transthyretin

Summary

Gene Symbol: transthyretin
Description: transthyretin
Alias: CTS, CTS1, HEL111, HsT2651, PALB, TBPA, transthyretin, ATTR, carpal tunnel syndrome 1, epididymis luminal protein 111, prealbumin, amyloidosis type I, thyroxine-binding prealbumin
Species: human

Top Publications

  1. ncbi Structure of a complex of two plasma proteins: transthyretin and retinol-binding protein
    H L Monaco
    Department of Genetics, University of Pavia, Italy
    Science 268:1039-41. 1995
  2. ncbi HIV-1 Tat depresses DNA-PK(CS) expression and DNA repair, and sensitizes cells to ionizing radiation
    Yi Sun
    Department of Radiation Toxicology and Oncology, Beijing Institute of Radiation Medicine, Beijing, People s Republic of China
    Int J Radiat Oncol Biol Phys 65:842-50. 2006
  3. ncbi DJ-1 degrades transthyretin and an inactive form of DJ-1 is secreted in familial amyloidotic polyneuropathy
    Shizuyo Koide-Yoshida
    Graduate School of Pharmaceutical Sciences, Hokkaido University, Sapporo 060 8589, Japan
    Int J Mol Med 19:885-93. 2007
  4. ncbi Structure of prealbumin: secondary, tertiary and quaternary interactions determined by Fourier refinement at 1.8 A
    C C Blake
    J Mol Biol 121:339-56. 1978
  5. ncbi A novel transthyretin mutation at position 30 (Leu for Val) associated with familial amyloidotic polyneuropathy
    T Murakami
    First Department of Internal Medicine, Kumamoto University School of Medicine, Japan
    Biochem Biophys Res Commun 187:397-403. 1992
  6. pmc Fibril in senile systemic amyloidosis is derived from normal transthyretin
    P Westermark
    Department of Pathology, University of Linkoping, Sweden
    Proc Natl Acad Sci U S A 87:2843-5. 1990
  7. ncbi Presence of an abnormal transthyretin (prealbumin) in Portuguese patients with familial amyloidotic polyneuropathy
    M J Saraiva
    Trans Assoc Am Physicians 96:261-70. 1983
  8. pmc The preaggregated state of an amyloidogenic protein: hydrostatic pressure converts native transthyretin into the amyloidogenic state
    A D Ferrão-Gonzales
    Programa de Biologia Estrutural, Departamento de Bioquimica Medica, Instituto de Ciencias Biomedicas, Universidade Federal do Rio de Janeiro, Rio de Janeiro, Brazil
    Proc Natl Acad Sci U S A 97:6445-50. 2000
  9. ncbi Trans-suppression of misfolding in an amyloid disease
    P Hammarstrom
    Department of Chemistry and The Skaggs Institute of Chemical Biology, The Scripps Research Institute, 10550 North Torrey Pines Road BCC265, La Jolla, CA 92037, USA
    Science 293:2459-62. 2001
  10. pmc Molecular conformation of a peptide fragment of transthyretin in an amyloid fibril
    Christopher P Jaroniec
    Department of Chemistry and Center for Magnetic Resonance, Francis Bitter Magnet Laboratory, Massachusetts Institute of Technology, Cambridge, MA 02139, USA
    Proc Natl Acad Sci U S A 99:16748-53. 2002

Research Grants

  1. BIOSYNTHETIC ACTIVITY OF THE CHOROID PLEXUS
    Joseph Herbert; Fiscal Year: 1993
  2. GENE EXPRESSION IN FAMILIAL AND SENILE CARDIAC AMYLOID
    Joel Buxbaum; Fiscal Year: 1993
  3. THYROID HORMONE ANALOGS
    Manfred Wolff; Fiscal Year: 1980
  4. Novel Insights into the Pathogenesis of Light Chain Cardiac Amyloidosis
    Flora Sam; Fiscal Year: 2011
  5. Molecular mechanism of senile cardiac amyloidosis
    Lawreen H Connors; Fiscal Year: 2013
  6. Lipid acceleration of transthyretin amyloidosis
    Irit Rappley; Fiscal Year: 2012
  7. Natalia Reixach; Fiscal Year: 2014
  8. Regina M Murphy; Fiscal Year: 2016
  9. Cardiac Amyloidosis in Aging African American
    Joel N Buxbaum; Fiscal Year: 2012
  10. INHIBITION OF AMYLOID FORMATION BY TRANSTHYRETIN & APOE
    Dmitry Goldgaber; Fiscal Year: 1999

Detail Information

Publications232 found, 100 shown here

  1. ncbi Structure of a complex of two plasma proteins: transthyretin and retinol-binding protein
    H L Monaco
    Department of Genetics, University of Pavia, Italy
    Science 268:1039-41. 1995
    The three-dimensional structure of the complex formed by two plasma proteins, transthyretin and retinol-binding protein, was determined from x-ray diffraction data to a nominal resolution of 3.1 angstroms...
  2. ncbi HIV-1 Tat depresses DNA-PK(CS) expression and DNA repair, and sensitizes cells to ionizing radiation
    Yi Sun
    Department of Radiation Toxicology and Oncology, Beijing Institute of Radiation Medicine, Beijing, People s Republic of China
    Int J Radiat Oncol Biol Phys 65:842-50. 2006
    ..Here we explored the effects of the HIV-1 Tat protein on cellular responses to ionizing radiation...
  3. ncbi DJ-1 degrades transthyretin and an inactive form of DJ-1 is secreted in familial amyloidotic polyneuropathy
    Shizuyo Koide-Yoshida
    Graduate School of Pharmaceutical Sciences, Hokkaido University, Sapporo 060 8589, Japan
    Int J Mol Med 19:885-93. 2007
    ..DJ-1 has a protease-like structure and transthyretin (TTR), a protein causing familial amyloidotic polyneuropathy (FAP), was identified as a substrate for DJ-1 ..
  4. ncbi Structure of prealbumin: secondary, tertiary and quaternary interactions determined by Fourier refinement at 1.8 A
    C C Blake
    J Mol Biol 121:339-56. 1978
  5. ncbi A novel transthyretin mutation at position 30 (Leu for Val) associated with familial amyloidotic polyneuropathy
    T Murakami
    First Department of Internal Medicine, Kumamoto University School of Medicine, Japan
    Biochem Biophys Res Commun 187:397-403. 1992
    A novel transthyretin (TTR) mutation associated with familial amyloidotic polyneuropathy was detected in a Japanese patient...
  6. pmc Fibril in senile systemic amyloidosis is derived from normal transthyretin
    P Westermark
    Department of Pathology, University of Linkoping, Sweden
    Proc Natl Acad Sci U S A 87:2843-5. 1990
    ..fibril in senile systemic amyloidosis (SSA), like that of familial amyloidotic polyneuropathy, is derived from transthyretin (TTR)...
  7. ncbi Presence of an abnormal transthyretin (prealbumin) in Portuguese patients with familial amyloidotic polyneuropathy
    M J Saraiva
    Trans Assoc Am Physicians 96:261-70. 1983
    ..Based on these observations, we postulate that this abnormal form of TTR selectively deposits in tissues as the amyloid characteristic of the disease...
  8. pmc The preaggregated state of an amyloidogenic protein: hydrostatic pressure converts native transthyretin into the amyloidogenic state
    A D Ferrão-Gonzales
    Programa de Biologia Estrutural, Departamento de Bioquimica Medica, Instituto de Ciencias Biomedicas, Universidade Federal do Rio de Janeiro, Rio de Janeiro, Brazil
    Proc Natl Acad Sci U S A 97:6445-50. 2000
    ..Here, the properties and formation of amyloidogenic intermediates of transthyretin (TTR) were investigated by the use of hydrostatic pressure and spectroscopic techniques...
  9. ncbi Trans-suppression of misfolding in an amyloid disease
    P Hammarstrom
    Department of Chemistry and The Skaggs Institute of Chemical Biology, The Scripps Research Institute, 10550 North Torrey Pines Road BCC265, La Jolla, CA 92037, USA
    Science 293:2459-62. 2001
    The transthyretin (TTR) amyloid diseases, representative of numerous misfolding disorders, are of considerable interest because there are mutations that cause or suppress disease...
  10. pmc Molecular conformation of a peptide fragment of transthyretin in an amyloid fibril
    Christopher P Jaroniec
    Department of Chemistry and Center for Magnetic Resonance, Francis Bitter Magnet Laboratory, Massachusetts Institute of Technology, Cambridge, MA 02139, USA
    Proc Natl Acad Sci U S A 99:16748-53. 2002
    The molecular conformation of peptide fragment 105-115 of transthyretin, TTR(105-115), previously shown to form amyloid fibrils in vitro, has been determined by magic-angle spinning solid-state NMR spectroscopy...
