Chapter 17, Problem 11P

Summary Introduction

To determine: The changes in metabolic pattern that would result from mutation in the muscle $\text{carnitine acyltransferase-1}$ in which the mutant protein has lost its affinity for $\text{malonyl-CoA}$ but not its catalytic activity.

Introduction:

Shuttle system having intermediate fatty acyl-carnitine is needed to transport molecules of fatty acid into mitochondria. Before transportation of fatty acid molecules, fatty acids are first transformed into fatty-acyl coenzyme A in the cytoplasm which is done by $\text{acyl-CoA}$ synthetase. After that fatty acyl groups are transported to carnitine present at the outer mitochondrial membrane by $\text{carnitine acyltransferase-1}$ enzyme.