Immunoelectron microscopic localization of dystrophin in myofibres - PubMed (original) (raw)
. 1988 Jun 30;333(6176):863-6.
doi: 10.1038/333863a0.
Affiliations
- PMID: 3290684
- DOI: 10.1038/333863a0
Immunoelectron microscopic localization of dystrophin in myofibres
S C Watkins et al. Nature. 1988.
Abstract
Duchenne muscular dystrophy, a common X-linked recessive human disease, has recently been shown to be caused by the deficiency of a large, low abundance protein called 'dystrophin'. Biochemical techniques have shown dystrophin to be membrane-associated in skeletal muscle, with enrichment of dystrophin in the t-tubules of 'triads'. Other studies using immunohistochemistry on thick (10 micron) sections have shown dystrophin to be located at the periphery of muscle fibres, possibly at the plasma membrane. These results have been interpreted as being either consistent and complementary, or contradictory. To localize dystrophin more precisely relative to these membrane systems we have employed highly sensitive and spatially accurate immuno-gold electron microscopy of ultra-thin (70-100 nm) cryosections. The major distribution of dystrophin was on the cytoplasmic face of the plasma membrane of muscle fibres, and possibly on the contiguous t-tubule membranes. The presented data, taken together with recently accumulated information regarding the primary structure of dystrophin, suggests that dystrophin is a component of the membrane cytoskeleton in myogenic cells. Thus, myofibre necrosis in patients affected with Duchenne muscular dystrophy is likely the result of plasma membrane instability.
Similar articles
- The Duchenne muscular dystrophy gene product is localized in sarcolemma of human skeletal muscle.
Zubrzycka-Gaarn EE, Bulman DE, Karpati G, Burghes AH, Belfall B, Klamut HJ, Talbot J, Hodges RS, Ray PN, Worton RG. Zubrzycka-Gaarn EE, et al. Nature. 1988 Jun 2;333(6172):466-9. doi: 10.1038/333466a0. Nature. 1988. PMID: 3287171 - Deficiency of a glycoprotein component of the dystrophin complex in dystrophic muscle.
Ervasti JM, Ohlendieck K, Kahl SD, Gaver MG, Campbell KP. Ervasti JM, et al. Nature. 1990 May 24;345(6273):315-9. doi: 10.1038/345315a0. Nature. 1990. PMID: 2188135 - Immunocytochemical study of dystrophin at the myotendinous junction.
Samitt CE, Bonilla E. Samitt CE, et al. Muscle Nerve. 1990 Jun;13(6):493-500. doi: 10.1002/mus.880130605. Muscle Nerve. 1990. PMID: 2195339 - Plasma membrane cytoskeleton of muscle: a fine structural analysis.
Watkins SC, Cullen MJ, Hoffman EP, Billington L. Watkins SC, et al. Microsc Res Tech. 2000 Feb 1-15;48(3-4):131-41. doi: 10.1002/(SICI)1097-0029(20000201/15)48:3/4<131::AID-JEMT2>3.0.CO;2-G. Microsc Res Tech. 2000. PMID: 10679961 Review. - The dystrophin glycoprotein complex: signaling strength and integrity for the sarcolemma.
Lapidos KA, Kakkar R, McNally EM. Lapidos KA, et al. Circ Res. 2004 Apr 30;94(8):1023-31. doi: 10.1161/01.RES.0000126574.61061.25. Circ Res. 2004. PMID: 15117830 Review.
Cited by
- Exercise-Induced Muscle Damage and Protein Intake: A Bibliometric and Visual Analysis.
Xu F, Zeng J, Liu X, Lai J, Xu J. Xu F, et al. Nutrients. 2022 Oct 14;14(20):4288. doi: 10.3390/nu14204288. Nutrients. 2022. PMID: 36296973 Free PMC article. Review. - Histopathology of Duchenne muscular dystrophy in correlation with changes in proteomic biomarkers.
Zweyer M, Sabir H, Dowling P, Gargan S, Murphy S, Swandulla D, Ohlendieck K. Zweyer M, et al. Histol Histopathol. 2022 Feb;37(2):101-116. doi: 10.14670/HH-18-403. Epub 2021 Dec 7. Histol Histopathol. 2022. PMID: 34873679 Review. - Complexity of skeletal muscle degeneration: multi-systems pathophysiology and organ crosstalk in dystrophinopathy.
Ohlendieck K, Swandulla D. Ohlendieck K, et al. Pflugers Arch. 2021 Dec;473(12):1813-1839. doi: 10.1007/s00424-021-02623-1. Epub 2021 Sep 22. Pflugers Arch. 2021. PMID: 34553265 Free PMC article. Review. - The Dystrophin Node as Integrator of Cytoskeletal Organization, Lateral Force Transmission, Fiber Stability and Cellular Signaling in Skeletal Muscle.
Dowling P, Gargan S, Murphy S, Zweyer M, Sabir H, Swandulla D, Ohlendieck K. Dowling P, et al. Proteomes. 2021 Feb 2;9(1):9. doi: 10.3390/proteomes9010009. Proteomes. 2021. PMID: 33540575 Free PMC article. Review. - Ixazomib, an oral proteasome inhibitor, exhibits potential effect in dystrophin-deficient mdx mice.
Micheletto MLJ, Hermes TA, Bertassoli BM, Petri G, Perez MM, Fonseca FLA, Carvalho AAS, Feder D. Micheletto MLJ, et al. Int J Exp Pathol. 2021 Feb;102(1):11-21. doi: 10.1111/iep.12383. Epub 2020 Dec 9. Int J Exp Pathol. 2021. PMID: 33296126 Free PMC article.
Publication types
MeSH terms
Substances
LinkOut - more resources
Full Text Sources
Other Literature Sources