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| LOCALIZATION AND QUANTITATION OF THE CHROMOSOME 6-ENCODED DYSTROPHIN-RELATED PROTEIN IN NORMAL AND PATHOLOGICAL HUMAN MUSCLE |
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| Author(s): KARPATI G, CARPENTER S, MORRIS GE, DAVIES KE, GUERIN C, HOLLAND P |
| Source: JOURNAL OF NEUROPATHOLOGY AND EXPERIMENTAL NEUROLOGY Volume: 52 Issue: 2 Pages: 119-128 Published: MAR 1993 |
| Times Cited: 113 References: 27 |
| Abstract: A dystrophin-related protein (DRP) encoded by a gene on chromosome 6 was studied in 14 normal and 79 pathological human skeletal muscle biopsies, as well as in cultured myotubes by light microscopic immunocytochemistry and quantitative immunoblots. In normal muscle immunoreactive DRP was present at the postjunctional surface membrane, at the surface of satellite cells, in the walls of blood vessels, in Schwann cells and in perineurium of intramuscular nerves. All of this produced a weak signal on immunoblots. In Duchenne/Becker dystrophy (DMD/BMD) and in polymyositis (PM) or dermatomyositis (DM) DRP was present throughout the extrajunctional surface membrane of extra- and intrafusal muscle fibers, particularly regenerating ones. This produced a 15-17-fold increase of DRP over normal in DMD/BMD and 4-10-fold increase over normal in PM and DM on immunoblots. In other pathological muscles, DRP localization pattern and quantity was about the same as in normals. Dystrophin-related protein was present in about the same amounts and distribution in normal and DMD cultured myoblasts and myotubes. The molecular stimulus for the marked upregulation of DRP in DMD/BMD and in the inflammatory myopathies is not known. In DMD/BMD the diffuse sarcolemmal DRP may partially compensate for dystrophin deficiency. |
| Document Type: Article |
| Language: English |
| Reprint Address: KARPATI, G (reprint author), MONTREAL NEUROL HOSP & INST, 3801 UNIV ST, MONTREAL H3A 2B4, QUEBEC CANADA |
Addresses:
1. MCGILL UNIV, DEPT NEUROL & NEUROSURG, MONTREAL H3A 2T5, QUEBEC CANADA
2. NE WALES INST, DIV RES, DEESIDE, CLWYD WALES
3. JOHN RADCLIFFE HOSP, INST MOLEC MED, MOLEC GENET GRP, OXFORD OX3 9DU, ENGLAND |
| Publisher: AMER ASSN NEUROPATHOLOGISTS INC, 1041 NEW HAMPSHIRE ST, LAWRENCE, KS 66044 |
| Subject Category: Clinical Neurology; Neurosciences; Pathology |
| IDS Number: KQ771 |
| ISSN: 0022-3069 |
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| | | | | | | | | Record from Web of Science® | | | | | | | | | |