Chapter 17, Problem 12P

(a)

Summary Introduction

To determine: The reason that why added carnitine increases the rate of oleate oxidation in the patient’s muscle homogenate.

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.

(b)

Summary Introduction

To determine: The reason that why the patient’s symptoms are aggravated by fasting, exercise, and a high-fat diet.

Introduction:

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.

(c)

Summary Introduction

To determine: The two possible reasons for deficiency of muscle carnitine in the individual.

Introduction:

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.