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Tytuł pozycji:

Altered calcium pump and secondary deficiency of γ-sarcoglycan and microspan in sarcoplasmic reticulum membranes isolated from δ-sarcoglycan knockout mice.

Tytuł :
Altered calcium pump and secondary deficiency of γ-sarcoglycan and microspan in sarcoplasmic reticulum membranes isolated from δ-sarcoglycan knockout mice.
Autorzy :
Solares-Pérez, Alhondra
Álvarez, Rocío
Crosbie, Rachelle H.
Vega-Moreno, Jesús
Medina-Monares, Joel
Estrada, Francisco J.
Ortega, Alicia
Coral-Vazquez, Ramón
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Temat :
CALCIUM channels
MEMBRANE proteins
SARCOPLASMIC reticulum
LABORATORY mice
MUSCULAR dystrophy
GENE expression
GENETIC mutation
IMMUNOBLOTTING
Źródło :
Cell Calcium; Jul2010, Vol. 48 Issue 1, p28-36, 9p
Czasopismo naukowe
Abstract: Sarcoglycans (SGs) and sarcospan (SSPN) are transmembrane proteins of the dystrophin-glycoprotein complex. Mutations in the genes encoding SGs cause many inherited forms of muscular dystrophy. In this study, using purified membranes of wild-type (WT) and δ-SG knockout (KO) mice, we found the specific localization of the SG-SSPN isoforms in transverse tubules (TT) and sarcoplasmic reticulum (SR) membranes. Immunoblotting revealed that the absence of δ-SG isoforms in TT and SR results in a secondary deficiency of γ-SG and μSPN. Our results showed augmented ATP hydrolytic activity, ATP-dependent calcium uptake and passive calcium efflux, probably through SERCA1 in KO compared to WT mice. Furthermore, we found a conformational change in SERCA1 isolated from KO muscle as demonstrated by calorimetric analysis. Following these alterations with mechanical properties, we found an increase in force in KO muscle with the same rate of fatigue but with a decreased fatigue recovery compared to WT. Together our observations suggest, for the first time, that the δ-SG isoforms may stabilize the expression of γ-SG and μSPN in the TT and SR membranes and that this possible complex may play a role in the maintenance of a stable level of resting cytosolic calcium concentration in skeletal muscle. [ABSTRACT FROM AUTHOR]
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