 370:465-78. 1996
    ..Both cDNAs, M6a and M6b, bore significant homology with the major myelin proteolipid protein, PLP/DM20...
  61. ncbi Myelin proteolipid protein (PLP), but not DM-20, is an inositol hexakisphosphate-binding protein
    Y Yamaguchi
    Laboratory of Neural Information, National Institute for Physiological Sciences, Okazaki National Research Institutes, Okazaki, Aichi 444, Japan
    J Biol Chem 271:27838-46. 1996
    b>Myelin proteolipid protein (PLP) and its alternatively spliced isoform, DM-20, are the major integral membrane proteins of central nervous system myelin...
  62. ncbi The pathobiology of myelin mutants reveal novel biological functions of the MBP and PLP genes
    A T Campagnoni
    Neuropsychiatric Institute, UCLA School of Medicine, 90024, USA
    Brain Pathol 11:74-91. 2001
    Substantial biological data indicate that the myelin basic protein (MBP) and myelin proteolipid protein (PLP/DM20) genes produce products with functions beyond that of serving as myelin structural proteins...
  63. ncbi Thiopalmitoylation of myelin proteolipid protein epitopes enhances immunogenicity and encephalitogenicity
    J M Greer
    Neuroimmunology Research Unit, Department of Medicine, University of Queensland, Royal Brisbane Hospital, Herston QLD 4029, Australia
    J Immunol 166:6907-13. 2001
    Proteolipid protein (PLP) is the most abundant protein of CNS myelin, and is posttranslationally acylated by covalent attachment of long chain fatty acids to cysteine residues via a thioester linkage...
  64. ncbi Alpha-amino-3-hydroxy-5-methylisoxazole-4-propionic acid-mediated excitotoxic axonal damage is attenuated in the absence of myelin proteolipid protein
    J H Fowler
    Division of Clinical Neuroscience, University of Glasgow, Wellcome Surgical Institute, Glasgow G61 1QH, United Kingdom
    J Neurosci Res 84:68-77. 2006
    ..Proteolipid protein (PLP) and DM20 are integral membrane proteins expressed by CNS oligodendrocytes and located in compact myelin...
  65. pmc Expression of a myelin proteolipid protein (Plp)-lacZ transgene is reduced in both the CNS and PNS of Plp(jp) mice
    Patricia A Wight
    Department of Physiology and Biophysics, University of Arkansas for Medical Sciences, Little Rock, AR, 72205, USA
    Neurochem Res 32:343-51. 2007
    ..It results from a point mutation in the acceptor splice site of myelin proteolipid protein (Plp) exon 5, producing transcripts that are missing exon 5, with a concomitant shift in the downstream ..
  66. ncbi DM20 mRNA splice product of the myelin proteolipid protein gene is expressed in the murine heart
    C W Campagnoni
    Mental Retardation Research Center, U C L A Medical School
    J Neurosci Res 33:148-55. 1992
    ..3 and 2.4 kb mRNAs that hybridized with a cDNA for the mouse proteolipid protein (PLP)...
  67. ncbi Selective expression of DM-20, an alternatively spliced myelin proteolipid protein gene product, in developing nervous system and in nonglial cells
    K Ikenaka
    Division of Regulation of Macromolecular Function, Osaka University, Japan
    J Neurochem 58:2248-53. 1992
    Mutations within the gene for myelin proteolipid protein (PLP), a major myelin structural protein, result in abnormal glial differentiation, suggesting that the PLP gene products play some other functional roles...
  68. ncbi Recovery of proteolipid protein in mice heterozygous for the jimpy gene
    J A Benjamins
    Department of Neurology, Wayne State University School of Medicine, Detroit, MI 48201
    J Neurochem 53:279-86. 1989
    We have measured levels and synthesis of proteolipid protein (PLP) and its transport into myelin in female mice heterozygous for the jimpy gene and in their normal female littermates...
  69. ncbi Encephalitogenicity of murine, but not bovine, DM20 in SJL mice is due to a single amino acid difference in the immunodominant encephalitogenic epitope
    J M Greer
    Department of Medicine, University of Queensland, Brisbane, Australia
    Neurochem Res 22:541-7. 1997
    b>Myelin proteolipid protein (PLP) contains 2 immunodominant encephalitogenic epitopes in SJL mice, namely PLP residues 139-151 and 178-191...
  70. pmc Antigen-specific suppression of experimental autoimmune encephalomyelitis by a novel bifunctional peptide inhibitor: structure optimization and pharmacokinetics
    Rahmawati Ridwan
    Department of Pharmaceutical Chemistry, University of Kansas, Simons Research Laboratories, 2095 Constant Avenue, Lawrence, KS 66047, USA
    J Pharmacol Exp Ther 332:1136-45. 2010
    The objective of this study was to optimize the in vivo activity of proteolipid protein (PLP)-bifunctional peptide inhibitor (BPI) molecule to suppress experimental autoimmune encephalomyelitis (EAE) in SJL/J mice and evaluate ..
