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Amino Acids and Proteins

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Total cards: 24•Content version: v4•Public deck•Updated: 8/1/2026

Card preview (24)

#1
Front (Question)

Class of inherited errors of metabolism in which there is an enzyme defect that inhibits the body’s ability to metabolize certain amino acids

Back (Answer)

Aminoacidopathies

#2
Front (Question)

Cause of phenylketonuria

Back (Answer)

Phenylalanine hydroxylase (PAH) deficiency

#3
Front (Question)

Urine odor of patient with phenylketonuria

Back (Answer)

Musty/Mousy odor

#4
Front (Question)

Patients with PKU may experience

Back (Answer)

Mental retardation Microcephaly

#5
Front (Question)

A co-factor in the enzymatic hydroxylation of phenylalanine, tryptophan, and tyrosine

Back (Answer)

Tetrahydrobiopterin

#6
Front (Question)

Non-protein chemical/entity that must bind to a particular enzyme before a reaction occurs

Back (Answer)

Co-factor

#7
Front (Question)

Allows early detection IEM and early implementation of the treatment

Back (Answer)

Newborn screening test

#8
Front (Question)

Goal of PKU treatment

Back (Answer)

Maintain blood level of phenylalanine (2-10 mg/dL or 120- 160 μmol/L)

#9
Front (Question)

First drug to manage PKU

Back (Answer)

Kuvan (sapropterin dihydrochloride)

#10
Front (Question)

Positive result of Ferric Chloride Tube Test for PKU

Back (Answer)

Permanent blue-green color

#11
Front (Question)

Positive result of Guthrie Bacterial Inhibition Test for PKU

Back (Answer)

Bacterial growth

#12
Front (Question)

Inhibitor of B. subtilis

Back (Answer)

B-2-thienylalanine

#13
Front (Question)

Reference method for quantitative serum phenylalanine

Back (Answer)

High-Performance Liquid Chromatography (HPLC)

#14
Front (Question)

Used in screening for inherited disorders in newborns

Back (Answer)

Tandem Mass Spectrometry (MS/MS)

#15
Front (Question)

Characterized by the excretion of tyrosine and tyrosine catabolites in urine

Back (Answer)

Tyrosinemia

#16
Front (Question)

Cause of type 1 tyrosinemia

Back (Answer)

Fumarylacetoacetate hydrolase deficiency

#17
Front (Question)

Most severe form of tyrosinemia

Back (Answer)

Type 1

#18
Front (Question)

Cause of type 2 tyrosinemia

Back (Answer)

Tyrosine aminotransferase deficiency

#19
Front (Question)

Cause of type 3 tyrosinemia

Back (Answer)

4-hydroxyphenylpyruvate dioxygenase or p-hydroxyphenylpyruvic acid dioxygenase deficiency

#20
Front (Question)

Positive result of Ferric chloride tube test for tyrosinemia

Back (Answer)

Transient green color

#21
Front (Question)

Positive result of Nitroso-naphthol test for tyrosinemia

Back (Answer)

Orange-red color

#22
Front (Question)

Drug to treat tyrosinemia

Back (Answer)

Nitrisinone

#23
Front (Question)

Cause of alkaptonuria

Back (Answer)

Homogentisate oxidase deficiency

#24
Front (Question)

Urine color of patient with alkaptonuria

Back (Answer)

Brownish-black after exposure to air