Lack of anionic phospholipid calcium binding sites in Duchenne muscular dystrophy.

Abstract:

: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 Ca2+ influx into cells causing muscle necrosis.

journal_name

Muscle Nerve

journal_title

Muscle & nerve

authors

Moggio M,Fagiolari G,Prelle A,Gallanti A,Sciacco M,Scarlato G

doi

10.1002/mus.880150311

subject

Has Abstract

pub_date

1992-03-01 00:00:00

pages

325-31

issue

3

eissn

0148-639X

issn

1097-4598

journal_volume

15

pub_type

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