  71. pmc The proteolipid protein promoter drives expression outside of the oligodendrocyte lineage during embryonic and early postnatal development
    John Paul Michalski
    Ottawa Hospital Research Institute, Ottawa, Ontario, Canada
    PLoS ONE 6:e19772. 2011
    The proteolipid protein (Plp) gene promoter is responsible for driving expression of one of the major components of myelin--PLP and its splice variant DM-20...
  72. ncbi Modification of aminoacyl-tRNA synthetases with pyridoxal-5'-phosphate. Identification of the labeled amino acid residues
    T Kalogerakos
    Laboratoire de Biochemie, CNRS URA 240, Ecole Polytechnique, Palaiseau, France
    Biochimie 76:33-44. 1994
    ..synthetases from Escherichia coli are lost upon incubation in the presence of pyridoxal-5'-phosphate (PLP)...
  73. ncbi Phenotypic severity of murine Plp mutants reflects in vivo and in vitro variations in transport of PLP isoproteins
    C E Thomson
    Department of Veterinary Clinical Studies, University of Glasgow, Bearsden, Scotland
    Glia 20:322-32. 1997
    Mutations of the major myelin gene, proteolipid protein (Plp), cause Pelizaeus-Merzbacher disease and some forms of spastic paraplegia in man and dysmyelinating phenotypes in animals...
  74. ncbi PLP overexpression perturbs myelin protein composition and myelination in a mouse model of Pelizaeus-Merzbacher disease
    Saadia A Karim
    Applied Neurobiology Group, Institute of Comparative Medicine, University of Glasgow, Bearsden, Glasgow, Scotland
    Glia 55:341-51. 2007
    Duplication of PLP1, an X-linked gene encoding the major myelin membrane protein of the human CNS, is the most frequent cause of Pelizaeus-Merzbacher disease (PMD)...
  75. ncbi A severe connatal form of Pelizaeus Merzbacher disease in a Czech boy caused by a novel mutation (725C>A, Ala242Glu) at the 'jimpy(msd) codon' in the PLP gene
    Pavel Seeman
    Department of Child Neurology, Second School of Medicine, Charles University and University Hospital Motol, Prague, 150 06 Prague 5, Czech Republic
    Int J Mol Med 9:125-9. 2002
    Pelizaeus Merzbacher disease (PMD) is an X-linked recessive disorder of the central nervous system myelination caused by mutations involving the proteolipid protein gene (PLP)...
  76. ncbi Role of Asp222 in the catalytic mechanism of Escherichia coli aspartate aminotransferase: the amino acid residue which enhances the function of the enzyme-bound coenzyme pyridoxal 5'-phosphate
    T Yano
    Department of Medical Chemistry, Osaka Medical College, Japan
    Biochemistry 31:5878-87. 1992
    ..and is located within a distance of strong ionic interaction with N(1) of the coenzyme, pyridoxal 5'-phosphate (PLP), or pyridoxamine 5'-phosphate (PMP)...
  77. ncbi Current concepts of PLP and its role in the nervous system
    I Griffiths
    Department of Veterinary Clinical Studies, University of Glasgow, Bearsden, Scotland
    Microsc Res Tech 41:344-58. 1998
    Proteolipid protein (PLP) and its smaller isoform DM20 constitute the major myelin proteins of the CNS...
  78. doi Hearing profile and MRI myelination of auditory pathway in Pelizaeus-Merzbacher disease
    Chen Chieh Kuan
    Department of Otolaryngology, School of Medicine, University of Tokyo, Tokyo, Japan
    Acta Otolaryngol 128:539-46. 2008
    This study showed that delayed auditory pathway myelination is common in Pelizaeus-Merzbacher disease (PMD), but this delay does not necessarily indicate poor hearing function.
  79. doi Deletion of a splicing enhancer disrupts PLP1/DM20 ratio and myelin stability
    Erming Wang
    Department of Neurology, University of Kentucky, Lexington, KY 40536, USA
    Exp Neurol 214:322-30. 2008
    b>PLP1 and DM20, major myelin proteins, are generated by developmentally regulated alternative splicing. In the post-natal brain, PLP1 is the predominant product...
  80. doi PLP/DM20 expression and turnover in a transgenic mouse model of Pelizaeus-Merzbacher disease
    Saadia A Karim
    The Beatson Institute for Cancer Research, Garscube Estate, Switchback Road, Bearsden, Glasgow, G61 1BD, Scotland
    Glia 58:1727-38. 2010
    The most common cause of Pelizaeus-Merzbacher (PMD) is due to duplication of the PLP1 gene but it is unclear how increased gene dosage affects PLP turnover and causes dysmyelination...
