Lack of anionic phospholipid calcium binding sites in Duchenne muscular dystrophy.
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.
Moggio
M
M
Department of Neurology, Centro Dino Ferrar, University of Milan, Italy.
Fagiolari
G
G
Prelle
A
A
Gallanti
A
A
Sciacco
M
M
Scarlato
G
G
D001665:Binding Sites; D002118:Calcium / Q000378:metabolism*; D002648:Child; D002675:Child, Preschool; D006801:Humans; D007150:Immunohistochemistry; D008297:Male; D009132:Muscles / Q000737:chemistry*; D009136:Muscular Dystrophies / Q000378:metabolism*; D019158:N-Acetylneuraminic Acid; D010743:Phospholipids / Q000378:metabolism*; D012794:Sialic Acids / Q000378:metabolism*
D010743:Phospholipids; D012794:Sialic Acids; D019158:N-Acetylneuraminic Acid; D002118:Calcium