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12.

GENETICS OF METABOLISM

123

I.

Introduction 123

II.

Metabolic Genetic Disorders Involving 
Carbohydrate Pathways 123

III.

Metabolic Genetic Disorders Involving Amino 
Acid Pathways 125

IV.

Metabolic Genetic Disorders Involving Lipid Pathways 126

V.

Metabolic Genetic Disorders Involving the Urea 
Cycle Pathway 128

VI.

Metabolic Genetic Disorders Involving 
Transport Pathways 128

VII.

Metabolic Genetic Disorders Involving 
Degradation Pathways 130

VIII.

Summary Tables of Metabolic Genetic Disorders 131

IX.

Selected Photographs of Metabolic Genetic Disorders 131

Review Test

138

Answers and Explanations

139

13.

GENETICS OF HEMOGLOBINOPATHIES

140

I.

Characteristics of Hemoglobin (Hb) 140

II.

Sickle Cell Disease (SCD) 140

III.

Alpha-Thalassemia 141

IV.

Beta-Thalassemia 142

V.

Summary Table of Hemoglobinopathies 143

VI.

Selected Photomicrographs of Hemoglobinopathies 144

Review Test

145

Answers and Explanations

146

14.

GENETICS OF BLEEDING DISORDERS

147

I.

Hemophilia A (Factor VIII Deficiency) 147

II.

Hemophilia B (Factor IX Deficiency; Christmas Disease) 148

III.

von Willebrand Disease (VWD) 148

IV.

Summary Table of Laboratory Findings in 
Bleeding Disorders 149

V.

Summary Table of Bleeding Disorders 150

Review Test

151

Answers and Explanations

152

15.

GENETICS OF DEVELOPMENT

153

I.

Causes of Human Birth Defects 153

II.

Types of Human Birth Defects 153

III.

Patterns of Human Birth Defects 154

IV.

Determination of the Left/Right (L/H) Axis 154

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Contents

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