K P Campbell

Summary

Affiliation: University of Iowa
Country: USA

Publications

  1. pmc Variable disease severity in Saudi Arabian and Sudanese families with c.3924 + 2 T > C mutation of LAMA2
    Claudia Di Blasi
    Division of Neuromuscular Diseases and Neuroimmunology, Fondazione IRCCS Istituto Neurologico C, Besta, Milan, Italy
    BMC Res Notes 4:534. 2011
  2. pmc Dystrophin deficiency exacerbates skeletal muscle pathology in dysferlin-null mice
    Renzhi Han
    Department of Molecular Physiology and Biophysics, Howard Hughes Medical Institute, Roy J and Lucille A Carver College of Medicine, The University of Iowa, 285 Newton Road, 4283 CBRB, Iowa City, IA 52242, USA
    Skelet Muscle 1:35. 2011
  3. pmc Proteomic analysis of plasma membrane and secretory vesicles from human neutrophils
    Deepa Jethwaney
    Buck Institute for Age Research, Novato, CA 94945, USA
    Proteome Sci 5:12. 2007
  4. ncbi request reprint Skeletal muscle basement membrane-sarcolemma-cytoskeleton interaction minireview series
    Kevin P Campbell
    Howard Hughes Medical Institute and Department of Physiology, University of Iowa College of Medicine, Iowa City, Iowa 52242, USA
    J Biol Chem 278:12599-600. 2003
  5. pmc Membrane targeting and stabilization of sarcospan is mediated by the sarcoglycan subcomplex
    R H Crosbie
    Howard Hughes Medical Institute, Department of Physiology and Biophysics, University of Iowa College of Medicine, Iowa City, Iowa 52242, USA
    J Cell Biol 145:153-65. 1999
  6. ncbi request reprint Expression of gamma -sarcoglycan in smooth muscle and its interaction with the smooth muscle sarcoglycan-sarcospan complex
    R Barresi
    Howard Hughes Medical Institute, Department of Physiology and Biophysics and Department of Neurology, University of Iowa College of Medicine, Iowa City, Iowa 52242, USA
    J Biol Chem 275:38554-60. 2000
  7. ncbi request reprint epsilon-sarcoglycan replaces alpha-sarcoglycan in smooth muscle to form a unique dystrophin-glycoprotein complex
    V Straub
    Howard Hughes Medical Institute, Department of Physiology and Biophysics, Department of Neurology, University of Iowa College of Medicine, Iowa City, Iowa 52242, USA
    J Biol Chem 274:27989-96. 1999
  8. ncbi request reprint Disruption of the beta-sarcoglycan gene reveals pathogenetic complexity of limb-girdle muscular dystrophy type 2E
    M Durbeej
    Howard Hughes Medical Institute, Department of Physiology and Biophysics, University of Iowa College of Medicine, Iowa City 52242, USA
    Mol Cell 5:141-51. 2000
  9. ncbi request reprint Molecular and genetic characterization of sarcospan: insights into sarcoglycan-sarcospan interactions
    R H Crosbie
    Howard Hughes Medical Institute, Department of Physiology and Biophysics, Department of Neurology, University of Iowa College of Medicine, Iowa City 52242, USA
    Hum Mol Genet 9:2019-27. 2000
  10. pmc Progressive muscular dystrophy in alpha-sarcoglycan-deficient mice
    F Duclos
    Howard Hughes Medical Institute, Department of Physiology and Biophysics and Department of Neurology, University of Iowa College of Medicine, Iowa City, Iowa 52242 1101, USA
    J Cell Biol 142:1461-71. 1998

Research Grants

  1. Therapeutic Potentential of Epsilon-Sarcoglycan in LGMD Type 2D
    Kevin Campbell; Fiscal Year: 2007
  2. Muscular Dystrophy Cooperative Research Center
    Kevin Campbell; Fiscal Year: 2007
  3. Epsilon-sarcoglycan in LGMD Type 2D
    Kevin Campbell; Fiscal Year: 2009

Detail Information

Publications127 found, 100 shown here

  1. pmc Variable disease severity in Saudi Arabian and Sudanese families with c.3924 + 2 T > C mutation of LAMA2
    Claudia Di Blasi
    Division of Neuromuscular Diseases and Neuroimmunology, Fondazione IRCCS Istituto Neurologico C, Besta, Milan, Italy
    BMC Res Notes 4:534. 2011
    ..abstract:..
  2. pmc Dystrophin deficiency exacerbates skeletal muscle pathology in dysferlin-null mice
    Renzhi Han
    Department of Molecular Physiology and Biophysics, Howard Hughes Medical Institute, Roy J and Lucille A Carver College of Medicine, The University of Iowa, 285 Newton Road, 4283 CBRB, Iowa City, IA 52242, USA
    Skelet Muscle 1:35. 2011
    ..abstract:..
  3. pmc Proteomic analysis of plasma membrane and secretory vesicles from human neutrophils
    Deepa Jethwaney
    Buck Institute for Age Research, Novato, CA 94945, USA
    Proteome Sci 5:12. 2007
    ....
  4. ncbi request reprint Skeletal muscle basement membrane-sarcolemma-cytoskeleton interaction minireview series
    Kevin P Campbell
    Howard Hughes Medical Institute and Department of Physiology, University of Iowa College of Medicine, Iowa City, Iowa 52242, USA
    J Biol Chem 278:12599-600. 2003
  5. pmc Membrane targeting and stabilization of sarcospan is mediated by the sarcoglycan subcomplex
    R H Crosbie
    Howard Hughes Medical Institute, Department of Physiology and Biophysics, University of Iowa College of Medicine, Iowa City, Iowa 52242, USA
    J Cell Biol 145:153-65. 1999
    ..In addition, the SG- SPN subcomplex functions to stabilize alpha-dystroglycan to the muscle plasma membrane. Taken together, our data provide important information about assembly and function of the SG-SPN subcomplex...
