Defects in fatty acid metabolism leads to a heterogenous group of inherited metabolic disorders due to impairment of mitochondrial beta oxidation pathway resulting in accumulation of lipid droplets within the muscle fibers. The molecular events underlying impaired lipid storage and mitochondrial changes have not been completely understood. Hence, biochemical analysis of mitochondrial function on skeletal muscle biopsies of five patients diagnosed based on morphological findings as lipid storage disorder (LSD) was carried out.

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