  11. ncbi Probing solvent accessibility of transthyretin amyloid by solution NMR spectroscopy
    Anders Olofsson
    Department of Molecular Biology, Umea University, Umea SE 901 87, Sweden
    J Biol Chem 279:5699-707. 2004
    The human plasma protein transthyretin (TTR) may form fibrillar protein deposits that are associated with both inherited and idiopathic amyloidosis...
  12. pmc Tissue damage in the amyloidoses: Transthyretin monomers and nonnative oligomers are the major cytotoxic species in tissue culture
    Natalia Reixach
    Division of Rheumatology Research, Department of Molecular and Experimental Medicine, The Scripps Research Institute, 10550 North Torrey Pines Road, La Jolla, CA 92037, USA
    Proc Natl Acad Sci U S A 101:2817-22. 2004
    The transthyretin (TTR) amyloidoses are human diseases in which the misfolded TTR protein aggregates in tissues with subsequent visceral, peripheral, and autonomic nerve dysfunction...
  13. ncbi L55P transthyretin accelerates subunit exchange and leads to rapid formation of hybrid tetramers
    Catherine A Keetch
    Department of Chemistry, University of Cambridge, Cambridge CB2 1EW, United Kingdom
    J Biol Chem 280:41667-74. 2005
    b>Transthyretin is a tetrameric protein associated with the commonest form of systemic amyloid disease...
  14. ncbi Oxidation inhibits amyloid fibril formation of transthyretin
    Simin D Maleknia
    School of Biological, Earth and Environmental Sciences, University of New South Wales, Sydney, NSW, Australia
    FEBS J 273:5400-6. 2006
    ..b>Transthyretin (TTR) is a soluble human plasma protein...
  15. ncbi Clinical indications for plasma protein assays: transthyretin (prealbumin) in inflammation and malnutrition
    A Myron Johnson
    Department of Pediatrics, University of North Carolina School of Medicine, Chapel Hill, NC 27410, USA
    Clin Chem Lab Med 45:419-26. 2007
    A large number of circumstances are associated with reduced serum concentrations of transthyretin (TTR), or prealbumin...
  16. doi Structural insight into pH-induced conformational changes within the native human transthyretin tetramer
    Satheesh K Palaninathan
    Department of Biochemistry and Biophysics, Texas A and M University, College Station, TX 77843, USA
    J Mol Biol 382:1157-67. 2008
    Acidification of the transthyretin (TTR) tetramer facilitates dissociation and conformational changes in the protein, allowing alternatively folded monomers to self-assemble into insoluble amyloid fibers by a downhill polymerization ..
  17. pmc Transthyretin and complex protein pattern in aqueous humor of patients with primary open-angle glaucoma
    F H Grus
    Experimental Ophthalmology, Department of Ophthalmology, Johannes Gutenberg University, Mainz, Germany
    Mol Vis 14:1437-45. 2008
    ..To analyze protein patterns in the aqueous humor of glaucoma patients in comparison to control subject using two different methods...
  18. pmc Heterogeneity of penetrance in familial amyloid polyneuropathy, ATTR Val30Met, in the Swedish population
    Urban Hellman
    Medical and Clinical Genetics, Umea University, Umea, Sweden
    Amyloid 15:181-6. 2008
    b>Transthyretin (TTR) familial amyloid polyneuropathies (FAP) are autosomal dominant devastating afflictions. They were first described in Portugal, later in Japan and Sweden and are now recognized worldwide...
  19. doi Analyses of transthyretin concentration in the cerebrospinal fluid of patients with Guillain-Barré syndrome and other neurological disorders
    Han Lin Chiang
    Department of Neurology, Chang Gung Memorial Hospital, Chang Gung University College of Medicine, Taipei, Taiwan
    Clin Chim Acta 405:143-7. 2009
    ..Previously, using two-dimensional gel electrophoresis and mass spectrometry, we found that the transthyretin level was altered in the cerebrospinal fluid (CSF) of GBS patients when compared to that in CSF of control ..
  20. pmc A possible role for miRNA silencing in disease phenotype variation in Swedish transthyretin V30M carriers
    Malin Olsson
    Department of Medicine, Umea University, Umea, Sweden
    BMC Med Genet 11:130. 2010
    Familial amyloidosis with polyneuropathy (FAP) is an autosomal dominant disease caused by transthyretin (TTR) mutations, of which V30M (TTR c.148G > A, p.Val50Met, "Val30Met") is the most common...
  21. doi Transthyretin-related hereditary amyloidosis in a Chinese family with TTR Y114C mutation
    Yu Zhang
    Department of Neurology and Institute of Neurology, Ruijin Hospital Affiliated to Shanghai Jiao Tong University School of Medicine, Shanghai, China
    Neurodegener Dis 8:187-93. 2011
    b>Transthyretin-related hereditary amyloidosis is an autosomal dominant inherited disease caused by mutations in the transthyretin (TTR) gene...
  22. doi Human serum transthyretin levels correlate inversely with Alzheimer's disease
    Sun Ho Han
    Department of Biochemistry and Biomedical Science, Seoul National University, College of Medicine, Seoul, Korea
    J Alzheimers Dis 25:77-84. 2011
    ..constitutes the epicenter of AD pathology, Aβ-binding proteins that regulate Aβ aggregation, such as transthyretin (TTR), have attracted much attention...
  23. pmc Heparan sulfate/heparin promotes transthyretin fibrillization through selective binding to a basic motif in the protein
    Fredrik Noborn
    Department of Medical Biochemistry and Microbiology, The Biomedical Center, Uppsala University, Husargatan 3, Box 582, 751 23 Uppsala, Sweden
    Proc Natl Acad Sci U S A 108:5584-9. 2011
    b>Transthyretin (TTR) is a homotetrameric protein that transports thyroxine and retinol...
  24. doi Vitreous amyloidosis in two large mainland Chinese kindreds resulting from transthyretin variant Lys35Thr and Leu55Arg
    Da Long
    Department of Ophthalmology, The 2nd Xiangya Hospital of Central South University, Changsha, China
    Ophthalmic Genet 33:28-33. 2012
    To describe the clinical and pathological findings of two large mainland Chinese kindreds with vitreous amyloidosis and associated transthyretin mutation.
  25. doi Plasma transthyretin as a candidate marker for Alzheimer's disease
    Latha Velayudhan
    Department of Old Age Psychiatry, NIHR Specialist Biomedical Research Centre for Mental Health at the South London and Maudsley NHS Foundation Trust, London, UK
    J Alzheimers Dis 28:369-75. 2012
    ..Among these, one of the most interesting candidates is transthyretin (TTR), the carrier of thyroxine and retinol, which also binds with amyloid-β (Aβ), and it has been suggested ..
  26. doi A new Arg54Gly transthyretin gene mutation associated with vitreous amyloidosis in Chinese
    Yining Shi
    Department of Ophthalmology, Shaanxi Provincial People s Hospital, China
    Eye Sci 26:230-8. 2011
    ..with familial vitreous amyloidosis in Liaoning Province, China, and to investigate the correlation between the clinical appearance of the disease and transthyretin (TTR) gene mutation, including the locus and type of TTR gene mutation.
  27. doi STT3B-dependent posttranslational N-glycosylation as a surveillance system for secretory protein
    Takashi Sato
    Department of Molecular Medicine, Global COE Cell Fate Regulation Research and Education Unit, Graduate School of Pharmaceutical Sciences, Kumamoto University, 5 1 Oe honmachi, Kumamoto 862 0973, Japan
    Mol Cell 47:99-110. 2012
    ..the cryptic N-glycosylation site as a recognition signal for unfolding of a natively nonglycosylated protein, transthyretin (TTR), involved in familial amyloidosis...
  28. pmc Identification of beta-amyloid-binding sites on transthyretin
    Jiali Du
    Chemical and Biological Engineering Department, University of Wisconsin Madison, Madison, WI 53706, USA
    Protein Eng Des Sel 25:337-45. 2012
    b>Transthyretin (TTR) binds to the Alzheimer-related peptide beta-amyloid (Aβ), and may protect against Aβ-induced neurotoxicity. In this work, the specific domains on TTR involved with binding to Aβ were probed...