  6. ncbi request reprint Expression of gamma -sarcoglycan in smooth muscle and its interaction with the smooth muscle sarcoglycan-sarcospan complex
    R Barresi
    Howard Hughes Medical Institute, Department of Physiology and Biophysics and Department of Neurology, University of Iowa College of Medicine, Iowa City, Iowa 52242, USA
    J Biol Chem 275:38554-60. 2000
    ..Taken together, our results prove that the sarcoglycan complex in vascular and visceral smooth muscle consists of epsilon-, beta-, gamma-, and delta-sarcoglycans and is associated with sarcospan...
  7. ncbi request reprint epsilon-sarcoglycan replaces alpha-sarcoglycan in smooth muscle to form a unique dystrophin-glycoprotein complex
    V Straub
    Howard Hughes Medical Institute, Department of Physiology and Biophysics, Department of Neurology, University of Iowa College of Medicine, Iowa City, Iowa 52242, USA
    J Biol Chem 274:27989-96. 1999
    ..Our results also suggest a molecular basis for possible differential smooth muscle dysfunction in sarcoglycan-deficient patients...
  8. ncbi request reprint Disruption of the beta-sarcoglycan gene reveals pathogenetic complexity of limb-girdle muscular dystrophy type 2E
    M Durbeej
    Howard Hughes Medical Institute, Department of Physiology and Biophysics, University of Iowa College of Medicine, Iowa City 52242, USA
    Mol Cell 5:141-51. 2000
    ..Thus, perturbation of vascular function together with disruption of the epsilon-sarcoglycan-containing complex represents a novel mechanism in the pathogenesis of LGMD 2E...
  9. ncbi request reprint Molecular and genetic characterization of sarcospan: insights into sarcoglycan-sarcospan interactions
    R H Crosbie
    Howard Hughes Medical Institute, Department of Physiology and Biophysics, Department of Neurology, University of Iowa College of Medicine, Iowa City 52242, USA
    Hum Mol Genet 9:2019-27. 2000
    ..These findings are important as they contribute to a greater understanding of the structural determinants required for proper sarcoglycan-sarcospan expression and function...
  10. pmc Progressive muscular dystrophy in alpha-sarcoglycan-deficient mice
    F Duclos
    Howard Hughes Medical Institute, Department of Physiology and Biophysics and Department of Neurology, University of Iowa College of Medicine, Iowa City, Iowa 52242 1101, USA
    J Cell Biol 142:1461-71. 1998
    ..The Sgca-deficient mice will be a valuable model for elucidating the pathogenesis of sarcoglycan deficient limb-girdle muscular dystrophies and for the development of therapeutic strategies for this disease...
  11. pmc Prevention of cardiomyopathy in mouse models lacking the smooth muscle sarcoglycan-sarcospan complex
    R D Cohn
    Howard Hughes Medical Institute, Department of Physiology and Biophysics, Department of Neurology, University of Iowa College of Medicine, Iowa City, Iowa 52242, USA
    J Clin Invest 107:R1-7. 2001
    ....
  12. pmc Molecular pathogenesis of muscle degeneration in the delta-sarcoglycan-deficient hamster
    V Straub
    Howard Hughes Medical Institute, Department of Physiology and Biophysics, University of Iowa College of Medicine, Iowa City 52242, USA
    Am J Pathol 153:1623-30. 1998
    ..6 hamster. Based on our results, we propose that loss of delta-sarcoglycan results in the impairment of sarcolemmal integrity, finally leading to muscular dystrophy and cardiomyopathy...
  13. ncbi request reprint Biochemical characterization of ultrastructural localization of a major junctional sarcoplasmic reticulum glycoprotein (triadin)
    C M Knudson
    Howard Hughes Medical Institute, Iowa City, Iowa
    J Biol Chem 268:12637-45. 1993
    ....
  14. ncbi request reprint Identification and characterization of the dystrophin anchoring site on beta-dystroglycan
    D Jung
    Howard Hughes Medical Institute, University of Iowa College of Medicine, Iowa City 52242, USA
    J Biol Chem 270:27305-10. 1995
    ....
  15. ncbi request reprint Disruption of the dystrophin-glycoprotein complex in the cardiomyopathic hamster
    S L Roberds
    Howard Hughes Medical Institute, University of Iowa College of Medicine, Iowa City 52242
    J Biol Chem 268:11496-9. 1993
    ..Thus, the disruption of the dystrophin-glycoprotein complex may play a role in skeletal and cardiac myocyte necrosis of the cardiomyopathic hamster...
  16. ncbi request reprint Disruption of the sarcoglycan-sarcospan complex in vascular smooth muscle: a novel mechanism for cardiomyopathy and muscular dystrophy
    R Coral-Vazquez
    Howard Hughes Medical Institute, Department of Physiology and Biophysics, University of Iowa College of Medicine, Iowa City 52242, USA
    Cell 98:465-74. 1999
    ..Our data indicate that disruption of the SG-SSPN complex in vascular smooth muscle perturbs vascular function, which initiates cardiomyopathy and exacerbates muscular dystrophy...
  17. ncbi request reprint Identification of alpha-syntrophin binding to syntrophin triplet, dystrophin, and utrophin
    B Yang
    Howard Hughes Medical Institute, University of Iowa College of Medicine, Iowa City 52242
    J Biol Chem 270:4975-8. 1995
    ..Our data suggest that alpha-syntrophin binds all syntrophin isoforms, and syntrophin directly interacts with dystrophin through more than one binding site in dystrophin exons 73 and 74 including amino acids 3447-3481...
  18. pmc Dystrophin-glycoprotein complex is highly enriched in isolated skeletal muscle sarcolemma
    K Ohlendieck
    Howard Hughes Medical Institute, University of Iowa College of Medicine, Iowa City 52242
    J Cell Biol 112:135-48. 1991
    ..Thus the absence of dystrophin in Duchenne muscular dystrophy may result in a major disruption of the cytoskeletal network underlying the sarcolemma in dystrophic muscle...
  19. pmc Animal models for muscular dystrophy show different patterns of sarcolemmal disruption
    V Straub
    Department of, Howard Hughes Medical Institute, University of Iowa College of Medicine, Iowa City, Iowa 52242, USA
    J Cell Biol 139:375-85. 1997
    ....
