muscular dystrophies/triacylglycerol

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Fatty acid profile of cardiac muscle phospholipid and triacylglycerol in MDX mice and C57BL/10ScSnJ controls.

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The mdx mouse is a model for Duchenne muscular dystrophy (DMD), a debilitating disease affecting striated muscle. It is established that the fatty acid (FA) composition of skeletal muscle phospholipid (PL) is altered in mdx mice, but it is not known if cardiac muscle is similarly affected by

Failure to up-regulate transcription of genes necessary for muscle adaptation underlies limb girdle muscular dystrophy 2A (calpainopathy).

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Limb girdle muscular dystrophy 2A is due to loss-of-function mutations in the Calpain 3 (CAPN3) gene. Our previous data suggest that CAPN3 helps to maintain the integrity of the triad complex in skeletal muscle. In Capn3 knock-out mice (C3KO), Ca2+ release and Ca2+/calmodulin kinase II (CaMKII)

Insights into lipid accumulation in skeletal muscle in dysferlin-deficient mice.

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Loss of dysferlin protein in humans results in Limb-Girdle Muscular Dystrophy 2B (LGMD2B), characterized by progressive loss of muscles in the distal limbs with impaired locomotion. The Dysferlin null (Bla/J) mouse develops severe steatotic muscles upon aging. Here we report a marked increase in

Changes in oleic acid oxidation and incorporation into lipids of differentiating L6 myoblasts cultured in normal or fatty acid-supplemented growth medium.

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L6 myoblasts accumulate large stores of neutral lipid (predominantly triacylglycerol) when cultured in fatty acid-supplemented growth medium. No accumulation of neutral lipid was evident in myotubes (differentiated myoblasts) when treated similarly. Triacylglycerol accumulation was rapid and

Role of aquaglyceroporins and caveolins in energy and metabolic homeostasis.

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Aquaglyceroporins and caveolins are submicroscopic integral membrane proteins that are particularly abundant in many mammalian cells. Aquaglyceroporins (AQP3, AQP7, AQP9 and AQP10) encompass a subfamily of aquaporins that allow the movement of water, but also of small solutes, such as glycerol,

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