  81. ncbi The proteolipid protein gene
    I R Griffiths
    Applied Neurobiology Group, University of Glasgow Veterinary School, UK
    Neuropathol Appl Neurobiol 21:85-96. 1995
    ..X-linked spastic paraplegia (SPG2) is allelic to PMD and is associated with PLP mutations in which the levels of the DM-20 isoform are probably ..
  82. ncbi Transgenic and natural mouse models of proteolipid protein (PLP)-related dysmyelination and demyelination
    I R Griffiths
    University of Glasgow Veterinary School, Bearsden, Scotland
    Brain Pathol 5:275-81. 1995
    The X chromosome-linked PLP/DM-20 gene is the CNS myelin gene most frequently associated with mutations, resulting in dysmyelination in several species including man (Pelizaeus-Merzbacher disease, X-linked Spastic Paraplegia)...
  83. ncbi Distinct phenotypes associated with increasing dosage of the PLP gene: implications for CMT1A due to PMP22 gene duplication
    T J Anderson
    Department of Veterinary Clinical Studies, University of Glasgow, Bearsden, Scotland
    Ann N Y Acad Sci 883:234-46. 1999
    Increased dosage of the proteolipid protein (Plp) gene causes CNS disease (Pelizaeus-Merzbacher disease [PMD]), which has many similarities to disorders of the PNS associated with duplication of the peripheral myelin protein-22 (PMP22) ..
  84. ncbi Mutations in noncoding regions of the proteolipid protein gene in Pelizaeus-Merzbacher disease
    G M Hobson
    Department of Research, Alfred I duPont Hospital for Children, Wilmington, DE 19899, USA
    Neurology 55:1089-96. 2000
    Pelizaeus-Merzbacher disease (PMD) is an X-linked recessive dysmyelinating disorder of the CNS. Duplications or point mutations in exons of the proteolipid protein (PLP) gene are found in most patients.
  85. ncbi Multiplex ligation-dependent probe amplification for rapid detection of proteolipid protein 1 gene duplications and deletions in affected males and carrier females with Pelizaeus-Merzbacher disease
    Ilka Warshawsky
    Department of Clinical Pathology, Cleveland Clinic Foundation, Cleveland, OH 44195, USA
    Clin Chem 52:1267-75. 2006
    ..is a rare X-linked neurodegenerative disorder caused by sequence variations in the proteolipid protein 1 gene (PLP1)...
  86. ncbi PLP1 gene duplication as a cause of the classic form of Pelizaeus-Merzbacher disease - case report
    Jacek Mądry
    Department of Neurology, Brodno Voivodship Hospital, Medical University of Warsaw
    Neurol Neurochir Pol 44:511-5. 2010
    ..PMD is caused by mutations in the PLP1 gene located at Xq22 and encoding the major myelin component in CNS, proteolipid protein 1 (PLP1)...
  87. ncbi Genetic background influences UPR but not PLP processing in the rumpshaker model of PMD/SPG2
    M McLaughlin
    Applied Neurobiology Group, Division of Cell Sciences, Institute of Comparative Medicine, University of Glasgow, Bearsden, Glasgow, G61 1QH, Scotland
    Neurochem Res 32:167-76. 2007
    ..of the proteolipid protein gene (PLP1) cause Pelizaeus-Merzbacher disease (PMD) and Spastic paraplegia type 2 (SPG2)...
  88. ncbi Late-onset neurodegeneration in mice with increased dosage of the proteolipid protein gene
    T J Anderson
    Applied Neurobiology Group, Department of Veterinary Clinical Studies, University of Glasgow, Scotland, United Kingdom
    J Comp Neurol 394:506-19. 1998
    Mutations of the proteolipid protein (Plp) gene cause a generalized central nervous system (CNS) myelin deficit in Pelizaeus-Merzbacher disease of man and various tremor syndromes in animal models...
  89. ncbi Genetic diagnosis of PLP gene duplications/deletions in patients with Pelizaeus-Merzbacher disease
    Q Gao
    Department of Medical and Molecular Genetics, Indiana University School of Medicine, Indianapolis, IN, USA
    Clin Genet 68:466-7. 2005
    Genetic diagnosis of PLP gene duplications/deletions in patients with Pelizaeus-Merzbacher disease.PMD is an X-linked recessive disorder due to a proteolipid protein (PLP) deficiency...
  90. ncbi Highly sensitive and simple liquid chromatographic determination in plasma of B6 vitamers, especially pyridoxal 5'-phosphate
    M Kimura
    Department of Social Medicine, Kyoto University, Japan
    J Chromatogr A 722:296-301. 1996
    ..e. pyridoxal and pyridoxal 5'-phosphate (PLP), and 4-pyridoxic acid (4-PA) in plasma was developed. The vitamers and 4-PA from plasma were extracted with 0...