  29. doi Genetic stabilization of transthyretin, cerebrovascular disease, and life expectancy
    Louise S Hornstrup
    Department of Clinical Biochemistry, Rigshospitalet, Copenhagen, Denmark
    Arterioscler Thromb Vasc Biol 33:1441-7. 2013
    b>Transthyretin can cause amyloidosis attributable to destabilization of transthyretin tetramers in plasma...
  30. ncbi Structure of the human prealbumin gene
    T Tsuzuki
    J Biol Chem 260:12224-7. 1985
    ..Unexpectedly, two independent open reading frames provided with respective regulatory sequences were found within the gene: one in the first intron and the other in the third intron...
  31. ncbi Amyloid fibril composition and transthyretin gene structure in senile systemic amyloidosis
    A Gustavsson
    Department of Pathology I, Linkoping University, Sweden
    Lab Invest 73:703-8. 1995
    In many different forms of amyloidosis, transthyretin (TTR) comprises the major amyloid fibril protein. In the familial forms, various TTR mutations are linked to disease...
  32. ncbi Transthyretin mutations in health and disease
    M J Saraiva
    Instituto de Ciencias Biomedicas, Universidade do Porto, Portugal
    Hum Mutat 5:191-6. 1995
    To date, over 40 different mutations in transthyretin (TTR) have been associated with amyloid deposition. The major unresolved problem is the correlation between the clinical heterogeneity and the genetic heterogeneity...
  33. ncbi The x-ray crystal structure refinements of normal human transthyretin and the amyloidogenic Val-30-->Met variant to 1.7-A resolution
    J A Hamilton
    Department of Biochemistry and Molecular Biology, Indiana University School of Medicine, Indianapolis 46202 5122
    J Biol Chem 268:2416-24. 1993
    The x-ray crystal structures of normal human transthyretin (prealbumin) and the amyloidogenic Val-30-Met variant have been refined at 1.7-A resolution to R-values of 0.168 and 0...
  34. ncbi Prevention of transthyretin amyloid disease by changing protein misfolding energetics
    Per Hammarstrom
    Department of Chemistry and The Skaggs Institute of Chemical Biology, The Scripps Research Institute, 10550 North Torrey Pines Road, La Jolla, CA 92037, USA
    Science 299:713-6. 2003
    ..Here, we describe a series of transthyretin amyloidosis inhibitors that functioned by increasing the kinetic barrier associated with misfolding, ..
  35. ncbi Haplotypes and DNA sequence variation within and surrounding the transthyretin gene: genotype-phenotype correlations in familial amyloid polyneuropathy (V30M) in Portugal and Sweden
    Miguel Luz Soares
    Division of Rheumatology Research and the W M Keck Autoimmune Disease Center, Department of Molecular and Experimental Medicine, The Scripps Research Institute, La Jolla, CA, USA
    Eur J Hum Genet 12:225-37. 2004
    ..polyneuropathy (FAP) is a lethal autosomal dominant disorder in which fibrils derived from mutant forms of transthyretin (TTR), the normal plasma carrier of thyroxine (T(4)) and retinol-binding protein, are deposited in tissues...
  36. ncbi Dimeric transthyretin variant assembles into spherical neurotoxins
    Kimiaki Matsubara
    Faculty of Pharmaceutical Sciences, Toyama Medical and Pharmaceutical University, Toyama 930 0194, Japan
    Biochemistry 44:3280-8. 2005
    Familial amyloidotic polyneuropathy is a hereditary autosomal-dominant disease in which the deposited transthyretin fibrils are derived from amyloidogenic mutation...
  37. pmc Impact of homozygosity for an amyloidogenic transthyretin mutation on phenotype and long term outcome
    G Holmgren
    J Med Genet 42:953-6. 2005
    Although amyloidogenic transthyretin (ATTR) mutations are common in several populations, such as black Americans, the small number of diagnosed patients homozygous for TTR amyloid and the short follow up in most studies has until now ..
  38. ncbi Transthyretin: no association between serum levels or gene variants and schizophrenia
    Dina Ruano
    Life and Health Sciences Research Institute ICVS, School of Health Sciences, University of Minho, Campus de Gualtar, 4710 057 Braga, Portugal
    J Psychiatr Res 41:667-72. 2007
    ..Among these is transthyretin (TTR), a major carrier of thyroid hormones and retinol-binding protein (RBP)...
  39. pmc Endoplasmic reticulum quality control regulates the fate of transthyretin variants in the cell
    Takashi Sato
    Graduate School of Pharmaceutical Sciences, Kumamoto University, Kumamoto, Japan
    EMBO J 26:2501-12. 2007
    The secretion of transthyretin (TTR) variants contributes to the pathogenesis of amyloidosis because they form aggregates in the extracellular environment...
  40. doi An aberrant protein complex in CSF as a biomarker of Alzheimer disease
    M A Lovell
    Department of Chemistry, Sanders Brown Center on Aging and Alzheimer s Disease Center, University of Kentucky, Lexington, KY 40536 0230, USA
    Neurology 70:2212-8. 2008
    To determine if an aberrant protein complex consisting of prostaglandin-d-synthase (PDS) and transthyretin (TTR) in CSF differentiates between subjects with Alzheimer disease (AD) and normal control (NC) subjects.
  41. pmc Association of TTR polymorphisms with hippocampal atrophy in Alzheimer disease families
    Karen T Cuenco
    Department of Medicine, Boston University School of Medicine, 715 Albany Street, Boston, MA 02118, USA
    Neurobiol Aging 32:249-56. 2011
    In vitro and animal model studies suggest that transthyretin (TTR) inhibits the production of the amyloid β protein, a major contributor to Alzheimer disease (AD) pathogenesis...
  42. doi Serum transthyretin levels in Swedish TTR V30M carriers
    Joel Buxbaum
    Department of Molecular and Experimental Medicine, WM Keck Autoimmune Disease Center, The Scripps Research Institute, University of Umea, Umea, Sweden
    Amyloid 17:83-5. 2010
    Serum transthyretin (TTR) levels have been reported to be reduced in Portuguese and Japanese patients with TTR V30M familial amyloidotic polyneuropathy and pre-symptomatic carriers of the allele as well as in the carriers of a number of ..
  43. doi Clinical and histopathological features of familial amyloidotic polyneuropathy with transthyretin Val30Ala in a Chinese family
    Jing yao Liu
    Department of Neurology, First Hospital, Jilin University, Changchun, China
    J Neurol Sci 304:83-6. 2011
    ..FAP) is characterized by extracellular deposition of amyloid fibrils caused by a point mutation in the transthyretin (TTR) gene...
  44. doi Altered cerebrospinal fluid index of prealbumin, fibrinogen, and haptoglobin in patients with Guillain-Barré syndrome and chronic inflammatory demyelinating polyneuropathy
    H L Zhang
    Division of Neurodegeneration, Department of Neurobiology, Care Sciences and Society, Karolinska Institute, Karolinska University Hospital, Huddinge, Stockholm, Sweden
    Acta Neurol Scand 125:129-35. 2012
    ..Changes in the CSF levels of proteins other than albumin in patients with GBS and CIDP are not as well studied. If altered, aberrant levels of CSF proteins may render it possible to establish useful biomarkers for GBS and CIDP...
  45. pmc Neuronal production of transthyretin in human and murine Alzheimer's disease: is it protective?
    Xinyi Li
    Department of Molecular and Experimental Medicine, The Scripps Research Institute, La Jolla, California 92037, USA
    J Neurosci 31:12483-90. 2011
    b>Transthyretin (TTR), a systemic amyloid precursor in the human TTR amyloidoses, interacts with β-amyloid (Aβ) in vitro, inhibits Aβ fibril formation, and suppresses the Alzheimer's disease (AD) phenotype in APP23 mice bearing a human ..
  46. doi Genetic analysis of genes involved in amyloid-β degradation and clearance in Alzheimer's disease
    Teemu Natunen
    Institute of Clinical Medicine Neurology, University of Eastern Finland and Department of Neurology, Kuopio University Hospital, Kuopio, Finland
    J Alzheimers Dis 28:553-9. 2012
    ..age of onset was observed in AD patients carrying the A allele of rs723744 and the C allele of rs3794884 in transthyretin (TTR) gene...
  47. doi The relative amounts of plasma transthyretin forms in familial transthyretin amyloidosis: a quantitative analysis by Fourier transform ion-cyclotron resonance mass spectrometry
    Cristina Ribeiro-Silva
    Center of Chemistry and Biochemistry, Department of Chemistry and Biochemistry, Faculty of Sciences University of Lisbon, Campo Grande, Lisbon, Portugal
    Amyloid 18:191-9. 2011
    Familial transthyretin amyloidosis (ATTR) is a fatal autosomal dominant disease characterized by the formation of amyloid fibers, mainly composed of transthyretin (TTR)...