  20. ncbi request reprint Sarcospan, the 25-kDa transmembrane component of the dystrophin-glycoprotein complex
    R H Crosbie
    Howard Hughes Medical Institute, Department of Physiology and Biophysics, University of Iowa College of Medicine, Iowa City, Iowa 52242, USA
    J Biol Chem 272:31221-4. 1997
    ..The gene encoding sarcospan maps to human chromosome 12p11.2, which falls within the genetic locus for congenital fibrosis of the extraocular muscle, an autosomal dominant muscular dystrophy...
  21. ncbi request reprint Heterogeneity of the 59-kDa dystrophin-associated protein revealed by cDNA cloning and expression
    B Yang
    Howard Hughes Medical Institute, University of Iowa College of Medicine, Iowa City 52242
    J Biol Chem 269:6040-4. 1994
    ..Our results suggest that the 59-DAP triplet may contain different protein species and that the 59-1 DAP may associate more specifically with dystrophin than with utrophin...
  22. ncbi request reprint Human dystroglycan: skeletal muscle cDNA, genomic structure, origin of tissue specific isoforms and chromosomal localization
    O Ibraghimov-Beskrovnaya
    Howard Hughes Medical Institute, University of Iowa College of Medicine, Iowa City 52242
    Hum Mol Genet 2:1651-7. 1993
    ..Therefore, we hypothesize that variable glycosylation of the conserved protein core might modulate laminin binding. The relationship of dystroglycan to human diseases is discussed...
  23. ncbi request reprint Primary structure and muscle-specific expression of the 50-kDa dystrophin-associated glycoprotein (adhalin)
    S L Roberds
    Howard Hughes Medical Institute, University of Iowa College of Medicine, Iowa City 52242
    J Biol Chem 268:23739-42. 1993
    ..Finally, 50-DAG mRNA is present in mdx and Duchenne muscular dystrophy (DMD) muscle, indicating that the down-regulation of this protein in DMD and the mdx mouse is likely a post-translational event...
  24. ncbi request reprint Biochemical characterization of the epithelial dystroglycan complex
    M Durbeej
    Howard Hughes Medical Institute, Department of Physiology and Biophysics, Department of Neurology, University of Iowa College of Medicine, Iowa City, Iowa 52242, USA
    J Biol Chem 274:26609-16. 1999
    ....
  25. ncbi request reprint Dystroglycan inside and out
    M D Henry
    Howard Hughes Medical Institute Department of Physiology and Biophysics Department of Neurology University of Iowa College of Medicine 400 Eckstein Medical Research Building, Iowa City, IA 52242, USA
    Curr Opin Cell Biol 11:602-7. 1999
    ..Together, these advances begin to help elucidate important biological roles for dystroglycan in development and disease...
  26. ncbi request reprint mdx muscle pathology is independent of nNOS perturbation
    R H Crosbie
    Howard Hughes Medical Institute, Department of Physiology and Biophysics, University of Iowa College of Medicine, Iowa City, IA 52242, USA
    Hum Mol Genet 7:823-9. 1998
    ..Furthermore, muscle from nNOS-dystrophin null mice maintain the histological features of mdx pathology. Our results demonstrate that relocalization of nNOS to the cytosol does not contribute significantly to mdx pathogenesis...
  27. ncbi request reprint Biochemical characterization and molecular cloning of cardiac triadin
    W Guo
    Howard Hughes Medical Institute, University of Iowa College of Medicine, Iowa City 52242, USA
    J Biol Chem 271:458-65. 1996
    ....
  28. ncbi request reprint Characterization of delta-sarcoglycan, a novel component of the oligomeric sarcoglycan complex involved in limb-girdle muscular dystrophy
    D Jung
    Howard Hughes Medical Institute and Department of Physiology and Biophysics, University of Iowa College of Medicine, Iowa City, Iowa 52242, USA
    J Biol Chem 271:32321-9. 1996
    ..Furthermore, we have mapped the human delta-sarcoglycan gene to chromosome 5q33-q34 in a region overlapping the recently linked autosomal recessive LGMD2F locus...
  29. ncbi request reprint Beta subunit heterogeneity in N-type Ca2+ channels
    V E Scott
    Howard Hughes Medical Institute and the Program in Neuroscience, Department of Physiology and Biophysics, University of Iowa College of Medicine, Iowa City, Iowa 52242, USA
    J Biol Chem 271:3207-12. 1996
    ..Thus, our data demonstrate important heterogeneity in the beta subunit composition of the N-type Ca2+ channels, which may be responsible for some of the diverse kinetic properties recorded from neurons...
  30. pmc Mild deficiency of dystrophin-associated proteins in Becker muscular dystrophy patients having in-frame deletions in the rod domain of dystrophin
    K Matsumura
    Howard Hughes Medical Institute, University of Iowa College of Medicine, Iowa City 52242
    Am J Hum Genet 53:409-16. 1993
    ....
  31. ncbi request reprint Absence of gamma-sarcoglycan (35 DAG) in autosomal recessive muscular dystrophy linked to chromosome 13q12
    D Jung
    Howard Hughes Medical Institute and Department of Physiology and Biophysics, University of Iowa College of Medicine, Iowa City 52242, USA
    FEBS Lett 381:15-20. 1996
    ..In addition, we show that in normal muscle alpha-, beta-, and gamma-sarcoglycan constitute a tightly associated sarcolemma complex which cannot be disrupted by SDS treatment...
  32. ncbi request reprint Mild congenital muscular dystrophy in two patients with an internally deleted laminin alpha2-chain
    V Allamand
    Howard Hughes Medical Institute and Department of Physiology and Biophysics, University of Iowa College of Medicine, Iowa City 52242, USA
    Hum Mol Genet 6:747-52. 1997
    ..This situation presents a striking analogy with Becker muscular dystrophy, where in-frame deletions in the dystrophin gene result in the expression of a semi-functional protein and lead to a mild phenotype...