  91. ncbi Three-dimensional spinal deformity in scoliosis associated with cerebral palsy and with progressive muscular dystrophy
    S Suzuki
    Department of Orthopaedic Surgery, Shiga Medical Center for Children, Japan
    Spine (Phila Pa 1976) 18:2290-4. 1993
    ..11 patients with scoliosis associated with cerebral palsy (CP) and 11 patients with progressive muscular dystrophy (PMD)...
  92. ncbi Human ornithine aminotransferase complexed with L-canaline and gabaculine: structural basis for substrate recognition
    S A Shah
    Department of Molecular Biophysics and Biochemistry, Yale University, New Haven, CT 06520, USA
    Structure 5:1067-75. 1997
    Ornithine aminotransferase (OAT) is a 45 kDa pyridoxal-5'-phosphate (PLP)-dependent enzyme that catalyzes the conversion of L-ornithine and 2-oxoglutarate to glutamate-delta-semialdehyde and glutamic acid, respectively...
  93. ncbi Crystal structure of serine dehydratase from rat liver
    Taro Yamada
    Department of Molecular Biosciences, University of Kansas, 1200 Sunnyside Avenue, Lawrence, Kansas 66045 7534, USA
    Biochemistry 42:12854-65. 2003
    SDH (L-serine dehydratase, EC 4.3.1.17) catalyzes the pyridoxal 5'-phosphate (PLP)-dependent dehydration of L-serine to yield pyruvate and ammonia. Liver SDH plays an important role in gluconeogenesis...
  94. ncbi Profilin inhibits pollen tube growth through actin-binding, but not poly-L-proline-binding
    Sylvester T McKenna
    Biology Department, Long Island University, Brooklyn, NY 11201, USA
    Planta 218:906-15. 2004
    ..Cell 12:1161-1175], which have defects in actin-binding, ability to inhibit polymerization, and poly- l-proline (PLP)-binding. Using Lilium longiflorum L. pollen and S...
  95. ncbi Efficient method for the extraction of the conditional probability distribution of polarization mode dispersion
    Antonio Mecozzi
    Department of Electrical Engineering, University of L Aquila, Poggio di Roio 67040 L Aquila, Italy
    Opt Lett 29:1482-4. 2004
    A new method for obtaining the probability density function of polarization mode dispersion (PMD) conditioned on the measured PMD value at an offset optical frequency is introduced...
  96. ncbi A new arrangement of (beta/alpha)8 barrels in the synthase subunit of PLP synthase
    Jianghai Zhu
    Department of Biological Sciences, Purdue University, West Lafayette, Indiana 47907, USA
    J Biol Chem 280:27914-23. 2005
    Pyridoxal 5'-phosphate (PLP, vitamin B6), a cofactor in many enzymatic reactions, has two distinct biosynthetic routes, which do not coexist in any organism...
  97. ncbi Enduring representational plasticity after somatosensory stimulation
    Carolyn W H Wu
    Laboratory of Functional and Molecular Imaging, Human Cortical Physiology Section, NINDS, NIH, Bethesda, MD 20892, USA
    Neuroimage 27:872-84. 2005
    ..in the motor network: primary motor cortex (M1), primary somatosensory cortex (S1), and dorsal premotor cortex (PMd) in healthy volunteers...
  98. ncbi Phantom limb pain treatment with mirtazapine: a case series
    Todd A Kuiken
    The Rehabilitation Institute of Chicago and the Department of PM and R, Northwestern University, Feinberg School of Medicine, Chicago, Illinois 60611, USA
    Pain Pract 5:356-60. 2005
    To pilot the efficacy of mirtazapine for relief of phantom limb pain (PLP); to correlate the putative drug mechanism with theoretical PLP mechanisms; and to develop a rationale for further study of mirtazapine in this population.
  99. ncbi Structural insights into catalysis and inhibition of O-acetylserine sulfhydrylase from Mycobacterium tuberculosis. Crystal structures of the enzyme alpha-aminoacrylate intermediate and an enzyme-inhibitor complex
    Robert Schnell
    Department of Medical Biochemistry and Biophysics, Karolinska Institutet, S 171 77 Stockholm, Sweden
    J Biol Chem 282:23473-81. 2007
    ..Recombinant CysK1 is a pyridoxal phosphate (PLP)-dependent enzyme that catalyzes the conversion of O-acetylserine to cysteine...
  100. ncbi The relationship between infectious and non-infectious herd factors with pneumonia at slaughter and productive parameters in fattening pigs
    Jorge Martinez
    Departamento de Atención Sanitaria, Universidad Cardenal Herrera CEU, Valencia, Spain
    Vet J 179:240-6. 2009
    ..Lung lesion classifications were catarrhal-purulent bronchopneumonia (CPBP), pleuropneumonia (PLP) and pleuritis. A mean lesion value (MLV) was calculated for each lesion...