  48. doi [Identification of a TTR gene mutation in a family with hereditary vitreous amyloidosis]
    Yuan Xie
    Laboratory of Molecular Biology, Guiyang Medical University, Guizhou, Guiyang 550004, P R China
    Zhonghua Yi Xue Yi Chuan Xue Za Zhi 29:13-5. 2012
    ..To study the disease gene in a family with hereditary vitreous amyloidosis...
  49. pmc Rabring7 degrades c-Myc through complex formation with MM-1
    Rina Narita
    Graduate School of Agriculture, Hokkaido University, Sapporo, Japan
    PLoS ONE 7:e41891. 2012
    ..These results suggest that Rabring7 stimulates c-Myc degradation via mono-ubiquitination of MM-1...
  50. doi On the origin of the transthyretin Val30Met familial amyloid polyneuropathy
    C Zaros
    Department of Neurology, Hopital Henri Mondor, 94000 Creteil, France
    Ann Hum Genet 72:478-84. 2008
    b>Transthyretin (TTR) familial amyloid polyneuropathy is a severe autosomal dominant neuropathy of adulthood, frequently linked to the pathogenic Val30Met variant of the TTR gene...
  51. ncbi Clinical and genetic analysis of three families with familiar amyloid polyneuropathy
    Yan feng Li
    Department of Neurology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100730
    Chin Med Sci J 23:230-3. 2008
    ..To study the clinical and genetic features of familiar amyloid polyneuropathy (FAP)...
  52. ncbi Susceptibility and modifier genes in Portuguese transthyretin V30M amyloid polyneuropathy: complexity in a single-gene disease
    Miguel L Soares
    Department of Molecular and Experimental Medicine, Division of Rheumatology Research and the WM Keck Autoimmune Disease Center, The Scripps Research Institute, La Jolla, CA 92037, USA
    Hum Mol Genet 14:543-53. 2005
    Familial amyloid polyneuropathy type I is an autosomal dominant disorder caused by mutations in the transthyretin (TTR) gene; however, carriers of the same mutation exhibit variability in penetrance and clinical expression...
  53. doi [Immune, inflammatory, and nutritional protein profile in children with iron deficiency in Côte d'Ivoire]
    Houphouët Félix Yapi
    Laboratoire de pharmacodynamie biochimique, UFR Biosciences, Université de Cocody Abidjan, Abidjan 22, Cote d Ivoire
    Sante 19:25-8. 2009
    ..05. Iron deficiency was also associated with reduced levels of thyroxine-binding prealbumin (TBPA) and albumin (p < 0.05). Inflammatory proteins did not differ significantly between the two groups (p > 0.05)...
  54. ncbi [The importance of TTR met 30 in the diagnosis of familial amyloidotic polyneuropathy in the absence of a positive family history]
    M Santos
    Unidade de Neurologia e Neurofisiologia, , Portugal
    Rev Neurol 30:929-31. 2000
    ..polyneuropathy type I is an hereditary autosomal dominant disease, characterized by having a mutant transthyretin (TTR met 30)...
  55. doi Recombinant transthyretin purification and competitive binding with organohalogen compounds in two gull species (Larus argentatus and Larus hyperboreus)
    Francisco Ucán-Marin
    National Wildlife Research Centre, Carleton University, Ottawa, Ontario, K1A 0H3, Canada
    Toxicol Sci 107:440-50. 2009
    ..Such compounds can potentially perturb hormone transport via binding interactions with proteins such as transthyretin (TTR, prealbumin)...
  56. ncbi Inhibition of serum protein binding of thyroxine in a hypothyroid patient with familial dysalbuminemic hyperthyroxinemia
    B N Premachandra
    Research Service, Veterans Administration Medical Center, St Louis, MO 63125, USA
    Clin Biochem 29:85-8. 1996
    ..To investigate unusual free thyroxine (FT4) responses to T4 replacement doses in a hypothyroid patient with familial dysalbuminemic hyperthyroxinemia (FDH)...
  57. ncbi Assessing the role of ortho-substitution on polychlorinated biphenyl binding to transthyretin, a thyroxine transport protein
    K R Chauhan
    Curriculum in Toxicology, University of North Carolina, Chapel Hill, North Carolina, USA
    Toxicol Appl Pharmacol 162:10-21. 2000
    ..Previous structure-activity relationship (SAR) studies on second messengers and transthyretin (TTR; prealbumin) binding focused little attention on the ortho-substituted PCBs...
  58. ncbi Evolution of the thyroid hormone-binding protein, transthyretin
    D M Power
    Centro de Ciências de MAR CCMAR, Universidade do Algarve, Campus de Gambelas, 8000 Faro, Portugal
    Gen Comp Endocrinol 119:241-55. 2000
    b>Transthyretin (TTR) belongs to a group of proteins, which includes thyroxine-binding globulin and albumin, that bind to and transport thyroid hormones in the blood...
  59. ncbi Impact of varying levels of protein intake on protein status indicators after gastric bypass in patients with multiple complications requiring nutritional support
    Elizabeth Rinaldi Schinkel
    Department of Medical Nutrition Therapy, Poudre Valley Hospital, Fort Collins, CO 80524, and Department of Public Health and Preventive Medicine, Oregon Health and Science University, Portland, OR, USA
    Obes Surg 16:24-30. 2006
    ..This study assesses the effect of varying protein-calorie intake in complicated patients after RYGBP on two markers of protein status: thyroxine-binding prealbumin (TBPA) and serum albumin.
  60. ncbi Binding activities of thyroxine binding globulin versus thyroxine binding prealbumin in rat sera: differential modulation by thyroid hormone ligands, oleic acid and pharmacological drugs
    L Savu
    U 224, INSERM, Faculte de Medecine Xavier Bichat, Paris, France
    Biochem Biophys Res Commun 159:919-26. 1989
    ..and electrophoretic techniques to compare the binding properties of thyroxine binding globulin and thyroxine binding prealbumin in rat sera...
  61. ncbi Transthyretin from discovery to now
    Jacob Robbins
    Genetics and Biochemistry Branch, National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, Bethesda, Maryland 20892, USA
    Clin Chem Lab Med 40:1183-90. 2002
    As introduction to the First International Congress on Transthyretin in Health and Disease, this lecture traces the origin of the subjectfrom the discovery in the 1950s that a serum protein migrating ahead of albumin in an electrical ..
  62. ncbi Cloning of putative piscine (Sparus aurata) transthyretin: developmental expression and tissue distribution
    B Funkenstein
    Israel Oceanographic and Limnological Research, National Institute of Oceanography, Tel Shikmona, Haifa, Israel
    Mol Cell Endocrinol 157:67-73. 1999
    cDNA encoding putative transthyretin (prealbumin, TTR) was cloned from liver of the marine fish Sparus aurata. The cDNA contains an open reading frame of 453 nt, encoding for a TTR precursor of 151 amino acids...
  63. ncbi Observations with regard to the National Kidney Foundation K/DOQI clinical practice guidelines concerning serum transthyretin in chronic renal failure
    Joel D Kopple
    Division of Nephrology and Hypertension and the Research and Education Institute at Harbor UCLA Medical Center, Torrance, CA 90502, USA
    Clin Chem Lab Med 40:1308-12. 2002
    ..Prealbumin, also known as serum transthyretin (TTR), was not recommended as a nutritional parameter of the same usefulness as the serum albumin...
  64. ncbi Electrically neutral microheterogeneity of human plasma transthyretin (prealbumin) detected by isoelectric focusing in urea gradients
    K Altland
    Institut fur Humangenetik, Justus Liebig Universitat, Giessen, Germany
    Electrophoresis 20:1349-64. 1999
    Mutants of the human plasma transthyretin (TTR, prealbumin) have attracted interest due to their rather frequent association with the autosomal dominant disease familial amyloidotic polyneuropathy (FAP)...
  65. ncbi [Fibril-forming proteins: the amyloidosis. New hopes for a disease that cardiologists must know]
    Eloisa Arbustini
    Laboratorio di Diagnostica Molecolare, Area Trapiantologica di Ricerca, IRCCS Policlinico San Matteo, Dipartimento di Biochimica, Universita degli Studi, Pavia
    Ital Heart J Suppl 3:590-7. 2002
    ..In transthyretin (TTR) amyloidosis, the non-senile forms are autosomal dominant diseases caused by defective proteins ..
