PRODUCE DA PROTEIN Flashcards
hyperammonemia can be brought about by the deficiency of all of the ff enzyme except
isovaleryl CoA dehydrogenase
argininosuccinate synthase, saccharopine dehydrogenase- yes
these amino acids are components of the polyamines– spermidine and spermine
met
arg
What are the similarities between oxidative and non-oxidative deamination?
both lead to the:
release of free ammonia
production of alpha-keto acids
amino acid that prefer transamination as their major means of catabolism
valine
all of the following amino acids enter the citric acid cycle by producing pyruvate first,
then pyruvate 🡆 acetyl CoA
alanine
cysteine
glycine
a loading dose of this amino acid is given to patients to diagnose VIT B6 deficiency
Tryptophan
This enzyme converts glutamine produced in the brain back to glutamic acid in the kidneys
glutaminase
2 pathways linked in the krebs cycle:
urea cycle
citric acid cycle
The entry points of the 2 ammonias that are detoxified per turn in the urea cycle..
(CPS) Carbamoyl phosphate synthase reaction
(ASS) Argininosuccinate synthetase reaction
culprit in the development of coma in severe liver disease
depletion of α-ketoglutarate
non-essential amino acid that can be converted to glucose and ketone bodies
tyrosine
causes of PKU except
deficiency of NADH
DEFICIENCY OF Phe monooxygenase and dihydrobiopterin reductase- yes
true regarding glycine
glycine is a component of creatine, glutathione and adenine
true regarding creatine synthesis
the first reactor involves the transfer of the guanido group of arginine to give glycine
intracellular protein degradation is made possible by
the use of lysosomes and ubiquitin
glucose-6-phosphate, fumarase and glycogen synthase are enzymes that have what kind of half lives
long half lives
true regarding meisters cycle
involves in the transport of amino acids into the cells of the kidney and intestines
major enzyme involved in the synthesis of dispensable amino acids
aminotransferase
amino acids that can be converted to glucose only but not into ketone bodies
alanine
serine
asparagine
true regarding oxidative deamination
it always leads to the release of free ammonia
true regarding transamination
it always leads to the synthesis of new amino acids
entry point of ammonia in the urea cycle
synthesis of carbamoyl phosphate
products of decarboxylation of amino acids
GABA
nitric oxide
epinephrine
committed steps in the urea cycle
synthesis of urea and ornithine, citrulline, carbamoyl phosphate
deficiency of carbamoyl phosphate synthetase 1 leads to
hyperanumonemia type 1
mental retardation is a major manifestation of this/these inborn errors of metabolism
curcinoid syndrome, hartnups disease, MSUD
as amino acids are degraded, their carbons are converted to
carbon dioxide, compounds that produce glucose in the liver, ketone bodies or their precursors
true regarding the catabolism of tryptophan
alanine can be synthesized from the catabolism of tryptophan
non-essential amino acids that can be derived from the catabolism of essential amino acids
cysteine, alanine, glycine
true regarding homocysteine
- degrades and inhibits the formation of collagen, elastin and proteoglycans in the arteries.
- degrades cysteine disulfide bridges in proteins
- regarded as a corrosive substance to the arteries
conjugation of glycine with ___ is considered to be a detoxification mechanism in our body
benzoic acid
coenzyme needed in conversion of
glutamic acid 🡆 GABA
pyridoxal PO4
transmethylation reactions where S-adenosylmethionine is needed as methyl donor
synthesis of choline
true regarding carnosine
used to enhance copper uptake
it can act as neurotransmitter
differences between carbamoyl phosphate synthetase 1 and carbamoyl phosphate synthetase 2
CPS 1 requires an activator, CPS 2 does not require an activator
true regarding gamma-aminobutyric acid
deficiency leads to convulsive seizures
true regarding the catabolism of branched-chain amino acids
it takes place primarily in the brain muscles, the first reaction involved is a transaminidation
all dispensable amino acids
can undergo gluconeogenesis
can undergo transamination
can become limiting amino acids
true regarding the kreb-henseleit cycle
it can be linked to the citric acid cycle thru fumarate
4 ATPS are required per turn and these are used in the 1st and 3rd reactions
In the treatment of ammonia intoxication..
glutamic acid: prevent depletion of a-ketoglutarate
lactulose: prevent absorption of ammonia in the GIT
binding substances: promote excretion of amino acids in the urine
true regarding the catabolism of methionine
leads to the formation of a substance that is “corrosive” to the lining of the arteries
[carnosine]
true regarding the catabolism of amino acids
niacin (B3) is needed as coenzyme in the oxidative deamination of glutamine
true regarding negative nitrogen balance
- indicates that protein turnover is very fast
- it occurs during recuperation from an illness
- indicates a rapid degradation of amino acids
what happens when amino acids undergo catabolism?
alanine 🡆 pyruvate
tryptophan 🡆 serotonin
phenylalanine 🡆 tyrosine
inborn error/s of protein metabolism that is/are manifested by mental retardation
maple syrup urine disease
blue diaper syndrome
true regarding catabolism of amino acids
oxidative deamination, non-oxidative deamination and transamination removes the a-amino group
decarboxylation leads to the production of amines
substances derived from the catabolism of amino acids
the brains natural tranquillizer is derived from from glutamic acid
a neurotransmitter that is a vasoconstrictor
a smooth muscle contractant derived from arginine
indispensible amino acids that can be converted to nonessential amino acids
lysine
hyperammonemia is a manifestation of the following clinical conditions
hyperlysinemia, citrullinemia, HHH syndrome
glycine is a component of the following substances
heme, creatinine, choline
amino acids that is/are first converted to pyruvate before they can undergo gluconeogenesis
alanine, threonine, serine
amino acids that undergo non-oxidative deamination
serine, tyrosine
in most cases of transamination the new amino acid that is formed is
glutamic acids
regarding tyrosinemia type 1
it can be diagnosed thru liver biopsy
true regarding MSUD
the characteristic odor of the urine is due to the excretion of keto acids derived from valine, leucine and isoleucine
negative nitrogen balance can be brought about by
prolonged illness
amino acids that are interconvertable
glycine and serine
true regarding transamination
it involves the transfer of the guanido group of arginine to glycine
intermediate derived form the catabolism of leucine
a-ketoisocaproic acid, isovaleryl CoA
reaction that converts glutamic acid to GABA
decarboxylation
aldolase, enolase, aconitase are proteins with what half lives
long ones nigga