  101. ncbi Partial bit delay correlative modulation used to improve the dispersion tolerance of an optical duobinary system
    Zhongxi Zhang
    Department of Physics, University of Ottawa, Ottawa, Ontario, Canada
    Opt Express 16:11344-53. 2008
    ..These conclusions are also valid for systems having non-negligible polarization-mode dispersion (PMD) and polarization dependent loss (PDL).

Research Grants65

  1. Shirley ShiDu Yan; Fiscal Year: 2014
    ..LRG-47 deficient mice targeted to oligodendrocytes under the control of mouse proteolipid protein (myelin, Plp-LRG-47-/-) 1 promoter, we will fully address our hypothesis and establish the benefits of LRG-47 blockade as a ..
  2. MYELIN PROTEIN GENE EXPRESSION IN DYSMYELINATING MUTANTS
    Wendy B Macklin; Fiscal Year: 2013
    DESCRIPTION (provided by applicant): The myelin proteolipid protein (PLP) is a well characterized CNS myelin protein, which constitutes 50% of the myelin membrane (Eng et al. 1968;Norton and Poduslo, 1973)...
  3. Vitamin B6 Effects on One-Carbon Metabolism
    Jesse F Gregory; Fiscal Year: 2013
    ..Through its coenzyme form pyridoxal phosphate (PLP), B6 plays essential roles in the acquisition and processing of one-carbon (1C) units by the glycine cleavage ..
  4. A Gene therapy Approach for the treatment of EAE
    Yacov Ron; Fiscal Year: 2007
    ..We have shown that expression of the myelin proteolipid protein (PLP) and myelin basic protein (MBP) encephalitogenic determinants in autologous B cells can effectively ..
  5. REACTION SPECIFICITY OF PYRIDOXAL PHOSPHATE ENZYMES
    MICHAEL TONEY; Fiscal Year: 2011
    DESCRIPTION (provided by applicant): Pyridoxal phosphate (PLP) dependent enzymes are ubiquitous in nitrogen metabolism and catalyze many medically important transformations...
  6. REGULATION OF PYRIDOXAL PHOSPHATE BIOSYNTHESIS IN E COLI
    Malcolm Winkler; Fiscal Year: 1999
    Pyridoxal 5'-phosphate (PLP) is an essential, ubiquitous coenzyme that participates in many aspects of amino acid and cellular metabolism...
  7. VITAMIN B6 ADEQUACY, SMOKING AND DNA DAMAGE
    Terry Shultz; Fiscal Year: 2002
    ..Vitamin B-6 metabolite concentrations [i.e., pyridoxal phosphate (PLP), pyridoxamine phosphate, pyridoxal, pyridoxine and 4-pyridoxic acid] will be determined in plasma and erythrocytes,..
  8. PERTURBATIONS IN VITAMIN B6 METABOLISM IN CANCER
    LOUISE NUTTER; Fiscal Year: 2000
    ..e., pyridoxal 5'-phosphate; PLP) is absent in certain liver and neurally-derived tumors and cell lines...
  9. VITAMIN B-6 STATUS IN PREGNANCY, LACTATION AND INFANCY
    PHYLIS MOSER VEILLON; Fiscal Year: 1993
    ..vitamin B-6 in pigs and humans, and (3) to compare the effectiveness of use of erythrocyte pyridoxal 5'-phosphate (PLP) concentration and hemoglobin-oxygen binding affinity as status indices of vitamin B-6 compared to other indices ..
  10. Richard B Silverman; Fiscal Year: 2016
    ..Studies involving isotopic labeling of 2 and of the pyridoxal phosphate (PLP) coenzyme will be carried out...
  11. Shelley D Copley; Fiscal Year: 2016
    ..coli that have been adapted to grow more efficiently on glucose while using serendipitous pathways to supply PLP. Aim 2 describes plans to characterize the molecular mechanisms of the mutations that allow E...
  12. Structure and Mechanisms of PLP Dependent Lyases
    Robert Phillips; Fiscal Year: 2006
    DESCRIPTION (provided by applicant) Enzymes containing pyridoxal-5'-phosphate (PLP) are ubiquitous in biology, performing essential reactions in metabolism of amino acids and amines...
  13. 5-Aminolevulinate Synthase and Heme Biosynthesis
    Gloria Ferreira; Fiscal Year: 2006
    DESCRIPTION (provided by applicant): 5-Aminolevulinate synthase (ALAS), a pyridoxal 5'-phosphate (PLP)-dependent enzyme of the alpha-oxoaminesynthase family, catalyzes the first and regulatory step of the mammalian heme biosynthetic ..
  14. John P Donoghue; Fiscal Year: 2016
    ..Dorsal premotor (PMd) and ventral premotor (PMv) areas of frontal cortex appear to represent separate processing streams that converge ..