  33. ncbi request reprint Early adenovirus-mediated gene transfer effectively prevents muscular dystrophy in alpha-sarcoglycan-deficient mice
    V Allamand
    Howard Hughes Medical Institute, Department of Physiology and Biophysics, University of Iowa College of Medicine, Iowa City 52242, USA
    Gene Ther 7:1385-91. 2000
    ....
  34. pmc Dystrophin-associated proteins are greatly reduced in skeletal muscle from mdx mice
    K Ohlendieck
    Howard Hughes Medical Institute, University of Iowa College of Medicine, Iowa City 52242
    J Cell Biol 115:1685-94. 1991
    ....
  35. ncbi request reprint Abnormal expression of dystrophin-associated proteins in Fukuyama-type congenital muscular dystrophy
    K Matsumura
    Howard Hughes Medical Institute, Iowa City, IA
    Lancet 341:521-2. 1993
    ....
  36. ncbi request reprint Beta-sarcoglycan: genomic analysis and identification of a novel missense mutation in the LGMD2E Amish isolate
    F Duclos
    Howard Hughes Medical Institute, Department of Physiology and Biophysics and Department of Neurology, University of Iowa College of Medicine, Iowa City 52242, USA
    Neuromuscul Disord 8:30-8. 1998
    ..Interestingly, in addition to the loss of the entire sarcoglycan complex, we detected a reduction of alpha-dystroglycan which suggests a role for the sarcoglycan complex in stabilizing alpha-dystroglycan at the sarcolemma...
  37. ncbi request reprint Direct binding of G-protein betagamma complex to voltage-dependent calcium channels
    M De Waard
    Howard Hughes Medical Institute, University of Iowa College of Medicine, Iowa City 52242, USA
    Nature 385:446-50. 1997
    ....
  38. ncbi request reprint Deficiency of merosin in dystrophic dy mice and genetic linkage of laminin M chain gene to dy locus
    Y Sunada
    Howard Hughes Medical Institute, University of Iowa College of Medicine, Iowa City 52242
    J Biol Chem 269:13729-32. 1994
    ....
  39. ncbi request reprint Duchenne muscular dystrophy: deficiency of dystrophin-associated proteins in the sarcolemma
    K Ohlendieck
    Howard Hughes Medical Institute, University of Iowa College of Medicine, Iowa City 52242
    Neurology 43:795-800. 1993
    ..The analysis of dystrophin-associated proteins is important in the assessment of experimental therapies that attempt to replace dystrophin in DMD muscle...
  40. ncbi request reprint Solubilization and biochemical characterization of the high affinity [3H]ryanodine receptor from rabbit brain membranes
    P S McPherson
    Howard Hughes Medical Institute, University of Iowa College of Medicine, Iowa City 52242
    J Biol Chem 265:18454-60. 1990
    ....
  41. ncbi request reprint Analysis of excitation-contraction-coupling components in chronically stimulated canine skeletal muscle
    K Ohlendieck
    Howard Hughes Medical Institute, University of Iowa College of Medicine, Iowa City
    Eur J Biochem 202:739-47. 1991
    ....
  42. ncbi request reprint Primary structure of the gamma subunit of the DHP-sensitive calcium channel from skeletal muscle
    S D Jay
    Howard Hughes Medical Institute, University of Iowa College of Medicine, Iowa City 52242
    Science 248:490-2. 1990
    ..The gamma subunit may play a role in assembly, modulation, or the structure of the skeletal muscle calcium channel...
  43. ncbi request reprint Primary structure and topological analysis of a skeletal muscle-specific junctional sarcoplasmic reticulum glycoprotein (triadin)
    C M Knudson
    Howard Hughes Medical Institute, Iowa City
    J Biol Chem 268:12646-54. 1993
    ..The abundance and localization of this protein suggest that it plays an important regulatory or structural role in excitation-contraction coupling in skeletal muscle...
  44. pmc Sarcospan-deficient mice maintain normal muscle function
    C S Lebakken
    Departments of Physiology and Biophysics and Neurology, Howard Hughes Medical Institute, Iowa City, Iowa 52242, USA
    Mol Cell Biol 20:1669-77. 2000
    ..These data suggest either that sarcospan is not required for normal DGC function or that the Sspn-deficient muscle is compensating for the absence of sarcospan, perhaps by utilizing another protein to carry out its function...
  45. ncbi request reprint Dystroglycan: an extracellular matrix receptor linked to the cytoskeleton
    M D Henry
    Howard Hughes Medical Institute, Department of Physiology, University of Iowa College of Medicine, 400 Eckstein Medical Research Building, Iowa City, IA 5224, 2 USA
    Curr Opin Cell Biol 8:625-31. 1996
    ....
  46. ncbi request reprint Adhalin mRNA and cDNA sequence are normal in the cardiomyopathic hamster
    S L Roberds
    Howard Hughes Medical Institute, University of Iowa College of Medicine, Iowa City 52242, USA
    FEBS Lett 364:245-9. 1995
    ..6 hamster cardiac muscle, and no mutation in adhalin coding sequence was found, indicating that the inherited myopathy and cardiomyopathy of the BIO 14.6 hamster are most likely not due to mutations in the adhalin gene...
  47. ncbi request reprint The ryanodine receptor/Ca2+ release channel
    P S McPherson
    Howard Hughes Medical Institute, University of Iowa College of Medicine, Iowa City 52242
    J Biol Chem 268:13765-8. 1993
  48. ncbi request reprint SH3 domain-mediated interaction of dystroglycan and Grb2
    B Yang
    Howard Hughes Medical Institute, College of Medicine, Iowa City, Iowa, USA
    J Biol Chem 270:11711-4. 1995
    ..The specific dystroglycan-Grb2 interaction may play an important role in extracellular matrix-mediated signal transduction and/or cytoskeleton organization in skeletal muscle that may be essential for muscle cell viability...
  49. ncbi request reprint Primary structure of dystrophin-associated glycoproteins linking dystrophin to the extracellular matrix
    O Ibraghimov-Beskrovnaya
    Howard Hughes Medical Institute, University of Iowa College of Medicine, Iowa City 52242
    Nature 355:696-702. 1992
    ....
