Concepts of Genetics (11th Edition)
Concepts of Genetics (11th Edition)
11th Edition
ISBN: 9780321948915
Author: William S. Klug, Michael R. Cummings, Charlotte A. Spencer, Michael A. Palladino
Publisher: PEARSON
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Chapter 14, Problem 12PDQ
Summary Introduction

To determine: The reason individual’s with phenylketonuria do not exhibit deficiency of tyrosine.

Introduction: The inherited disorder that is developed as a result of reduced phenylalanine metabolism is termed as phenylketonuria. The development of phenylketonuria results in many disabilities, including mental disorders and intellectual disabilities.

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Individuals with phenylketonuria cannot convert phenylalanine to tyrosine. Why don’t these individuals exhibit a deficiency of tyrosine?
An enzyme that catalyzes disulfide– sulfhydryl exchange reactions, called protein disulfide isomerase (PDI), has been isolated. PDI rapidly converts inactive scrambled ribonuclease into enzymatically active ribonuclease. In contrast, insulin is rapidly inactivated by PDI. What does this important observation imply about the relation between the amino acid sequence of insulin and its threedimensional structure?
Individuals with phenylketonuria (PKU) are sensitive to phenylalanine in their diet. Why is a warning on foods containing aspartame (L-aspartyl-l-phenylalanine methyl ester) of concern to individuals with PKU?

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Concepts of Genetics (11th Edition)

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