  15. Activation of Plp gene expression in oligodendrocytes
    Patricia A Wight; Fiscal Year: 2010
    The myelin proteolipid protein (PLP) gene (Plp1) encodes the most abundant protein present in CNS myelin. Its expression in oligodendrocytes is tightly regulated...
  16. Treatment for Dysmyelination in PMD and SPG2
    Pamela E Knapp; Fiscal Year: 2013
    ..The PLP1 gene codes for both PLP, the major intrinsic CNS myelin protein, as well as the less abundant DM20 protein...
  17. Lyn B Jakeman; Fiscal Year: 2014
    ..of the study, the primary motor cortex (M1), the supplementary motor area (SMA), and the dorsal premotor cortex (PMd). M1 and SMA are strong sources of corticospinal projections...
  18. Visual/Spatial Properties of Posterior Parietal Neurons
    Richard A Andersen; Fiscal Year: 2011
    ..In Aim 1 we will advance a new finding, made in dorsal premotor cortex (PMd) during the last grant period, of a relative coding of the position of the hand, eyes and goal of a reach...
  19. Molecular genetics of Pelizaeus-Merzbacher disease
    Grace M Hobson; Fiscal Year: 2013
    ..disease (PMD) is an X-linked leukodystrophy caused by genetic defects of the proteolipid protein 1 gene (PLP1) that encodes the major central nervous system myelin protein...
  20. Robert P Skoff; Fiscal Year: 2016
    ..Increased levels of PLP may be caused by duplications of the native proteolipid protein (PLP1) gene as happens in the human disease Pelizaeus-Merzbacher Disease or by increased expression levels of Plp1mRNA ..
  21. ECoG Correlates of Visuomotor Transformation and Application to a Force-Based BCI
    Jordan J Williams; Fiscal Year: 2012
    ..We will first examine the epidural ECoG spectra of arrays implanted over M1, PMd, and parietal area 5 for correlations with movement parameters of the joystick or cursor, and compare these ..
  22. The role of focal adhesion kinase in CNS myelination
    Audrey D Lafrenaye; Fiscal Year: 2010
    ..using transgenic mice that express a tamoxifen-inducible Cre protein under the control of the proteolipid protein (PLP) promoter (PLP/CreERT) and mice that harbor homologous recombined FAK alleles in which the second kinase domain of ..
  23. James Ashe; Fiscal Year: 2016
    ..multi-electrode arrarys and mico-electroencephalography (ECoG) arrays in primary motor (M1) and dorsal premotor (PMd) cortex, We plan to study several different neural signals including single unit activity (SUA), multi-unit ..
  24. PATHOGENESIS AND IMMUNOREGULATION OF PLP-INDUCED R-EAE
    Stephen D Miller; Fiscal Year: 2010
    ....
  25. Jose Luis Millan; Fiscal Year: 2016
    ..lead to the early demise of Akp2-/- mice and are caused by inadequate utilization of pyridoxal-5'-phosphate (PLP - a hydrophilic form of Vitamin B6), another natural substrate of TNAP...
  26. mTOR complexes in oligodendrocyte differentiation
    STACEY ELIZABETH CIFELLI; Fiscal Year: 2013
    ..Bigenic mice carrying floxed-mTOR and either a CNP-Cre transgene or an inducible PLP-Cre transgene will be created for oligodendrocyte specific knockdown of mTOR in vivo...
  27. Trb3-Mediated Modulation of Oligodendrocyte Stress
    Alexander Gow; Fiscal Year: 2011
    ..study oligodendrocytes as the model cell and use naturally-occurring missense mutations in the proteolipid protein (Plp1) gene to induce the UPR in vivo (i.e. mouse models) and in vitro (transfected cells)...
  28. AUTOMATED DETECTION OF GENE DUPLICATIONS OR DELETIONS
    Fatima Merchant; Fiscal Year: 2005
    ..Palsies (HNPP), (2) neurological disorders; Pelizaeus-Merzbacher Disease (PMD) and X-Linked Spastic Paraplegia (SPG2), (3) muscular wasting disorders; Duchene (DMD) and Becker Muscular Dystrophy (BMD), (4) contiguous-gene syndromes; ..
  29. Bruce D Trapp; Fiscal Year: 2014
    ..Late onset axonopathies and axonal degeneration develop in mice null for PLP and in mice where PLP was replaced by P0 protein...
  30. AUTOMATED DETECTION OF GENE DUPLICATIONS OR DELETIONS
    Fatima Merchant; Fiscal Year: 2000
    ..Palsies (HNPP), (2) neurological disorders; Pelizaeus-Merzbacher Disease (PMD) and X-Linked Spastic Paraplegia (SPG2), (3) muscular wasting disorders; Duchene (DMD) and Becker Muscular Dystrophy (BMD), (4) contiguous-gene syndromes; ..
