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Title: GREG cells, a dysferlin-deficient myogenic mouse cell line

Abstract

The dysferlinopathies (e.g. LGMD2b, Myoshi myopathy) are progressive, adult-onset muscle wasting syndromes caused by mutations in the gene coding for dysferlin. Dysferlin is a large ({approx} 200 kDa) membrane-anchored protein, required for maintenance of plasmalemmal integrity in muscle fibers. To facilitate analysis of dysferlin function in muscle cells, we have established a dysferlin-deficient myogenic cell line (GREG cells) from the A/J mouse, a genetic model for dysferlinopathy. GREG cells have no detectable dysferlin expression, but proliferate normally in growth medium and fuse into functional myotubes in differentiation medium. GREG myotubes exhibit deficiencies in plasma membrane repair, as measured by laser wounding in the presence of FM1-43 dye. Under the wounding conditions used, the majority ({approx} 66%) of GREG myotubes lack membrane repair capacity, while no membrane repair deficiency was observed in dysferlin-normal C2C12 myotubes, assayed under the same conditions. We discuss the possibility that the observed heterogeneity in membrane resealing represents genetic compensation for dysferlin deficiency.

Authors:
; ;  [1];  [2];  [1];  [3];  [1]
  1. Program in Physical Biology, Eunice Kennedy Schriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD 20892 (United States)
  2. Center for Human Genetics, Leiden University Medical Center, Leiden (Netherlands)
  3. Research Center for Genetic Medicine, Children's National Medical Center, Washington, DC 20010 (United States)
Publication Date:
OSTI Identifier:
22212290
Resource Type:
Journal Article
Resource Relation:
Journal Name: Experimental Cell Research; Journal Volume: 318; Journal Issue: 2; Other Information: Copyright (c) 2011 Elsevier Science B.V., Amsterdam, The Netherlands, All rights reserved.; Country of input: International Atomic Energy Agency (IAEA)
Country of Publication:
United States
Language:
English
Subject:
60 APPLIED LIFE SCIENCES; BIOLOGICAL REPAIR; MICE; MUSCLES; PROTEINS; WOUNDS

Citation Formats

Humphrey, Glen W., Mekhedov, Elena, Blank, Paul S., Morree, Antoine de, Pekkurnaz, Gulcin, Nagaraju, Kanneboyina, and Zimmerberg, Joshua, E-mail: zimmerbj@mail.nih.gov. GREG cells, a dysferlin-deficient myogenic mouse cell line. United States: N. p., 2012. Web. doi:10.1016/J.YEXCR.2011.10.004.
Humphrey, Glen W., Mekhedov, Elena, Blank, Paul S., Morree, Antoine de, Pekkurnaz, Gulcin, Nagaraju, Kanneboyina, & Zimmerberg, Joshua, E-mail: zimmerbj@mail.nih.gov. GREG cells, a dysferlin-deficient myogenic mouse cell line. United States. doi:10.1016/J.YEXCR.2011.10.004.
Humphrey, Glen W., Mekhedov, Elena, Blank, Paul S., Morree, Antoine de, Pekkurnaz, Gulcin, Nagaraju, Kanneboyina, and Zimmerberg, Joshua, E-mail: zimmerbj@mail.nih.gov. Sun . "GREG cells, a dysferlin-deficient myogenic mouse cell line". United States. doi:10.1016/J.YEXCR.2011.10.004.
@article{osti_22212290,
title = {GREG cells, a dysferlin-deficient myogenic mouse cell line},
author = {Humphrey, Glen W. and Mekhedov, Elena and Blank, Paul S. and Morree, Antoine de and Pekkurnaz, Gulcin and Nagaraju, Kanneboyina and Zimmerberg, Joshua, E-mail: zimmerbj@mail.nih.gov},
abstractNote = {The dysferlinopathies (e.g. LGMD2b, Myoshi myopathy) are progressive, adult-onset muscle wasting syndromes caused by mutations in the gene coding for dysferlin. Dysferlin is a large ({approx} 200 kDa) membrane-anchored protein, required for maintenance of plasmalemmal integrity in muscle fibers. To facilitate analysis of dysferlin function in muscle cells, we have established a dysferlin-deficient myogenic cell line (GREG cells) from the A/J mouse, a genetic model for dysferlinopathy. GREG cells have no detectable dysferlin expression, but proliferate normally in growth medium and fuse into functional myotubes in differentiation medium. GREG myotubes exhibit deficiencies in plasma membrane repair, as measured by laser wounding in the presence of FM1-43 dye. Under the wounding conditions used, the majority ({approx} 66%) of GREG myotubes lack membrane repair capacity, while no membrane repair deficiency was observed in dysferlin-normal C2C12 myotubes, assayed under the same conditions. We discuss the possibility that the observed heterogeneity in membrane resealing represents genetic compensation for dysferlin deficiency.},
doi = {10.1016/J.YEXCR.2011.10.004},
journal = {Experimental Cell Research},
number = 2,
volume = 318,
place = {United States},
year = {Sun Jan 15 00:00:00 EST 2012},
month = {Sun Jan 15 00:00:00 EST 2012}
}