The consequences of a carnitine deficiency for fatty acid oxidation should be discussed. Concept Introduction: Most of the enzymes of ß oxidation pathway are in the mitochondrial matrix. Short chain fatty acids are transported to the matrix as free acids and convert to acyl CoA derivatives. Long chain fatty acids cannot be transported to the matrix directly. Therefore, they must be converted to acylcarnitine derivatives. Carnitine acyltransferase I is associated with the outer mitochondrial membrane. It catalyzes the formation of O-acylcarnitine. This is transported across the inner mitochondrial membrane by translocase. Then acylcarnitine is transferred to carnitine acyltransferase II on matrix side. This enzyme transfers fatty acyl group back to CoA leaving free carnitine which can return across the membrane via translocase.

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Chapter 23, Problem 10P

Interpretation Introduction

Interpretation:

The consequences of a carnitine deficiency for fatty acid oxidation should be discussed.

Concept Introduction:

Most of the enzymes of ß oxidation pathway are in the mitochondrial matrix. Short chain fatty acids are transported to the matrix as free acids and convert to acyl CoA derivatives. Long chain fatty acids cannot be transported to the matrix directly. Therefore, they must be converted to acylcarnitine derivatives. Carnitine acyltransferase I is associated with the outer mitochondrial membrane. It catalyzes the formation of O-acylcarnitine. This is transported across the inner mitochondrial membrane by translocase. Then acylcarnitine is transferred to carnitine acyltransferase II on matrix side. This enzyme transfers fatty acyl group back to CoA leaving free carnitine which can return across the membrane via translocase.

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