  50. ncbi request reprint Calcium channel beta-subunit binds to a conserved motif in the I-II cytoplasmic linker of the alpha 1-subunit
    M Pragnell
    Howard Hughes Medical Institute, Department of Physiology, University of Iowa College of Medicine, Iowa City 52242
    Nature 368:67-70. 1994
    ..Conservation of the beta-subunit binding motif in these functionally distinct calcium channels suggests a critical role for the I-II cytoplasmic linker of the alpha 1-subunit in channel modulation by the beta-subunit...
  51. ncbi request reprint Biochemical and biophysical evidence for gamma 2 subunit association with neuronal voltage-activated Ca2+ channels
    M G Kang
    Howard Hughes Medical Institute, Department of Physiology and Biophysics, University of Iowa College of Medicine, Iowa City, Iowa 52242, USA
    J Biol Chem 276:32917-24. 2001
    ..Furthermore, the gamma(2) subunit likely contributes to the fine-tuning of neuronal Ca(2+) channels by counterbalancing the effects of the alpha(2)delta subunit...
  52. ncbi request reprint Reduced expression of dystroglycan in breast and prostate cancer
    M D Henry
    Howard Hughes Medical Institute, Department of Physiology and Biophysics, University of Iowa College of Medicine, Iowa City, IA 52242, USA
    Hum Pathol 32:791-5. 2001
    ..These data suggest that reduced expression of dystroglycan in prostate and breast cancers may lead to abnormal cell-extracellular matrix interactions and thus contribute to progression to metastatic disease...
  53. ncbi request reprint Association of triadin with the ryanodine receptor and calsequestrin in the lumen of the sarcoplasmic reticulum
    W Guo
    Howard Hughes Medical Institute, University of Iowa College of Medicine, Iowa City 52242, USA
    J Biol Chem 270:9027-30. 1995
    ....
  54. ncbi request reprint Distinct roles for dystroglycan, beta1 integrin and perlecan in cell surface laminin organization
    M D Henry
    Howard Hughes Medical Institute, University of Iowa College of Medicine, Department of Physiology and Biophysics and Department of Neurology, Iowa City, IA 52242, USA
    J Cell Sci 114:1137-44. 2001
    ....
  55. ncbi request reprint Dystroglycan in development and disease
    M Durbeej
    Howard Hughes Medical Institute, Department of Physiology and Biophysics, Iowa City, IA 52242, USA
    Curr Opin Cell Biol 10:594-601. 1998
    ....
  56. ncbi request reprint A monoclonal antibody to the beta subunit of the skeletal muscle dihydropyridine receptor immunoprecipitates the brain omega-conotoxin GVIA receptor
    J Sakamoto
    Howard Hughes Medical Institute, Department of Physiology and Biophysics, University of Iowa College of Medicine, Iowa City 52242
    J Biol Chem 266:18914-9. 1991
    ..These results suggest that the brain omega-conotoxin-sensitive calcium channel contains a component homologous to the beta subunit of the dihydropyridine-sensitive calcium channel of skeletal muscle and brain...
  57. ncbi request reprint Muscular dystrophies and the dystrophin-glycoprotein complex
    V Straub
    Howard Hughes Medical Institute, Department of Physiology and Biophysics, University of Iowa College of Medicine, Iowa City 52242, USA
    Curr Opin Neurol 10:168-75. 1997
    ....
  58. ncbi request reprint Identification of a novel mutant transcript of laminin alpha 2 chain gene responsible for muscular dystrophy and dysmyelination in dy2J mice
    Y Sunada
    Howard Hughes Medical Institute, University of Iowa College of Medicine, Iowa City 52242, USA
    Hum Mol Genet 4:1055-61. 1995
    ..Thus, the mutant alpha 2 chain could disrupt the formation of the laminin network and lead to muscle cell degeneration. Our results provide a molecular basis of muscular dystrophy and dysmyelination of peripheral nerve...
  59. ncbi request reprint Absence of the skeletal muscle sarcolemma chloride channel ClC-1 in myotonic mice
    C A Gurnett
    Howard Hughes Medical Institute, Department of Physiology and Biophysics, University of Iowa, College of Medicine, Iowa City 52242, USA
    J Biol Chem 270:9035-8. 1995
    ..Immunohistochemistry of skeletal muscle from ADR and other mouse models of human muscle disease demonstrate that the absence of ClC-1 chloride channel is a defect specific to ADR mice...
  60. ncbi request reprint Merosin-negative congenital muscular dystrophy associated with extensive brain abnormalities
    Y Sunada
    Howard Hughes Medical Institute, University of Iowa College of Medicine, Iowa City 52242, USA
    Neurology 45:2084-9. 1995
    ..We report here two patients with merosin-negative CMD presenting extensive brain abnormalities characterized by cortical anomaly, polymicrogyria, and abnormal white matter signals...
  61. ncbi request reprint Limb-girdle muscular dystrophy in the United States
    Steven A Moore
    University of Iowa, Iowa City, 52242, USA
    J Neuropathol Exp Neurol 65:995-1003. 2006
    ..The most common LGMDs in the United States are calpainopathies, dysferlinopathies, sarcoglycanopathies, and dystroglycanopathies...
  62. ncbi request reprint Loss of sarcolemma nNOS in sarcoglycan-deficient muscle
    Rachelle H Crosbie
    Howard Hughes Medical Institute, Department of Physiology and Biophysics, University of Iowa College of Medicine, Iowa City, Iowa 52242, USA
    FASEB J 16:1786-91. 2002
    ..Our data suggest that loss of nNOS may contribute to muscle pathology in AR-LGMD with primary mutations in the sarcoglycans...
  63. pmc Exercise-induced left ventricular systolic dysfunction in women heterozygous for dystrophinopathy
    Robert M Weiss
    University of Iowa Hospitals and Clinics, Division of Cardiovascular Medicine, Department of Internal Medicine, 200 Hawkins Drive, Room E317 1 GH, Iowa City, IA 52242, USA
    J Am Soc Echocardiogr 23:848-53. 2010
    ..Female "carriers" also can develop overt disease. The purpose of this study was to ascertain the prevalence of cardiac contractile abnormalities in dystrophinopathy carriers...