  66. ncbi Cutaneous lymphatic amyloid deposits in "Hungarian-type" familial transthyretin amyloidosis: a case report
    Tibor Harkany
    Department of Molecular Neurobiology, University of Groningen, PO Box 14, Kerklaan 30, NL 9750 AA Haren, The Netherlands
    Br J Dermatol 146:674-9. 2002
    Multiple transthyretin (TTR) mutations have recently been identified and implicated in the development of familial systemic amyloidoses, but early diagnosis of these disorders is still largely unresolved...
  67. doi Chain reaction of amyloid fibril formation with induction of basement membrane in familial amyloidotic polyneuropathy
    Yohei Misumi
    Department of Neurology, Graduate School of Medical Sciences, Kumamoto University, Kumamoto 860 081, Japan
    J Pathol 219:481-90. 2009
    ..FAP) is characterized by extracellular deposition of amyloid fibrils caused by a point mutation in the transthyretin (TTR) gene...
  68. pmc Matrix metalloproteinases and their tissue inhibitors in cardiac amyloidosis: relationship to structural, functional myocardial changes and to light chain amyloid deposition
    Andreia Biolo
    Whitaker Cardiovascular Institute, Boston University School of Medicine, 715 Albany Street, Boston, MA 02118, USA
    Circ Heart Fail 1:249-57. 2008
    ..prognosis compared with non-light chain cardiac amyloidoses (ie, forms associated with wild-type or mutated transthyretin [TTR])...
  69. ncbi Risk of transmission of systemic transthyretin amyloidosis after domino liver transplantation
    Laura Llado
    Departments of Surgery, Liver Transplant Unit, Hospital Universitari de Bellvitge, IDIBELL, Barcelona, Spain
    Liver Transpl 16:1386-92. 2010
    Recent reports of the transmission of systemic transthyretin (TTR) amyloidosis after domino liver transplantation (DLT) using grafts from patients with familial amyloid polyneuropathy (FAP) have raised concerns about the procedure...
  70. doi Risk of transmission of systemic transthyretin amyloidosis after domino liver transplantation
    Laura Llado
    Departments of Surgery, Liver Transplant Unit, Hospital Universitari de Bellvitge, IDIBELL, Barcelona, Spain
    Liver Transpl 16:1386-92. 2010
    Recent reports of the transmission of systemic transthyretin (TTR) amyloidosis after domino liver transplantation (DLT) using grafts from patients with familial amyloid polyneuropathy (FAP) have raised concerns about the procedure...
  71. ncbi Pathogenesis of and therapeutic strategies to ameliorate the transthyretin amyloidoses
    Yoshiki Sekijima
    Department of Neurology, Shinshu University School of Medicine, 3 1 1 Asahi, Matsumoto 390 8621, Japan
    Curr Pharm Des 14:3219-30. 2008
    b>Transthyretin (TTR) is a homotetrameric serum and cerebrospinal fluid protein that transports both thyroxine (T(4)) and the retinol-retinol binding protein complex (holoRBP)...
  72. ncbi Liver transplantation for hereditary transthyretin amyloidosis
    O B Suhr
    Department of Medicine, Umea University Hospital, Umea, Sweden
    Liver Transpl 6:263-76. 2000
    b>Transthyretin (TTR) amyloidosis is the most common form of hereditary amyloidosis...
  73. ncbi Amyloid and nonfibrillar deposits in mice transgenic for wild-type human transthyretin: a possible model for senile systemic amyloidosis
    M H Teng
    Research Service, New York Department of Veterans Affairs Medical Center, New York, USA
    Lab Invest 81:385-96. 2001
    The human serum protein transthyretin (TTR) is highly fibrillogenic in vitro and is the fibril precursor in both autosomal dominant (familial amyloidotic polyneuropathy [FAP] and familial amyloidotic cardiomyopathy [FAC]) and sporadic (..
  74. ncbi Familial ATTR amyloidosis: microalbuminuria as a predictor of symptomatic disease and clinical nephropathy
    Luísa Lobato
    Department of Nephrology and Centro de Estudos de Paramiloidose, Hospital Geral de Santo António and Institute for Molecular and Cell Biology, Porto, Portugal
    Nephrol Dial Transplant 18:532-8. 2003
    Portuguese type familial amyloid polyneuropathy (FAP) is a neuropathic amyloidosis caused by a mutant transthyretin (TTR). Varying degrees of renal involvement have been reported...
  75. ncbi Sequence, genetic analysis, and expression of Actinobacillus pleuropneumoniae transferrin receptor genes
    G C Gonzalez
    Department of Microbiology and Infectious Diseases, University of Calgary, AB, Canada
    Microbiology 141:2405-16. 1995
    The tbpA and tbpB genes encoding the transferrin receptor proteins Tbp1 and Tbp2 from a serotype 7 strain of Actinobacillus pleuropneumoniae were cloned, sequenced, and expressed in Escherichia coli...
  76. ncbi A dynamic model of the meningococcal transferrin receptor
    I C Boulton
    Centre for Applied Microbiology and Research, Salisbury, SP4 0JG, UK
    J Theor Biol 198:497-505. 1999
    ..It has been established that transferrin-binding proteins (designated TbpA and TbpB) form a discrete, specific complex which may be composed of a transmembrane species (composed of the TbpA ..
  77. pmc Transferrin-binding protein B isolated from Neisseria meningitidis discriminates between apo and diferric human transferrin
    I C Boulton
    Metalloprotein Research Group, Division of Biochemistry and Molecular Biology, United Medical and Dental School of Guy s and St Thomas s Hospitals, Guy s Hospital, London SE1 9RT
    Biochem J 334:269-73. 1998
    ..To characterize this process, functionally active meningococcal transferrin-binding protein A (TbpA) and TbpB have been purified from N. meningitidis using a novel purification procedure...
  78. ncbi Thyroid function in sojourners and acclimatised low landers at high altitude in man
    R C Sawhney
    Defence Institute of Physiology and Allied Sciences, Delhi Cantonment, India
    Horm Metab Res 23:81-4. 1991
    ..01) at HA, whereas plasma rT3, TBG and T4 binding capacities of TBG and TBPA did not show any appreciable change...
  79. pmc Neisseria meningitidis expressing transferrin binding proteins of Actinobacillus pleuropneumoniae can utilize porcine transferrin for growth
    D J Litt
    Institute of Infections and Immunity, Queen s Medical Centre, Nottingham, NG7 2UH, United Kingdom
    Infect Immun 68:550-7. 2000
    ..or porcine transferrin because of loss of both transferrin binding proteins (Tbp) A and B [strain B16B6(Str(r))/tbpA(-)B(-)] and (ii) an ability to bind porcine transferrin but not human transferrin [strain B16B6(Str(r))/tbpA(ap)B(..
  80. doi Low serum levels of prohepcidin, but not hepcidin-25, are related to anemia in familial amyloidosis TTR V30M
    Idalina Beirão
    Department of Nephrology, HGSA, Santo Antonio General Hospital, Porto, Portugal
    Blood Cells Mol Dis 41:175-8. 2008
    ..In general, the decreased observed/expected EPO levels in FAP-I correlated with the prohepcidin levels, therefore raising the possibility that a common defect in these two hormones may be somehow involved in the genesis of the disease...
  81. pmc Evidence for a common gene pool and frequent recombinational exchange of the tbpBA operon in Mannheimia haemolytica, Mannheimia glucosida and Bibersteinia trehalosi
    Inkyoung Lee
    Institute of Infection, Immunity and Inflammation, College of Medical, Veterinary and Life Sciences, Sir Graeme Davies Building, 120 University Place, University of Glasgow, Glasgow G12 8TA, UK
    Microbiology 157:123-35. 2011
    ..A total of 27 tbpB and 20 tbpA alleles were identified whilst the tbpBA operon was represented by 28 unique alleles that could be assigned to ..
  82. pmc TTR familial amyloid polyneuropathy: does a mitochondrial polymorphism entirely explain the parent-of-origin difference in penetrance?
    Bernard Bonaïti
    INRA GABI, Jouy en Josas, France
    Eur J Hum Genet 18:948-52. 2010
    The Val30Met transthyretin familial amyloid polyneuropathy (TTR-V30M-FAP) is the most frequent familial amyloidosis, with autosomal dominant transmission. This severe disease shows important differences in age of onset and penetrance...
  83. pmc Inhibition by lead of production and secretion of transthyretin in the choroid plexus: its relation to thyroxine transport at blood-CSF barrier
    W Zheng
    School of Public Health, Department of Pharmacology, College of Physicians and Surgeons, Columbia University, New York, NY 10032, USA
    Toxicol Appl Pharmacol 155:24-31. 1999
    Long-term, low-dose Pb exposure in rats is associated with a significant decrease in transthyretin (TTR) concentrations in the CSF...
