Beta-oxidation

β-oxidation may be defined as an oxidation of fatty acids on the β-carbon atom. This results in the sequential removal of a two carbon fragment, acetyl CoA.

Three stages:

1. Activation of fatty acids to Acyl CoA, occurring in the cytosol
2. Transport of acyl CoA into mitochondria
3. β-oxidation proper in the mitochondrial matrix

To differentiate between myopathic CAT/CPT deficiency and McArdles disease

both presents as a child with muscle aches, weakness and myoglobinuria especially provoked by exercise

Next step is to do - Muscle biopsy and then,

"Oil Red O" staining for lipid => CAT/CPT deficiency (N.B. another staining for lipids is sudan black)

PAS stain for carbohydrate material/glycogen => McArdles

MCAD deficiency

Fatty acyl-CoA dehydrogenase (LCAD, MCAD)

links with SIDS