Zobrazeno 1 - 10
of 70
pro vyhledávání: '"Jack H. Van Der Meulen"'
Autor:
Vanessa E. Jahnke, Jennifer M. Peterson, Jack H. Van Der Meulen, Jessica Boehler, Kitipong Uaesoontrachoon, Helen K. Johnston, Aurelia Defour, Aditi Phadke, Qing Yu, Jyoti K. Jaiswal, Kanneboyina Nagaraju
Publikováno v:
Skeletal Muscle, Vol 10, Iss 1, Pp 1-17 (2020)
Abstract Background Nonsense or loss-of-function mutations in the non-lysosomal cysteine protease calpain-3 result in limb-girdle muscular dystrophy type 2A (LGMD2A). While calpain-3 is implicated in muscle cell differentiation, sarcomere formation,
Externí odkaz:
https://doaj.org/article/050731eeba884453b57d2710d7a8e532
Autor:
Daniel C. Bittel, Sen Chandra Sreetama, Goutam Chandra, Robin Ziegler, Kanneboyina Nagaraju, Jack H. Van der Meulen, Jyoti K. Jaiswal
Publikováno v:
The Journal of Clinical Investigation, Vol 132, Iss 1 (2022)
Efficient sarcolemmal repair is required for muscle cell survival, with deficits in this process leading to muscle degeneration. Lack of the sarcolemmal protein dysferlin impairs sarcolemmal repair by reducing secretion of the enzyme acid sphingomyel
Externí odkaz:
https://doaj.org/article/2286d1a20dd14e03a5b1ecfa798ba42c
Autor:
Jack H. Van der Meulen, Jennifer M. Peterson, Helen Johnston, Aditi Phadke, Jessica F. Boehler, Aurelia Defour, Vanessa E. Jahnke, Kanneboyina Nagaraju, Qing Yu, Jyoti K. Jaiswal, Kitipong Uaesoontrachoon
Publikováno v:
Skeletal Muscle, Vol 10, Iss 1, Pp 1-17 (2020)
Skeletal Muscle
Skeletal Muscle
BackgroundNonsense or loss-of-function mutations in the non-lysosomal cysteine protease calpain-3 result in limb-girdle muscular dystrophy type 2A (LGMD2A). While calpain-3 is implicated in muscle cell differentiation, sarcomere formation, and muscle
Autor:
Margaret E Benny Klimek, Arpana Sali, Sree Rayavarapu, Jack H Van der Meulen, Kanneboyina Nagaraju
Publikováno v:
PLoS ONE, Vol 11, Iss 5, p e0155944 (2016)
Duchenne muscular dystrophy (DMD) is an X-linked muscle disease caused by mutations in the dystrophin gene. The pathology of DMD manifests in patients with progressive muscle weakness, loss of ambulation and ultimately death. One of the characteristi
Externí odkaz:
https://doaj.org/article/dee8d8ed992648e6a31ffce488f3537c
Autor:
Jack H. Van der Meulen, Zenaide M.N. Quezado, Martha Quezado, Luis E.F. Almeida, Sayuri Kamimura, Kanneboyina Nagaraju, Li Wang, Paul Wakim
Publikováno v:
Nitric Oxide. 80:70-81
Sickle cell disease (SCD) patients can have limited exercise capacity and muscle dysfunction characterized by decreased force, atrophy, microvascular abnormalities, fiber distribution changes, and skeletal muscle energetics abnormalities. Growing evi
Publikováno v:
PLoS ONE, Vol 8, Iss 6, p e65970 (2013)
BACKGROUND: Current treatments for idiopathic inflammatory myopathies (collectively called myositis) focus on the suppression of an autoimmune inflammatory response within the skeletal muscle. However, it has been observed that there is a poor correl
Externí odkaz:
https://doaj.org/article/00242daba01e42f2b610cea7d81c6751
Autor:
Arpana Sali, Gina M Many, Heather Gordish-Dressman, Jack H van der Meulen, Aditi Phadke, Christopher F Spurney, Avital Cnaan, Eric P Hoffman, Kanneboyina Nagaraju
Publikováno v:
PLoS ONE, Vol 8, Iss 7, p e66617 (2013)
In Duchenne muscular dystrophy (DMD), loss of the membrane stabilizing protein dystrophin results in myofiber damage. Microinjury to dystrophic myofibers also causes secondary imbalances in sarcolemmic ion permeability and resting membrane potential,
Externí odkaz:
https://doaj.org/article/6365d2c0d17747f2b375666423eb7e53
Autor:
Sree Rayavarapu, Jack H Van der Meulen, Heather Gordish-Dressman, Eric P Hoffman, Kanneboyina Nagaraju, Susan M Knoblach
Publikováno v:
PLoS ONE, Vol 5, Iss 9, p e12981 (2010)
The dysferlin deficient SJL/J mouse strain is commonly used to study dysferlin deficient myopathies. Therefore, we systematically evaluated behavior in relatively young (9-25 weeks) SJL/J mice and compared them to C57BL6 mice to determine which funct
Externí odkaz:
https://doaj.org/article/c297953393754b1e828e83b37247ebbf
Autor:
Kanneboyina Nagaraju, Jack H. Van der Meulen, Jessica F. Boehler, Marshall W. Hogarth, Apostolos Malatras, William Duddy, Sushma Medikayala, Aurelia Defour, Jyoti K. Jaiswal, Nicholas Holdreith
Publikováno v:
Human Molecular Genetics. 26:1979-1991
Repair of skeletal muscle after sarcolemmal damage involves dysferlin and dysferlin-interacting proteins such as annexins. Mice and patient lacking dysferlin exhibit chronic muscle inflammation and adipogenic replacement of the myofibers. Here, we sh
Autor:
Laurent P. Bogdanik, James S. Novak, Jack H. Van der Meulen, Allison Saunders, Whitney Andrews, William Coley, Gregory A. Cox, Catherine Lammert, Terence A. Partridge, Connor P. Dolan, Maria Candida Vila, Cathleen M. Lutz, Marie Nearing, Qing Yu, Andrew Austin, James L Quinn, Sree Rayavarapu, Kanneboyina Nagaraju
Publikováno v:
Human Molecular Genetics. 25:130-145
Genetic background significantly affects phenotype in multiple mouse models of human diseases, including muscular dystrophy. This phenotypic variability is partly attributed to genetic modifiers that regulate the disease process. Studies have demonst