  84. pmc Identification of TbpA residues required for transferrin-iron utilization by Neisseria gonorrhoeae
    Jennifer M Noto
    Department of Microbiology and Immunology, Virginia Commonwealth University Medical Center, Richmond, VA 23298 0678, USA
    Infect Immun 76:1960-9. 2008
    ..The gonococcal transferrin-iron uptake system is composed of two transferrin binding proteins, TbpA and TbpB...
  85. ncbi Detection and subtyping of Actinobacillus pleuropneumoniae strains by PCR-RFLP analysis of the tbpA and tbpB genes
    V A de la Puente Redondo
    Section of Microbiology and Immunology, Department of Animal Health, Faculty of Veterinary Medicine, , Spain
    Res Microbiol 151:669-81. 2000
    ..The A. pleuropneumoniae tbpA and tbpB genes were used as targets for amplification of DNA fragments, with a pair of specific primers for each ..
  86. ncbi Thyroxine binding in a TTR Met 119 kindred
    I L Alves
    Centro de Estudos de Paramiloidose, Hospital de Santo Antonio, Porto, Portugal
    J Clin Endocrinol Metab 77:484-8. 1993
    Recently, a transthyretin variant, TTR Met 119, in which methionine substitutes for threonine 119, a component of the protein's iodothyronine binding site, was identified in individuals with transient euthyroid hyperthyroxinemia...
  87. pmc Transthyretin, thyroxine, and retinol-binding protein in human cerebrospinal fluid: effect of lead exposure
    W Zheng
    Division of Environmental Health Sciences, School of Public Health, Columbia University, New York, New York 10032, USA
    Toxicol Sci 61:107-14. 2001
    b>Transthyretin (TTR), synthesized by the choroid plexus, is proposed to have a role in transport of thyroid hormones in the brain...
  88. ncbi Secondary glaucoma in patients with familial amyloidotic polyneuropathy
    Akira Kimura
    Department of Ophthalmology, Kumamoto University School of Medicine, 1 1 1 Honjo, Kumamoto 860 8556, Japan
    Arch Ophthalmol 121:351-6. 2003
    To elucidate the clinical features and surgical outcomes of the treatment of secondary glaucoma associated with transthyretin (TTR)-related familial amyloidotic polyneuropathy (FAP).
  89. ncbi A new transthyretin variant (Glu61Gly) associated with cardiomyopathy
    Michael Rosenzweig
    Amyloid Treatment and Research Program, Boston University School of Medicine, MA 02118, USA
    Amyloid 14:65-71. 2007
    We report the identification of a new transthyretin (TTR) gene mutation and variant protein, Glu61Gly, in a 55-year-old man with progressive cardiomyopathy, mild peripheral neuropathy and bilateral carpal tunnel syndrome...
  90. pmc Gonococcal genes encoding transferrin-binding proteins A and B are arranged in a bicistronic operon but are subject to differential expression
    C Ronpirin
    Department of Microbiology and Immunology, Medical College of Virginia, Virginia Commonwealth University, Richmond, Virginia 23298 0678, USA
    Infect Immun 69:6336-47. 2001
    ..The receptor involved in transferrin iron acquisition is composed of two distinct transferrin-binding proteins, TbpA and TbpB...
  91. ncbi The evolutionary and integrative roles of transthyretin in thyroid hormone homeostasis
    G Schreiber
    Department of Biochemistry and Molecular Biology, The University of Melbourne, Parkville, 3010 Victoria, Australia
    J Endocrinol 175:61-73. 2002
    In larger mammals, thyroid hormone-binding plasma proteins are albumin, transthyretin (TTR) and thyroxine (T4)-binding globulin. They differ characteristically in affinities and release rates for T4 and triiodothyronine (T3)...
  92. ncbi Differential binding of apo and holo human transferrin to meningococci and co-localisation of the transferrin-binding proteins (TbpA and TbpB)
    N B Powell
    Microbial Pathogenicity Research Group, Institute of Infections and Immunity, Queen s Medical Centre, Nottingham
    J Med Microbiol 47:257-64. 1998
    ..001). The ability of meningococcal mutants lacking either transferrin-binding protein A (TbpA) or TbpB to discriminate between apo-hTf and holo-hTf was also investigated...
  93. pmc Purified meningococcal transferrin-binding protein B interacts with a secondary, strain-specific, binding site in the N-terminal lobe of human transferrin
    I C Boulton
    Metalloprotein Research Group, Division of Biomolecular Sciences, King s College London, Guy s Campus, Guy s Hospital, London SE1 9RT, UK
    Biochem J 339:143-9. 1999
    Neisseria meningitidis, grown in iron-limited conditions, produces two transferrin-binding proteins (TbpA and TbpB) that independently and specifically bind human serum transferrin (hTF) but not bovine serum transferrin (bTF)...
  94. pmc Demonstration and characterization of a specific interaction between gonococcal transferrin binding protein A and TonB
    Christopher D Kenney
    Department of Pathology, Medical College of Virginia Campus, Virginia Commonwealth University, Richmond 23298 0678, USA
    J Bacteriol 184:6138-45. 2002
    ..Efficient transferrin-iron acquisition is dependent on the combined action of two proteins, designated TbpA and TbpB...
  95. pmc tert-Butylphenylacetylene is a potent mechanism-based inactivator of cytochrome P450 2B4: inhibition of cytochrome P450 catalysis by steric hindrance
    Haoming Zhang
    Department of Pharmacology, The University of Michigan, Ann Arbor, MI 48109 5632, USA
    Mol Pharmacol 76:1011-8. 2009
    We have demonstrated that 4-(tert-butyl)-phenylacetylene (tBPA) is a potent mechanism-based inactivator for cytochrome P450 2B4 (P450 2B4) in the reconstituted system...
  96. doi Structure-based investigation on the binding interaction of hydroxylated polybrominated diphenyl ethers with thyroxine transport proteins
    Jie Cao
    State Key Laboratory of Environmental Chemistry and Ecotoxicology, Research Center for Eco Environmental Sciences, Chinese Academy of Sciences, P O Box 2871, Beijing 100085, China
    Toxicology 277:20-8. 2010
    ..Binding affinity of the 14 OH-PBDEs with transthyretin (TTR) and thyroxine-binding globulin (TBG) was measured by competitive fluorescence displacement assay...
  97. ncbi Doxycycline disrupts transthyretin amyloid: evidence from studies in a FAP transgenic mice model
    I Cardoso
    Molecular Neurobiology, IBMC, Porto University, Porto, Portugal
    FASEB J 20:234-9. 2006
    ..polyneuropathy is an autosomal dominant disorder mainly characterized by the extracellular deposition of transthyretin, with special involvement of the peripheral nerve...
  98. ncbi Determination of a cut-off value for the molar ratio of retinol-binding protein to transthyretin (RBP:TTR) in Bangladeshi patients with low hepatic vitamin A stores
    Francisco J Rosales
    Nutrition Department, The Pennsylvania State University, University Park, USA
    J Nutr 132:3687-92. 2002
    The purpose of this study was to determine a cut-off value of the molar ratio of retinol-binding protein to transthyretin (RBP:TTR) to indicate marginal vitamin A (VA) deficiency...
  99. ncbi Molecular cloning, tissue distribution, and developmental expression of lamprey transthyretins
    Richard G Manzon
    Department of Biology, University of Regina, 3737 Wascana Parkway, Regina, Sask, Canada S4S 0A2
    Gen Comp Endocrinol 151:55-65. 2007
    We isolated and cloned full-length cDNAs of transthyretin (TTR) from 2 genera of lamprey, Petromyzon marinus and Lampetra appendix. These sequences represent the first report of TTR sequences in vertebrates basal to teleost fishes...
  100. ncbi Homology modelling of transferrin-binding protein A from Neisseria meningitidis
    Jonathan S Oakhill
    Metalloprotein Research Group, Randall Division of Cell and Molecular Biophysics, King s College London, New Hunt s House, Guy s Campus, London SE1 1UL, UK
    Protein Eng Des Sel 18:221-8. 2005
    ..meningitis, obtains transferrin-bound iron by expressing two outer membrane located transferrin-binding proteins, TbpA and TbpB. TbpA is thought to be an integral outer membrane pore that facilitates iron uptake...