  64. ncbi request reprint Structural characterization of the dihydropyridine-sensitive calcium channel alpha 2-subunit and the associated delta peptides
    S D Jay
    Howard Hughes Medical Institute, University of Iowa College of Medicine, Iowa City 52242
    J Biol Chem 266:3287-93. 1991
    ..The data support a model of the alpha 2-subunit in which the propeptide is processed into two chains that remain attached through disulfide linkages...
  65. ncbi request reprint Defective membrane repair in dysferlin-deficient muscular dystrophy
    Dimple Bansal
    Howard Hughes Medical Institute, Department of Physiology and Biophysics, University of Iowa Roy J and Lucille A Carver College of Medicine, Iowa City, Iowa 52242, USA
    Nature 423:168-72. 2003
    ..Our findings show that disruption of the muscle membrane repair machinery is responsible for dysferlin-deficient muscle degeneration, and highlight the importance of this basic cellular mechanism of membrane resealing in human disease...
  66. ncbi request reprint Dysferlin and the plasma membrane repair in muscular dystrophy
    Dimple Bansal
    Howard Hughes Medical Institute, Department of Physiology and Biophysics and Department of Neurology, University of Iowa, Roy J and Lucille A Carver College of Medicine, Iowa City, IA 52242, USA
    Trends Cell Biol 14:206-13. 2004
    ..Here, we discuss the current knowledge of dysferlin function in the repair of the plasma membrane of the skeletal muscle cells...
  67. ncbi request reprint Muscular dystrophies involving the dystrophin-glycoprotein complex: an overview of current mouse models
    Madeleine Durbeej
    Howard Hughes Medical Institute, Department of Physiology and Biophysics, University of Iowa College of Medicine, Iowa City, Iowa 52242, USA
    Curr Opin Genet Dev 12:349-61. 2002
    ....
  68. pmc Gene transfer establishes primacy of striated vs. smooth muscle sarcoglycan complex in limb-girdle muscular dystrophy
    Madeleine Durbeej
    Howard Hughes Medical Institute, Department of Physiology and Biophysics, University of Iowa, Roy J and Lucille A Carver College of Medicine, Iowa City, IA 52242 1101, USA
    Proc Natl Acad Sci U S A 100:8910-5. 2003
    ..Thus, virus-mediated gene transfer of sarcoglycans to skeletal muscle in combination with pharmacological prevention of cardiomyopathy constitute promising therapeutic strategies for limb-girdle muscular dystrophies...
  69. pmc O-mannosyl phosphorylation of alpha-dystroglycan is required for laminin binding
    Takako Yoshida-Moriguchi
    Howard Hughes Medical Institute, University of Iowa Roy J and Lucille A Carver College of Medicine, 4283 Carver Biomedical Research Building, 285 Newton Road, Iowa City, IA 52242 1101, USA
    Science 327:88-92. 2010
    ..These findings expand our understanding of the mechanisms that underlie congenital muscular dystrophy...
  70. pmc Basal lamina strengthens cell membrane integrity via the laminin G domain-binding motif of alpha-dystroglycan
    Renzhi Han
    Howard Hughes Medical Institute, Department of Molecular Physiology, The University of Iowa, Iowa City, IA 52242, USA
    Proc Natl Acad Sci U S A 106:12573-9. 2009
    ..These data provide direct mechanistic insight into how the dystroglycan-linked basal lamina contributes to the maintenance of sarcolemmal integrity and protects muscles from damage...
  71. ncbi request reprint Molecular recognition by LARGE is essential for expression of functional dystroglycan
    Motoi Kanagawa
    Howard Hughes Medical Institute, Department of Physiology and Biophysics, The University of Iowa Roy J and Lucille A Carver College of Medicine, Iowa City, Iowa 52242, USA
    Cell 117:953-64. 2004
    ..Therefore, molecular recognition of dystroglycan by LARGE is a key determinant in the biosynthetic pathway to produce mature and functional dystroglycan...
  72. ncbi request reprint Association of dystrophin-related protein with dystrophin-associated proteins in mdx mouse muscle
    K Matsumura
    Howard Hughes Medical Institute, University of Iowa College of Medicine, Iowa City 52242
    Nature 360:588-91. 1992
    ..Because these muscles show minimal pathological changes, our results could provide a basis for the upregulation of DRP as a potential therapeutic approach...
  73. ncbi request reprint Fukutin-related protein associates with the sarcolemmal dystrophin-glycoprotein complex
    Aaron M Beedle
    Howard Hughes Medical Institute HHMI, Departments of Molecular Physiology, University of Iowa Carver College of Medicine, Iowa City, Iowa 52242, USA
    J Biol Chem 282:16713-7. 2007
    ..These data offer the first evidence of an FKRP complex in muscle and suggest that FKRP may influence the glycosylation status of dystroglycan from within the sarcolemmal dystrophin-glycoprotein complex...
  74. pmc Dysferlin-mediated membrane repair protects the heart from stress-induced left ventricular injury
    Renzhi Han
    Howard Hughes Medical Institute, The University of Iowa, Roy J and Lucille A Carver College of Medicine, Iowa City, IA 52242, USA
    J Clin Invest 117:1805-13. 2007
    ..Thus, our study establishes what we believe is a novel mechanism underlying the cardiomyopathy that results from a defective membrane repair in the absence of dysferlin...
  75. pmc Dysferlin and muscle membrane repair
    Renzhi Han
    Howard Hughes Medical Institute, Department of Molecular Physiology and Biophysics, Roy J and Lucille A Carver College of Medicine, The University of Iowa, Iowa City, IA 52242, USA
    Curr Opin Cell Biol 19:409-16. 2007
    ..Future research on dysferlin and its interacting partners will enhance the understanding of this important process and provide novel avenues to potential therapies...
