Definition of the following terminologies: (a) Albinism (b) Maple syrup urine disease (c) Alkaptonuria (d) Methylmalonic acidemia (e) Phenylketonuria Introduction: The catabolism of various amino acids requires enzymesand is necessary for the proper functioning of different biological processes. The various end products released after catabolism of amino acids are acetyl CoA (coenzyme A), pyruvate, acetoacetyl CoA, and several intermediates of the citric acid cycle such as alpha-ketoglutarate, succinyl CoA, and oxaloacetate. Enzymes are function optimally in healthy individuals. The disorders associated with amino acid metabolism are genetic and occurmainly due to mutations in genes that produce defective enzymes.

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Chapter 15, Problem 8RQ

Summary Introduction

To review:

Definition of the following terminologies:

(a) Albinism

(b) Maple syrup urine disease

(c) Alkaptonuria

(d) Methylmalonic acidemia

(e) Phenylketonuria

Introduction:

The catabolism of various amino acids requires enzymesand is necessary for the proper functioning of different biological processes. The various end products released after catabolism of amino acids are acetyl CoA (coenzyme A), pyruvate, acetoacetyl CoA, and several intermediates of the citric acid cycle such as alpha-ketoglutarate, succinyl CoA, and oxaloacetate. Enzymes are function optimally in healthy individuals. The disorders associated with amino acid metabolism are genetic and occurmainly due to mutations in genes that produce defective enzymes.

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