  101. ncbi ApoA-I cleaved by transthyretin has reduced ability to promote cholesterol efflux and increased amyloidogenicity
    Márcia Almeida Liz
    Molecular Neurobiology Group, Instituto de Biologia Molecular e Celular IBMC, Porto, Portugal
    J Lipid Res 48:2385-95. 2007
    A fraction of plasma transthyretin (TTR) circulates in HDL through binding to apolipoprotein A-I (apoA-I). Moreover, TTR is able to cleave the C terminus of lipid-free apoA-I...

Research Grants64

  1. BIOSYNTHETIC ACTIVITY OF THE CHOROID PLEXUS
    Joseph Herbert; Fiscal Year: 1993
    ..We (and others) have demonstrated the specific and abundant synthesis of transthyretin (TTR, prealbumin), a transport protein for thyroxine and retinol, within the CP epithelium...
  2. GENE EXPRESSION IN FAMILIAL AND SENILE CARDIAC AMYLOID
    Joel Buxbaum; Fiscal Year: 1993
    ..Systemic (cardiac) Amyloidosis have been shown to be caused by the deposition of thyroxine-binding prealbumin (transthyretin)-related molecules in various tissues, notably peripheral and autonomic nerves, heart, gastrointestinal tract, ..
  3. THYROID HORMONE ANALOGS
    Manfred Wolff; Fiscal Year: 1980
    ..vitro tests include measurement of relative binding affinities to the plasma protein, thyroxine-binding prealbumin (TBPA), and to hormone receptor preparations from rat liver cell nuclei...
  4. Novel Insights into the Pathogenesis of Light Chain Cardiac Amyloidosis
    Flora Sam; Fiscal Year: 2011
    ..e. amyloidosis associated with wild-type transthyretin (TTR): the senile systemic amyloidosis (SSA) or a mutant transthyretin (ATTR);the prognosis for patients with ..
  5. Molecular mechanism of senile cardiac amyloidosis
    Lawreen H Connors; Fiscal Year: 2013
    ..In SSA, wild-type transthyretin (TTR), normally a serum protein, forms amyloid deposits which disrupt normal cardiac cell functioning...
  6. Lipid acceleration of transthyretin amyloidosis
    Irit Rappley; Fiscal Year: 2012
    DESCRIPTION (provided by applicant): The transthyretin (TTR) amyloidoses, including senile systemic amyloidosis, familial amyloid cariomyopathy, and familial amyloid polyneuropathy, are characterized by extracellular amyloid deposits ..
  7. Natalia Reixach; Fiscal Year: 2014
    DESCRIPTION (provided by applicant): The transthyretin (TTR) amyloidoses are fatal disorders of secondary protein structure characterized by extracellular deposition of aggregates, including amyloid fibrils, derived from the secreted ..
  8. Regina M Murphy; Fiscal Year: 2016
    ..Recent studies have uncovered a possible explanation: APPSw mice upregulate synthesis of transthyretin (TTR), a transport protein found in plasma and cerebrospinal fluid, and TTR appears to protect the mice from ..
  9. Cardiac Amyloidosis in Aging African American
    Joel N Buxbaum; Fiscal Year: 2012
    ..form of late-onset amyloidosis due to a substitution of ILE for VAL at position 122 in the serum protein transthyretin (TTR) contributes to this racial disparity...
  10. INHIBITION OF AMYLOID FORMATION BY TRANSTHYRETIN & APOE
    Dmitry Goldgaber; Fiscal Year: 1999
    ..Recently, we found that two Ab binding proteins, transthyretin (TTR) and apolipoprotein E (apoE), inhibit Ab amyloid formation in vitro...
  11. Mechanistic studies of transthyretin misfolding and amyloid formation through a c
    Kwang Hun Lim; Fiscal Year: 2013
    ..This research program is aimed at investigating amyloid formation mechanisms of a natively folded protein, transthyretin (TTR), using both solution and solid-state NMR...
  12. Cardiotrophin-Like Cytokine 1, a Candidate Molecule for the FSGS Factor
    Virginia J Savin; Fiscal Year: 2010
    ..We have documented that serum or plasma from FSGS patients increases glomerular albumin permeability (Palb) and that the specific activity of certain protein fractions of FSGS plasma is more than 10,000-fold greater than ..
  13. Early Disease Biomarkers of PCB-exposed Human Population
    Sisir K Dutta; Fiscal Year: 2010
    ..Fourth, high affinity binding of hydroxylated PCBs to Transthyretin (TTR) results in selective delivery of these hormones over the blood-brain barrier and over the placenta to ..
  14. Patient-Level Determinants of Oral Anticoagulation Control in the VHA
    Adam J Rose; Fiscal Year: 2013
    ..Finally, we will develop and pilot a tool to communicate our recommendations for quality improvement to each site. This tool will eventually serve as the basis for a project to improve anticoagulation care throughout the VHA. ..
  15. Effect of diflunisal (IND68092) on familial amyloidosis
    JOHN BERK; Fiscal Year: 2009
    Familial amyloidotic polyneuropathy (FAP) is a lethal autosomal dominant disease mediated by misfolding of transthyretin, a transport protein produced by the liver...
  16. Effect of NF1 mutation on choroid plexus function
    Sally Temple; Fiscal Year: 2013
    ..We will generate an inducible conditional knockout by crossing the Transthyretin-cre-ERT mouse line Tg(Ttr- cre/Esr1*)1Vco which expresses cre specifically in the CP from early times in ..
  17. Exercise and energy balance: role of hypothalamic transthyretin
    Sheng Bi; Fiscal Year: 2013
    ..These analyses have shown that transthyretin (TTR) gene expression is induced in the dorsomedial hypothalamus (DMH) of exercised rats...
  18. The structure of telomerase RNA during catalysis
    DAUD COLE; Fiscal Year: 2012
    ..SHAPE technology will then be used to reveal the secondary structure of tTR during these processes. ..
  19. Konstantin Petrukhin; Fiscal Year: 2015
    ..Formation of the tertiary retinol-binding protein 4 (RBP4)-transthyretin (TTR)-retinol complex in serum is required for retinol uptake from circulation to the RPE...
  20. Commercialization of a Diagnostic Test for Alzheimer's Disease
    MARK ANTHONY LOVELL; Fiscal Year: 2011
    ..complex consisting of 1 molecule of lipocalin (brain-specific) prostaglandin-d-synthase (PDS) and 1 dimer of transthyretin (TTR) quantified in cerebrospinal fluid (CSF) using an enzyme linked immunoassay (ELISA) developed and ..
  21. Development of a Plasma Biomarker of Alzheimer's Disease
    MARK ANTHONY LOVELL; Fiscal Year: 2012
    ..aberrant protein/protein complex consisting or lipocalin (brain-specific) prostaglandin-d- synthase (PDS) and transthyretin (TTR) are decreased early in disease progression and that baseline (pre-conversion) PDS/TTR levels are ..
  22. Human Corneal Model for Ocular Irritation Assay
    Yulia Kaluzhny; Fiscal Year: 2013
    ..Due to these enhanced capabilities, the CFT will provide toxicologists and scientists with a tool that will dramatically decrease, if not entirely eliminate, the need for animal testing to determine ocular irritancy. ..
  23. Lisa Marie Guay-Woodford; Fiscal Year: 2014
    ..abstract_text> ..
  24. Lisa Marie Guay-Woodford; Fiscal Year: 2015
    ....
  25. Development of the HTS assay for visual cycle inhibitors
    Konstantin Petrukhin; Fiscal Year: 2009
    ..Formation of the tertiary retinol-binding protein (RBP)- transthyretin (TTR)-retinol complex is required for retinol uptake from serum to the RPE...
  26. Richard J Roman; Fiscal Year: 2014
    ..TGF-beta also directly increased the permeability of glomeruli to albumin (Palb) and inhibited the glomerular formation of 20-HETE...
  27. Wendee M Wechsberg; Fiscal Year: 2015
    ....
  28. Indiana Clinical and Translational Science Institute (UL1)
    Anantha Shekhar; Fiscal Year: 2011
    ..V. Leverage the Resources of the Greater Indiana Community by connecting to a broad array of resources from multiple partner institutions throughout the state to realize the full potential of the Indiana CTSI. ..
  29. Indiana Clinical and Translational Science Institute (UL1)
    Anantha Shekhar; Fiscal Year: 2013
    ..V. Leverage the Resources of the Greater Indiana Community by connecting to a broad array of resources from multiple partner institutions throughout the state to realize the full potential of the Indiana CTSI...
  30. Stimulators of Neuronal Transthyretin (TTR) Transcription as Alzheimer's Therapy
    Joel N Buxbaum; Fiscal Year: 2012
    DESCRIPTION (provided by applicant): We have shown that over-expression of a wild type human transthyretin (TTR) gene can suppress the neuropathologic and behavioral phenotypes of a well-studied validated transgenic model of human ..
