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36 Cards in this Set

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essential amino acids
Phenylalanine
Valine
Tryptophan
Threonine
Isoleucine
Methionine
Histidine*
Arginine*
Leucine
Lysine

PVT TIM HALL

* - required during periods of growth
ketogenic amino acids
leucine
lysine
essential, glucogenic/ketogenic amino acids
phenylalanine
tryptophan
isoleucine
essential, glucogenic amino acids
valine
threonine
methionine
histidine
arginine
most basic amino acid
arginine
phenylalanine can be converted into
tyrosine
tyrosine can be converted into (2)
DOPA
thyroxine
tryptophan can be converted into (3)
5-HT
melatonin
niacin (vit. B3)
histidine can be converted into
histamine
glycine can be converted into
porphyrin
arginine can be converted into (3)
urea
creatine
NO
glutamate can be converted into

enzyme used and cofactor
GABA

glutamate decarboxylase; pyridoxine (vit. B6)
treatment for hyperammonemia
arginine
enzymes of the urea cycle found in the mitochondrial matrix
carbamoyl phosphate synthase I
ornithine transcarbamoylase
rate-limiting step in the urea cycle
carbamoyl phosphate synthase I (CO2 + NH4 --> carbamoyl phosphate)
urea cycle product formed in the mitochondria that enters the cytoplasm
citruline
urea cycle product formed in the cytoplasm that enter the mitochondria
ornithine
amino acid that enters the urea cycle
aspartate
steps in the urea cycle
ornithine + carbamoyl phosphate --> citruline --> arginosuccinate --> fumarate --> arginine --> urea

Ornery Careless Crappers Are Frivolous About Urine
enzyme deficiency in PKU
phenylalanine hydroxylase
cofactor deficiency in PKU
tetrahydrobiopterin
disease:

mental retardation
growth retardation
fair skin
eczema
must body odor
PKU

(due to build up of phenylketones: phenylacetate, phenyllactate, and phenylpyruvate)
PKU:

MOI
AR
enzyme deficiency in alkaptonuria
homogentisic acid oxidase
function of homogentisic acid oxidase
breaks down tyrosine
disease:

urine turns black on standing
dark CT
debilitating arthralgias
alkaptonuria
albinism:

enzyme deficiency

MOI
tyrosinase

AR
defective transporter in albinism
tyrosine transporter
enzyme deficiencies in homocystinuria (2)
methionine synthase
cystathionine synthase (Tx: ↑ cysteine in diet)
cystathionine synthase cofactor
pyridoxal phosphate (vit. B6)
disease:

mental retardation
osteoporosis
tall stature
kyphosis
lens sublaxation (down and in)
atherosclerosis --> stroke, MI
homocystinuria
cytinuria due to
defect of renal tubular amino acid transporter
amino acids not reabsorded in cystinuria
Cystine
Ornithine
Lysine
Arginine

COLA
Tx for cytinuria
acetazolamide

(alkalinizes the urine to prevent formation of cystine kidney stones)
enzyme deficiency in maple syrup urine disease
α-ketoacid dehydrogenase
amino acids affected by maple syrup urine disease (3)
valine
leucine
isoleucine