  76. pmc Sarcolemma-localized nNOS is required to maintain activity after mild exercise
    Yvonne M Kobayashi
    Howard Hughes Medical Institute, University of Iowa, Roy J and Lucille A Carver College of Medicine, 4283 Carver Biomedical Research Building, 285 Newton Road, Iowa City, Iowa 52242 1101, USA
    Nature 456:511-5. 2008
    ..Our results suggest that patients with an exaggerated fatigue response to mild exercise would show clinical improvement in response to treatment strategies aimed at improving exercise-induced signalling...
  77. pmc Sarcoglycan complex: implications for metabolic defects in muscular dystrophies
    Séverine Groh
    Howard Hughes Medical Institute, University of Iowa Roy J and Lucille A Carver College of Medicine, Iowa City, Iowa 52242 1101, USA
    J Biol Chem 284:19178-82. 2009
    ....
  78. pmc Visual impairment in the absence of dystroglycan
    Jakob S Satz
    Department of Molecular Physiology and Biophysics, Roy J and Lucille A Carver College of Medicine, Howard Hughes Medical Institute, University of Iowa, Iowa City, Iowa 52242, USA
    J Neurosci 29:13136-46. 2009
    ..In contrast to the role of alpha-dystroglycan extracellular interactions during early development of the CNS, beta-dystroglycan intracellular interactions are important for visual function but not the laminar development of the retina...
  79. doi request reprint Unraveling the ribbon synapse
    Jakob S Satz
    Howard Hughes Medical Institute, Department of Molecular Physiology and Biophysics, 4283 Carver Biomedical Research Building, 285 Newton Road, Roy J and Lucille A Carver College of Medicine, University of Iowa, Iowa City, Iowa 52242, USA
    Nat Neurosci 11:857-9. 2008
  80. ncbi request reprint Dystrophin-glycoprotein complex: post-translational processing and dystroglycan function
    Daniel E Michele
    Department of Physiology and Biophysics, Howard Hughes Medical Institute, 400 ERMB, Carver College of Medicine, University of Iowa, Iowa City, IA 52242 1101, USA
    J Biol Chem 278:15457-60. 2003
  81. pmc A common disease-associated missense mutation in alpha-sarcoglycan fails to cause muscular dystrophy in mice
    Kazuhiro Kobuke
    Howard Hughes Medical Institute, The University of Iowa Roy J and Lucille A Carver College of Medicine, Iowa City, Iowa 52242, USA
    Hum Mol Genet 17:1201-13. 2008
    ..Our study presents an unexpected difference in the behavior of a missense-mutated protein in mice versus human patients, and emphasizes the need to understand species-specific protein quality control systems...
  82. ncbi request reprint Disruption of DAG1 in differentiated skeletal muscle reveals a role for dystroglycan in muscle regeneration
    Ronald D Cohn
    Howard Hughes Medical Institute, Department of Physiology and Biophysics, Department of Neurology, Iowa City, IA 52242, USA
    Cell 110:639-48. 2002
    ..Therefore, inadequate repair of skeletal muscle by satellite cells represents an important mechanism affecting the pathogenesis of muscular dystrophy...
  83. ncbi request reprint Characterization of aquaporin-4 in muscle and muscular dystrophy
    Rachelle H Crosbie
    Howard Hughes Medical Institute, Department of Physiology and Biophysics, University of Iowa College of Medicine, Iowa City, Iowa 52242, USA
    FASEB J 16:943-9. 2002
    ..Taken together, our data demonstrate that AQP4 loss in skeletal muscle correlates with muscular dystrophy and is a common feature of pathogenesis...
  84. ncbi request reprint Limb-girdle muscular dystrophies
    Federica Piccolo
    Department of Physiology and Biophysics and Department of Neurology, Howard Hughes Medical Institute, University of Iowa College of Medicine, Iowa City, Iowa 52242, USA
    Adv Neurol 88:273-91. 2002
  85. pmc Distinct functions of glial and neuronal dystroglycan in the developing and adult mouse brain
    Jakob S Satz
    Howard Hughes Medical Institute, and Department of Molecular Physiology, University of Iowa, Roy J and Lucille A Carver College of Medicine, Iowa City, Iowa 52242, USA
    J Neurosci 30:14560-72. 2010
    ..Differences in dystroglycan glycosylation in distinct cell types of the CNS may contribute to the diversity of dystroglycan function in the CNS, as well as to the broad clinical spectrum of type II lissencephalies...
  86. ncbi request reprint Unique role of dystroglycan in peripheral nerve myelination, nodal structure, and sodium channel stabilization
    Fumiaki Saito
    Howard Hughes Medical Institute, Department of Physiology and Biophysics, University of Iowa Roy J and Lucille A Carver College of Medicine, Iowa City, IA 52242, USA
    Neuron 38:747-58. 2003
    ..Dystroglycan may be required for the normal maintenance of voltage-gated sodium channels at nodes of Ranvier, possibly by mediating trans interactions between Schwann cell microvilli and the nodal axolemma...
  87. ncbi request reprint Post-translational disruption of dystroglycan-ligand interactions in congenital muscular dystrophies
    Daniel E Michele
    Howard Hughes Medical Institute, Department of Physiology and Biophysics, University of Iowa, Iowa City, Iowa 52242 1101, USA
    Nature 418:417-22. 2002
    ....
  88. pmc Basolateral entry and release of New and Old World arenaviruses from human airway epithelia
    Douglas E Dylla
    The Roy J and Lucille A Carver College of Medicine, University of Iowa, Iowa City, Iowa 52242, USA
    J Virol 82:6034-8. 2008
    ..Viral egress from infected epithelia exhibited basolateral polarity. This study demonstrates that respiratory entry of arenaviruses occurs via basolateral receptors...
  89. ncbi request reprint Disruption of perlecan binding and matrix assembly by post-translational or genetic disruption of dystroglycan function
    Motoi Kanagawa
    Department of Physiology and Biophysics, Howard Hughes Medical Institute, Roy J and Lucille A Carver College of Medicine, The University of Iowa, 400 Eckstein Medical Building, Iowa City, IA 52242, USA
    FEBS Lett 579:4792-6. 2005
    ..These data suggest functional disruption of the trimolecular complex in glycosylation-deficient muscular dystrophy...