  31. Clinical and Translational Science Institute at Children's National
    Mendel Tuchman; Fiscal Year: 2011
    ..Translational Technologies and Resources (TTR) supports a diverse group of 15 core functions. Tracking and Evaluation (TE): CTSI-CN activities will be guided and modified by a thoughtful and detailed evaluation plan. ..
  32. CNS DEVELOPMENT UNDER HYPERGRAVITY : MODULATION BY TH?
    ELIZABETH SAJDEL SULKOWSKA; Fiscal Year: 2004
    ..These studies will aid in developing an understanding of how altered gravity affects CNS development, what role the thyroid hormone plays in that response, and whether this response can be modulated by hormonal therapy. ..
  33. ETIOLOGIC FACTOR IN FOCAL GLOMERULOSCLEROSIS
    Virginia Savin; Fiscal Year: 2000
    ..therapy, and our observation that plasma from FSGS patients increases glomerular albumin permeability (Palb) following in vitro incubation of isolated glomeruli to serum or plasma of certain FSGS patients...
  34. Testicular Torsion: Mechanisms and Treatment
    Terry Turner; Fiscal Year: 2005
    ..It is likely that these investigations will be relevant to other forms of oxidative stress inducing testicular dysfunction. ..
  35. Retargeting HSV-1 Vectors to Motor Neurons
    Joseph Glorioso; Fiscal Year: 2005
    ..The soluble HveC adapter will be expected to block gD binding to cellular HveC while directly or indirectly re-targeting the virus to TTx receptors. ..
  36. Temporal Dynamics
    JOHN STADDON; Fiscal Year: 2006
    ..We believe that these studies may lead to an entirely new unified approach to choice. ..
  37. Abcb11 and Hepatic Transport
    Anne Henkel; Fiscal Year: 2009
    ..We will characterize the role of bile salts in regulating gene and protein expression in the TTR-Abcb11 model as a means to establish the mechanism by which Abcbl 1 predisposes to cholelithiasis and hepatic steatosis. ..
  38. BIOSYNTHESIS OF VITAMIN B12 AND ANAEROBIC METABOLISM
    John Roth; Fiscal Year: 2004
    ..coli. Understanding this metabolism may contribute to understanding the natural lifestyle of Salmonellae. ..
  39. A MOLECULAR STUDY OF THE GONOCOCCAL TRANSFERRIN RECEPTOR
    Cynthia Nau Cornelissen; Fiscal Year: 2010
    Two transferrin-binding proteins, TbpA and TbpB are components of a surface-exposed, human transferrin receptor expressed by Neisseria gonorrhoeae...
  40. RETINOL BINDING PROTEIN AND HEART DEVELOPMENT
    John Lough; Fiscal Year: 2003
    ..We have recently determined that endoderm strongly expresses the vitamin A transport proteins transthyretin (TTR) and retinol binding protein (RBP)...
  41. Estrogen Regulated Genes and Neural Protection
    Cheryl Sisk; Fiscal Year: 2001
    ..These genes encode transthyretin precursor (TTR), presenilin 1 (PS1), and presenilin 2 (PS2)...
  42. Neuroprotective Drugs for Alzheimer's Disease
    TREVOR TWOSE; Fiscal Year: 2006
    ..Protection results from the coordinated expression of transthyretin (TTR), insulin-like growth factor-2 (IGF-2) and other growth factor-related genes...
  43. Placebo Cntrld db PH.ll/lll stdy Fx-1006A trtmnt fam Amyloid Polyneuropathy
    JEFFREY PACKMAN; Fiscal Year: 2009
    ..Deposition of amyloid fibrils of variant transthyretin (TTR) (primarily V30M) in peripheral nerve tissue produces the condition called FAP...
  44. Neuroprotection by Ginkgo biloba Extract EGB761
    JONATHAN KERN; Fiscal Year: 2004
    ..indicates that Ginkgo biloba extract EGb761 activates the antioxidant response element (ARE) and also induces transthyretin (TTR) expression...
  45. METABOLISM AND NUTRITION
    WILLIAM BLANER; Fiscal Year: 1992
    ..pathways and processes involved and their regulation; (5) the tissue sites of catabolism of RBP and transthyretin (TTR); (6) cell surface receptors for RBP; (7) the functional domains of RBP by means of monoclonal antibodies ..
  46. METABOLISM AND NUTRITION
    DE WITT GOODMAN; Fiscal Year: 1991
    ..pathways and processes involved and their regulation; (5) the tissue sites of catabolism of RBP and transthyretin (TTR); (6) cell surface receptors for RBP; (7) the functional domains of RBP by means of monoclonal antibodies ..
  47. A Neuropeptide Gene in Psychiatric Illness
    Wade Berrettini; Fiscal Year: 2004
    ..We have assembled sets (n>600) of parent-child trios for both SZ and BPD to test the hypothesis that variation in this gene increases risks for these disorders. These studies may identify a susceptibility gene for SZ and/or BPD. ..
  48. GENOME TARGETED INHIBITORS OF RETROVIRUSES
    VIRENDRA PANDEY; Fiscal Year: 2007
    ..Do _t use suffixes such as 3a, 3b, ..
  49. PTSD and Drug Dependence: Neuroimaging of Reward Circui*
    Igor Elman; Fiscal Year: 2007
    ..Number of publications and manuscdpts accepted for publication (not to exceed 10) Other items (list): Appendix I Color Plates of Figures in Preliminary Studies PHS 398 (Rev. 05/01) Page 3 Form Page 3 ..
  50. 6th International Symposium on FAP Disorders and 5th...
    Joel Buxbaum; Fiscal Year: 2006
    ..Sixth International Workshop on The Familial Amyloidotic Polyneuropathies and Cardiomyopathies and Other Transthyretin Related Disorders and the Fifth International Workshop of Liver Transplantation in Familial Amyloid ..
  51. PATHOGENESIS OF HEREDITARY TRANSTHYRETIN AMYLOIDOSIS
    Merrill Benson; Fiscal Year: 2003
    ..abstract): The overall objective of this proposal is to define the pathophysiology of the autosomal dominant transthyretin amyloidoses...
  52. TRANSTHYRETIN DEPOSITION IN AGING TRANSGENIC MICE
    Joel Buxbaum; Fiscal Year: 2004
    ..Islet Amyloid Polypeptide), Alzheimer's Disease (AbetaPP), Aortic Amyloid (ApoA1) and Senile Cardiac Amyloid (Transthyretin), occur with increasing frequency with increasing age...
  53. Simulations on the early events of TTR amyloidogenesis
    Shuanghong Huo; Fiscal Year: 2005
    ..In this proposal, we focus on the study of early events along the pathway of human transthyretin (TTR) amyloid formation using computational approaches at the atomic level...
  54. Mechanism of Amyloid Formation by Transthyretin
    AMY HURSHMAN; Fiscal Year: 2004
    b>Transthyretin (TTR) is a carrier protein that is involved in the binding and transport of thyroid hormone and the retinol binding protein/vitamin A complex...
  55. Cytokines and Symptoms in Lung Cancer treatment
    Xin Wang; Fiscal Year: 2006
    ..These pilot studies may lead to additional multi-disciplinary animal and clinical studies of the mechanisms underlying disease-related and treatment-related cancer symptoms. ..
  56. Development of SPR/MS protein array platform
    Dobrin Nedelkov; Fiscal Year: 2005
    ....
  57. Development of SPR/MS protein array platform
    Dobrin Nedelkov; Fiscal Year: 2007
    ..abstract_text> ..
  58. Affinity-based Mass Spectrometry Protein Assays
    Dobrin Nedelkov; Fiscal Year: 2004
    ..abstract_text> ..
  59. A KINETIC STUDY OF INFLAMMATION-INDUCED HYPORETINEMIA
    Francisco Rosales; Fiscal Year: 2005
    ..Previously, it has been demonstrated that the synthesis of hepatic retinol-binding protein (RBP) and transthyretin (TTR) are reduced during acute inflammation causing a reduction of plasma retinol concentrations (..
  60. DETERMINANTS OF CLINICAL OUTCOME/TOXICITY IN HISPANICS
    HEINZ LENZ; Fiscal Year: 2002
    ....
  61. T cell modulation of renal ischemia reperfusion injury.
    Hamid Rabb; Fiscal Year: 2009
    ..The long term goal will be to identify the antigens and molecular pathways that fuel the injury processes in renal IRI, and develop targeted immune therapies for ARF. ..