  31. ASSEMBLY AND MAINTENANCE OF THE MYELIN SHEATH
    Pierre Morell; Fiscal Year: 2002
    ..It contains unique proteins, including proteolipid protein (PLP), which accounts for over one-third of myelin protein, and unique lipids, including galactosylceramide (cerebroside)..
  32. Viruses and Autoimmunity ot the Central Nervous System
    ROBERT FUJINAMI; Fiscal Year: 2004
    ..We have made a recombinant vaccinia virus (VV) that encodes myelin proteolipid protein (PLP). This initial infection (VV-PLP) does not appear to incite CNS disease by itself...
  33. HYPOMYELINATED MUTANT AND DOUBLE MUTANT
    Merrill Wolf; Fiscal Year: 2000
    ..by studying hypomyelinating mutations in the mouse genes for myelin basic protein, MBP, and proteolipid protein, PLP, the target of human Pelizaeus- Merzbacher's disease...
  34. Sienmi Du; Fiscal Year: 2014
    ..Our laboratory previously focused on generation of immune responses in EAE and showed that the transfer of PLP 139-151 sensitized lymph node cells with XX sex chromosome complement leads to greater disease severity than cells ..
  35. PLP Splicing: In Vivo Analysis in A Mouse Model
    Franca Cambi; Fiscal Year: 2004
    The long-term goals of this project are to elucidate the function of a G-rich 19-bp sequence in intron 3 of the PLP gene in PLP-DM20 splice site selection in vivo...
  36. Arthur A Vandenbark; Fiscal Year: 2014
    ..such as myelin oligodendroglial glycoprotein (MOG), myelin basic protein (MBP) and proteolipid protein (PLP) can induce severe signs of experimental autoimmune encephalomyelitis (EAE) in mice that include ascending ..
  37. GENE THERAPEUTIC APPROACH FOR TOLERANCE INDUCTION
    DAVID WILLIAM SCOTT; Fiscal Year: 2013
    ..CNS antigens, such as myelin oligodendrocyte glycoprotein (MOG), myelin basic protein (MBP) and phospholipoprotein (PLP);hence, modulation of these immune responses is a goal for therapeutic intervention in MS...
  38. Yeast Based Assays for Chemical Screens Against SARS-CoV Targets
    DANIEL A contact ENGEL; Fiscal Year: 2010
    ..based assay will be used for the identification of compounds with activity against the SARS-CoV targets Nsp1 and PLP. Both targets give pronounced phenotypes when expressed in yeast, forming the basis for chemical screens to ..
  39. Mast cell actions in chronic progressive versus relapsing remitting EAE
    JULIANNE K HATFIELD; Fiscal Year: 2013
    ..Arguably, the PLP-induced model in SJL mice is more relevant because it presents with a relapsing-remitting (RR) course experienced ..
  40. Kamal D Moudgil; Fiscal Year: 2014
    ..A variety of autoantigens, including myelin oligodendrocyte glycoprotein (MOG), proteolipid protein (PLP), and myelin basic protein (MBP) have been implicated in the pathogenesis of MS...
  41. LPL-Deficient Cats-A Quantitative Model of Gene Delivery
    PHILIP RICHARD VULLIET; Fiscal Year: 2010
    ..mutation and cannot hydrolyze chylomicrons resulting in identical aberrations of their plasma lipid profile (PLP)...
  42. AMBER MARIE SMITH; Fiscal Year: 2014
    ..In aim 1 an inactive PLP synthase mutant will be co-crystallized with its substrates...
  43. Pawel A Osmulski; Fiscal Year: 2014
    ..Both autophagy and proteasome-mediated degradation [PMD] play critical roles in intracellular protein quality control...
  44. THE ROLE OF CHEMOKINES IN AUTOIMMUNE ENCEPHALOMYELITIS
    William Karpus; Fiscal Year: 2009
    ..1 of the major goals of this application is to address the role of chemokines and chemokine receptors in PLP-induced EAE, including determining the chemokines that are important in regulating macrophage, microglia, and ..
  45. Johanna M Rommens; Fiscal Year: 2015
    ..Motivated by previous work that has shown variations in percent mammographic density (PMD) to be associated with large differences in risk of breast cancer in women of middle age and older, to be highly ..
  46. Steven E Ealick; Fiscal Year: 2016
    ..In yeast, thiamin pyrophosphate is synthesized from glycine, cysteine, NAD, PLP and histidine and requires four enzymes...
  47. Secretion of Virulence Factors by Mycobacterium tuberculosis
    Jeffery S Cox; Fiscal Year: 2010
    ..tuberculosis influences host-pathogen contacts by utilizing two virulence secretion systems: the MmpL family of transporters, which secretes polyketide lipids to the bacterial cell surface, and the Snm secretion ..
  48. Homocysteine &Heme: A Role in Thrombosis?
    Jan P Kraus; Fiscal Year: 2012
    ..that requires three different cofactors: S-adenosylmethionine (AdoMet), heme and pyridoxal 5'-phosphate (PLP)...
