The DOI matches its Crossref record; 1 of the 1 references it cites were found in a registry or an index.
Integrins (alpha7beta1) in muscle function and survival. Disrupted expression in merosin-deficient congenital muscular dystrophy.
Open accessAbstract
Mutations in genes coding for dystrophin, for alpha, beta, gamma, and delta-sarcoglycans, or for the alpha2 chain of the basement membrane component merosin (laminin-2/4) cause various forms of muscular dystrophy. Analyses of integrins showed an abnormal expression and localization of alpha7beta1 isoforms in myofibers of merosin-deficient human patients and mice, but not in dystrophin-deficient or sarcoglycan-deficient humans and animals. It was shown previously that skeletal muscle fibers require merosin for survival and function (Vachon, P.H., F. Loechel, H. Xu, U.M. Wewer, and E. Engvall. 1996. J. Cell Biol. 134:1483-1497). Correction of merosin deficiency in vitro through cell transfection with the merosin alpha2 chain restored the normal localization of alpha7beta1D integrins as well as myotube survival. Overexpression of the apoptosis-suppressing molecule Bcl-2 also promoted the survival of merosin-deficient myotubes, but did not restore a normal expression of alpha7beta1D integrins. Blocking of beta1 integrins in normal myotubes induced apoptosis and severely reduced their survival. These findings (a) identify alpha7beta1D integrins as the de facto receptors for merosin in skeletal muscle; (b) indicate a merosin dependence for the accurate expression and membrane localization of alpha7beta1D integrins in myofibers; (c) provide a molecular basis for the critical role of merosin in myofiber survival; and (d) add new insights to the pathogenesis of neuromuscular disorders.
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Cites
RGD AND OTHER RECOGNITION SEQUENCES FOR INTEGRINS
Ruoslahti, E. (1996) · Annual Review of Cell and Developmental Biology · doi:10.1146/annurev.cellbio.12.1.697 · Cited by 3,191
Cited by
Garcı́a, A. J., Vega, M. D., & Boettiger, D. (1999) · Molecular Biology of the Cell · doi:10.1091/mbc.10.3.785 · Cited by 659
Rando, T. A. (2001) · Muscle & Nerve · doi:10.1002/mus.1192 · Cited by 424
Integrins as receptors for laminins
Belkin, A. M., & Stepp, M. A. (2000) · Microscopy Research and Technique · doi:10.1002/1097-0029(20001101)51:3<280::aid-jemt7>3.0.co;2-o · Cited by 384
Mutations in the integrin α7 gene cause congenital myopathy
Hayashi, Y. K., Chou, F.-L., Engvall, E., Ogawa, M., Matsuda, C., Hirabayashi, S., Yokochi, K., Ziober, B. L., Kramer, R. H., Kaufman, S. J., et al. (1998) · Nature Genetics · doi:10.1038/ng0598-94 · Cited by 366
Self-citationDystrophin, its interactions with other proteins, and implications for muscular dystrophy
Ervasti, J. M. (2007) · Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease · doi:10.1016/j.bbadis.2006.05.010 · Cited by 324
Metadata
- Authors
- Vachon, P. H., Xu, H., Liu, L., Loechel, F., Hayashi, Y., Arahata, K., Reed, J. C., Wewer, U. M., & Engvall, E.
- Venue
- Journal of Clinical Investigation
- Published
- 1997-10-01
- Volume
- 100
- Issue
- 7
- Pages
- 1870-1881
- ISSN
- 0021-9738 (ISSN-L)
- OpenAlex
- W2066334706
- Cited by
- 185
- References
- 1
- FWCI
- 4.72
- Type
- journal article
- Language
- en
- Open access
- bronze
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- Vachon, P. H., Xu, H., Liu, L., Loechel, F., Hayashi, Y., Arahata, K., Reed, J. C., Wewer, U. M., & Engvall, E. (1997). Integrins (alpha7beta1) in muscle function and survival. Disrupted expression in merosin-deficient congenital muscular dystrophy. Journal of Clinical Investigation. https://doi.org/10.1172/jci119716
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Contains information from the Semantic Scholar Academic Graph Datasets, made available under the ODC Attribution License (ODC-BY 1.0).
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