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Sites of genetic defects that impair lipid metabolism in muscle

Sites of genetic defects that impair lipid metabolism in muscle
Overview of fat utilization in skeletal muscle indicating sites of genetic defects that impair fat metabolism.
ATGL: adipose triglyceride lipase; Carn: carnitine; CoA: coenzyme A; CPT1: carnitine palmitoyl transferase 1; CPT2: carnitine palmitoyl transferase 2; Cyt c: cytochrome c; enolyl CoA, 3-hydroxy FA-CoA, 3 keto FA-CoA are intermediates in beta oxidation ultimately yielding acetyl-acyl-coenzyme A (for oxidation by the tricarboxylic acid cycle and a fatty acyl-coenzyme A (FA-CoA) shortened by two carbons for further beta oxidation; ETF: electron transfer flavoprotein (receives electrons from the dehydrogenase reaction catalyzed by VLCAD, MCAD and SCAD); ETFDH: electron transfer flavoprotein dehydrogenase (receives electrons from ETF, transfers them to coenzyme Q10 for oxidation via respiratory chain complexes III and IV); FFA: free fatty acid; GCI-58: protein involved in activation of ATGL; LCHAD: long chain hydroxy-acyl-coenzyme A dehydrogenase, one of the three enzymatic reactions (indicated by stars) catalyzed by mitochondrial trifunctional protein; LPIN1: protein involved in triglyceride synthesis; MCAD and SCAD: medium and short chain acyl-coenzyme A dehydrogenase; NADH: nicotinamide adenine dinucleotide; NADH produced in the reaction catalyzed by LCHAD is oxidized via complex I of the respiratory chain; OCTN2: carnitine transporter; Translocase: the carnitine, fatty acyl-coenzyme A transferase; VLCAD: very long chain acyl-coenzyme A dehydrogenase.
Reproduced from: Sharp LJ, Haller RG. Metabolic and mitochondrial myopathies. Neurol Clin 2014; 32:777. Illustration used with the permission of Elsevier Inc. All rights reserved.
Graphic 99532 Version 1.0

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