  90. ncbi request reprint Dystroglycan: from biosynthesis to pathogenesis of human disease
    Rita Barresi
    Howard Hughes Medical Institute, Department of Physiology and Biophysics, Roy J and Lucille A Carver College of Medicine, University of Iowa, Iowa City, IA 52242, USA
    J Cell Sci 119:199-207. 2006
    ..Our increased understanding of the mechanisms of interaction of dystroglycan with its ligands has become an essential tool in deciphering the biological processes related to the human diseases in which the proteins are implicated...
  91. pmc Brain and eye malformations resembling Walker-Warburg syndrome are recapitulated in mice by dystroglycan deletion in the epiblast
    Jakob S Satz
    Howard Hughes Medical Institute and Department of Molecular Physiology, University of Iowa, Iowa City, Iowa 52242, USA
    J Neurosci 28:10567-75. 2008
    ....
  92. ncbi request reprint LARGE can functionally bypass alpha-dystroglycan glycosylation defects in distinct congenital muscular dystrophies
    Rita Barresi
    Howard Hughes Medical Institute, Department of Physiology and Biophysics, Roy J and Lucille A Carver College of Medicine, University of Iowa, Iowa City, Iowa 52242, USA
    Nat Med 10:696-703. 2004
    ..Our findings indicate that modulation of LARGE expression or activity is a viable therapeutic strategy for glycosyltransferase-deficient congenital muscular dystrophies...
  93. pmc Loss of alpha-dystroglycan laminin binding in epithelium-derived cancers is caused by silencing of LARGE
    Daniel Beltran Valero de Bernabe
    Howard Hughes Medical Institute, University of Iowa, Roy J and Lucille A Carver College of Medicine, Iowa City, Iowa 52242 1101, USA
    J Biol Chem 284:11279-84. 2009
    ....
  94. ncbi request reprint Deletion of brain dystroglycan recapitulates aspects of congenital muscular dystrophy
    Steven A Moore
    Department of Pathology, The University of Iowa, Iowa City, Iowa 52242 1101, USA
    Nature 418:422-5. 2002
    ..Our data strongly support the hypothesis that defects in dystroglycan are central to the pathogenesis of structural and functional brain abnormalities seen in CMD...
  95. pmc A functional AMPA receptor-calcium channel complex in the postsynaptic membrane
    Myoung Goo Kang
    Howard Hughes Medical Institute and Departments of Physiology and Biophysics, Internal Medicine, and Neurology, University of Iowa Roy J and Lucille A Carver College of Medicine, Iowa City, IA 52242, USA
    Proc Natl Acad Sci U S A 103:5561-6. 2006
    ....
  96. ncbi request reprint Abnormal coronary function in mice deficient in alpha1H T-type Ca2+ channels
    Chien Chang Chen
    Howard Hughes Medical Institute, University of Iowa, Iowa City, IA 52242, USA
    Science 302:1416-8. 2003
    ..Furthermore, acute blockade of T-channels with Ni2+ prevented relaxation of wild-type coronary arteries. Thus, Ca2+ influx through alpha1H T-type Ca2+ channels is essential for normal relaxation of coronary arteries...
  97. ncbi request reprint C-terminal titin deletions cause a novel early-onset myopathy with fatal cardiomyopathy
    Virginie Carmignac
    Institut National de la Sante et de la Recherche Médicale U582, Institut de Myologie, Groupe Hospitalier Pitie Salpetriere, France
    Ann Neurol 61:340-51. 2007
    ..Our aim was to delineate the phenotype and determine the genetic defects in two consanguineous families with an early-onset, recessive muscle and cardiac disorder...
  98. ncbi request reprint Congenital muscular dystrophy with glycosylation defects of alpha-dystroglycan in Japan
    Hiroshi Matsumoto
    Department of Neuromuscular Research, National Institute of Neuroscience, National Center of Neurology and Psychiatry NCNP, 4 1 1 Ogawahigashi, Kodaira, Tokyo 187 8502, Japan
    Neuromuscul Disord 15:342-8. 2005
    ..This result suggests that other factors can modify clinical features of the patients with glycosylation defects of alpha-DG...
  99. ncbi request reprint Fukutin gene mutations cause dilated cardiomyopathy with minimal muscle weakness
    Terumi Murakami
    Department of Neuromuscular Research, National Institute of Neuroscience, National Center of Neurology and Psychiatry, Tokyo, Japan
    Ann Neurol 60:597-602. 2006
    ..Fukuyama-type congenital muscular dystrophy is one of the disorders associated with glycosylation defects of alpha-dystroglycan, an indispensable molecule for intra-extra cell membrane linkage...
  100. pmc Alpha6beta4 integrin and dystroglycan cooperate to stabilize the myelin sheath
    Alessandro Nodari
    Department of Biological and Technological Research, San Raffaele Scientific Institute, 20132 Milan, Italy
    J Neurosci 28:6714-9. 2008
    ..These data indicate that, similar to its role in skin, alpha6beta4 integrin confers stability to myelin in peripheral nerves...
  101. ncbi request reprint Gamma 1 subunit interactions within the skeletal muscle L-type voltage-gated calcium channels
    Jyothi Arikkath
    Howard Hughes Medical Institute, Department of Physiology, University of Iowa, Iowa City 52242, USA
    J Biol Chem 278:1212-9. 2003
    ..1. Because subunit interactions are conserved, these studies have broad implications for gamma heterogeneity, function and subunit association with voltage-gated calcium channels...

Research Grants8

  1. Therapeutic Potentential of Epsilon-Sarcoglycan in LGMD Type 2D
    Kevin Campbell; Fiscal Year: 2007
    ..on an endogenous protein like epsilon-sarcoglycan will bypass any acquired therapeutic immune response, and provides a platform for new targets for therapy and drug treatments aimed at up-regulating epsilon-sarcoglycan ..
  2. Muscular Dystrophy Cooperative Research Center
    Kevin Campbell; Fiscal Year: 2007
    ....
  3. Epsilon-sarcoglycan in LGMD Type 2D
    Kevin Campbell; Fiscal Year: 2009
    ....