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Lack of anionic phospholipid calcium binding sites in duchenne muscular dystrophy
Author(s) -
Moggio Maurizio,
Fagiolari Gigliola,
Prelle Alessandro,
Gallanti Andrea,
Sciacco Monica,
Scarlato Guglielmo
Publication year - 1992
Publication title -
muscle and nerve
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.025
H-Index - 145
eISSN - 1097-4598
pISSN - 0148-639X
DOI - 10.1002/mus.880150311
Subject(s) - calcium , duchenne muscular dystrophy , sarcolemma , muscular dystrophy , basal lamina , chemistry , endocrinology , itga7 , medicine , biology , skeletal muscle , biochemistry , anatomy , ultrastructure
We studied membrane ultrastructural localization of anionic phospholipids (AP) and sialic acid (SA) calcium binding sites in muscle biopsies from Duchenne muscular dystrophy (DMD) and 3 Becker's muscular dystrophy (BMD) patients using polymyxin B (PXB) and limulus polyphemus (LP) as cytochemical markers. We found that AP calcium binding sites are lacking at muscle cell surface in all DMD muscle tissues, in both intact and degenerating muscle fibers. In BMD, AP have an unusual distribution along plasma membrane. Sialic acid calcium binding sites have the same localization along plasma membrane and basal lamina in DMD, BMD, and control muscles. The absence or alterations of structures involved in calcium binding in DMD and BMD may alter membrane calcium permeability, leading to abnormal Ca 2+ influx into cells causing muscle necrosis.