Acyl carnitine functions in:

Ans: a (Transport of long....) Ref: Vasudevan, 4th ed, p. 129The long chain fatty acyl CoA cannot pass through the inner mitochondrial membrane. There fore a transporter, carnitine is involved in transfer of fatty acids.Beta oxidation:Beta oxidation is absent in brain and erythrocytes (because erythrocytes lack mitochondria and fatty acids do not cross BBB).Carnitine acyl transferase -1 is inhibited by Malonyl CoA.Clinical correlate:Carnitine acyl transferase(CAT) deficiency (myopathic form)Although all tissues contain CAT most common form of genetic deficiency is myopathic form and due to a defect in muscle specific CAT gene.Features:Muscle aches, red urineRhabdomyolysis, myoglobinuriaProvoked by exerciseIncreased by high fat; low carbohydrate dietMuscle biopsy: increased muscle triglyceride in cytoplasmTreatment: Cease muscle activity, give glucose.