Adaptation of striated muscles to Wolframin deficiency in mice: Alterations in cellular bioenergetics

ConclusionsWfs1-deficiency in both cardiac and skeletal muscles results in functional alterations of energy transport from mitochondria to ATP-ases. There was a substrate-dependent decrease in the maximal Complex I –linked respiratory capacity of the electron transport system in muscles of Wfs1 KO mice. Moreover, in cardiac and gastrocnemius white muscles a decrease in the function of one pathway were balanced by the increase in the activity of the parallel pathway.General significanceThis work provides new insights to the muscle involvement at early stages of metabolic syndrome like WS as well as developing glucose intolerance.
Source: Biochimica et Biophysica Acta (BBA) General Subjects - Category: Biochemistry Source Type: research