Tubular aggregates: sarcoplasmic reticulum origin, calcium storage ability, and functional implications.
Muscle biopsy specimens from three patients with an autosomal dominant myopathy and tubular aggregates in both type 1 and type 2 fibers were investigated for immunofluorescent staining with antibodies to sarcoplasmic reticulum (SR) Ca-pump protein and calsequestrin and for Ca2+ loading ability. The results show that type 1 and type 2 fibers are differentially reactive to anti-Ca-pump protein IgG and similarly reactive with affinity-purified antibody to calsequestrin, which is in agreement with earlier observations in rat skeletal muscle. Tubular aggregates, which are shown to be highly reactive with either kind of antibody, appear to be sites of calcium accumulation for oxalate-facilitated adenosine triphosphate (ATP) dependent Ca uptake by chemically skinned fibers and thereby increase markedly the Ca loading capacity of the affected fibers.
Salviati
G
G
National Research Council Unit for Muscle Biology and Physiopathology, University of Padova, Italy.
Pierobon-Bormioli
S
S
Betto
R
R
Damiani
E
E
Angelini
C
C
Ringel
S P
SP
Salvatori
S
S
Margreth
A
A
D015153:Blotting, Western / Q000379:methods; D002118:Calcium / Q000378:metabolism*; D000252:Calcium-Transporting ATPases / Q000378:metabolism; D002155:Calsequestrin / Q000378:metabolism; D004797:Enzyme-Linked Immunosorbent Assay / Q000379:methods; D005192:Family Health; D005455:Fluorescent Antibody Technique / Q000379:methods; D006801:Humans; D046529:Microscopy, Electron, Transmission / Q000379:methods; D018485:Muscle Fibers, Skeletal / Q000378:metabolism / Q000473:pathology / Q000648:ultrastructure; D018482:Muscle, Skeletal / Q000378:metabolism / Q000473:pathology / Q000648:ultrastructure; D020914:Myopathies, Structural, Congenital / Q000378:metabolism / Q000473:pathology / Q000503:physiopathology; D012519:Sarcoplasmic Reticulum / Q000378:metabolism / Q000473:pathology / Q000648:ultrastructure; D053498:Sarcoplasmic Reticulum Calcium-Transporting ATPases
D002155:Calsequestrin; D053498:Sarcoplasmic Reticulum Calcium-Transporting ATPases; D000252:Calcium-Transporting ATPases; D002118:Calcium