  49. Alberto J Espay; Fiscal Year: 2015
    ..foundations for a successful pursuit of an independent clinical research career on psychogenic movement disorders (PMD)...
  50. Rulla M Tamimi; Fiscal Year: 2016
    ..of mammographic density utilizes an operator-assisted technique based on the percentage of mammographic density (PMD)...
  51. Evidence-Based Pain Intervention for Veterans: Leveraging Mobile &Social Media
    Sara S Johnson; Fiscal Year: 2013
    ..and PTSD, pain and Traumatic Brain Injury (TBI), and all three conditions (Post-deployment Multi-symptom Disorder (PMD)) can complicate and reduce the effectiveness of treatment for pain...
  52. NITRIC OXIDE, PLP ACYLATION & THE PATHOPHYSIOLOGY OF MS
    OSCAR BIZZOZERO; Fiscal Year: 2003
    ..We hypothesize that both the structure and fatty acid acylation of the abundant myelin proteolipid protein (PLP) are affected by NO and peroxynitrites, and that this may lead to myelin instability...
  53. Babar A Khan; Fiscal Year: 2016
    ..neuroprotection in the context of a large randomized clinical trial, the Pharmacological Management of Delirium (PMD) trial. Dr...
  54. Nicholas G Hatsopoulos; Fiscal Year: 2016
    ..LFP) recordings in non-human primates that propagates horizontally across primary motor (MI), dorsal premotor (PMd), and ventral premotor (PMv) cortices...
  55. David A Agard; Fiscal Year: 2016
    ..Our SIM/STORM imaging has revealed unexpected structural domains within the centrosomal pericentriolar material. We continue these efforts assessing interactions and assembly including a structural analysis of Plp.
  56. Optical Surgical Probe for Assessing Human Oral Mucosa Graft Vascularization
    David E Wolf; Fiscal Year: 2011
    ..by applicant): This proposal's main objective is the development of a novel Perfusion Monitoring Device (PMD) based upon diffuse correlation spectroscopy (DCS), which will enable microsurgeons to assess the level of ..
  57. Synergy between T cells and Complement in EAE
    Feng Lin; Fiscal Year: 2010
    ..In recent work we have found that 1) autoreactivity in MOG 35-55 induced EAE extends to proteolipid protein (PLP) and 2) mice doubly deficient in DAF and CD59, a related intrinsic complement inhibitor that works together with ..
  58. Multiple Antigenic Peptides to Alter the Course of Autoimmune Disease
    David J Hinrichs; Fiscal Year: 2013
    ..the influence of our peptide-based immunotherapy and we have used encephalitogenic peptides of proteolipid protein (PLP) and myelin basic protein (MBP) to induce disease and to construct the octameric peptides...
  59. Development of a Single Motor Propelled Wheelchair (SIMPL-WC) Phase II
    CHRIS WILLEMS; Fiscal Year: 2012
    ..Motor Propelled Wheelchair (SIMPL-WC) that incorporates a novel drive-train which allows for a true continuum of PMD solutions...
  60. PROFILIN I--AN ESSENTIAL PROTEIN IN CELLS AND ANIMALS
    Pascal Goldschmidt Clermont; Fiscal Year: 1999
    ..in vitro: (1) profilin binds to monomeric actin (stoichiometry; one to one): (ii) profilin binds to poly-L-proline (PLP,one profilin binds to a stretch of greater than prolines); (iii) profilin binds membranes containing ..
  61. Richard A Andersen; Fiscal Year: 2014
    ..Aim 2 will examine the dorsal premotor cortex (PMd) to see if it is similar to the parietal reach region (PRR) in representing both potential plans and outcomes...
  62. Claudio Soto; Fiscal Year: 2015
    ..This proposal is structured around the hypothesis that misfolded proteins associated to one PMD might initiate or accelerate the pathogenic cascade of a second PMD-related disease through a cross-seeding ..
  63. BASOPHIL/MAST CELL FUNCTION
    Ann Dvorak; Fiscal Year: 2004
    DESCRIPTION: (Adapted from the Investigator's abstract): Piecemeal degranulation (PMD) of basophils and mast cells plays a central role in the secretion of histamine which is effected by the transport of histamine-loaded vesicles from ..
  64. Investigating Reach Trajectory Planning in Macaque Dorsal Premotor Cortex
    THOMAS MICHAEL PEARCE; Fiscal Year: 2013
    ..Specifically, this proposal investigates how a particular cortical region - dorsal premotor cortex (PMd) - is involved in controlling reaching, from the initial trajectory to the final position...
  65. MALIGNANCY-ASSOCIATED HYPERCALCEMIA
    Gordon Strewler; Fiscal Year: 1993
    ..The PTH-like protein (PLP) is expressed by squamous and renal carcinomas, binds with high affinity to the PTH receptor in kidney and bone, ..