PRODUCE DA PROTEIN Flashcards

1
Q

hyperammonemia can be brought about by the deficiency of all of the ff enzyme except

A

isovaleryl CoA dehydrogenase

argininosuccinate synthase, saccharopine dehydrogenase- yes

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2
Q

these amino acids are components of the polyamines– spermidine and spermine

A

met

arg

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3
Q

What are the similarities between oxidative and non-oxidative deamination?

A

both lead to the:
release of free ammonia
production of alpha-keto acids

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4
Q

amino acid that prefer transamination as their major means of catabolism

A

valine

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5
Q

all of the following amino acids enter the citric acid cycle by producing pyruvate first,
then pyruvate 🡆 acetyl CoA

A

alanine
cysteine
glycine

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6
Q

a loading dose of this amino acid is given to patients to diagnose VIT B6 deficiency

A

Tryptophan

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7
Q

This enzyme converts glutamine produced in the brain back to glutamic acid in the kidneys

A

glutaminase

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8
Q

2 pathways linked in the krebs cycle:

A

urea cycle

citric acid cycle

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9
Q

The entry points of the 2 ammonias that are detoxified per turn in the urea cycle..

A

(CPS) Carbamoyl phosphate synthase reaction

(ASS) Argininosuccinate synthetase reaction

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10
Q

culprit in the development of coma in severe liver disease

A

depletion of α-ketoglutarate

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11
Q

non-essential amino acid that can be converted to glucose and ketone bodies

A

tyrosine

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12
Q

causes of PKU except

A

deficiency of NADH

DEFICIENCY OF Phe monooxygenase and dihydrobiopterin reductase- yes

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13
Q

true regarding glycine

A

glycine is a component of creatine, glutathione and adenine

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14
Q

true regarding creatine synthesis

A

the first reactor involves the transfer of the guanido group of arginine to give glycine

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15
Q

intracellular protein degradation is made possible by

A

the use of lysosomes and ubiquitin

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16
Q

glucose-6-phosphate, fumarase and glycogen synthase are enzymes that have what kind of half lives

A

long half lives

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17
Q

true regarding meisters cycle

A

involves in the transport of amino acids into the cells of the kidney and intestines

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18
Q

major enzyme involved in the synthesis of dispensable amino acids

A

aminotransferase

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19
Q

amino acids that can be converted to glucose only but not into ketone bodies

A

alanine
serine
asparagine

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20
Q

true regarding oxidative deamination

A

it always leads to the release of free ammonia

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21
Q

true regarding transamination

A

it always leads to the synthesis of new amino acids

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22
Q

entry point of ammonia in the urea cycle

A

synthesis of carbamoyl phosphate

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23
Q

products of decarboxylation of amino acids

A

GABA
nitric oxide
epinephrine

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24
Q

committed steps in the urea cycle

A

synthesis of urea and ornithine, citrulline, carbamoyl phosphate

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25
Q

deficiency of carbamoyl phosphate synthetase 1 leads to

A

hyperanumonemia type 1

26
Q

mental retardation is a major manifestation of this/these inborn errors of metabolism

A

curcinoid syndrome, hartnups disease, MSUD

27
Q

as amino acids are degraded, their carbons are converted to

A

carbon dioxide, compounds that produce glucose in the liver, ketone bodies or their precursors

28
Q

true regarding the catabolism of tryptophan

A

alanine can be synthesized from the catabolism of tryptophan

29
Q

non-essential amino acids that can be derived from the catabolism of essential amino acids

A

cysteine, alanine, glycine

30
Q

true regarding homocysteine

A
  1. degrades and inhibits the formation of collagen, elastin and proteoglycans in the arteries.
  2. degrades cysteine disulfide bridges in proteins
  3. regarded as a corrosive substance to the arteries
31
Q

conjugation of glycine with ___ is considered to be a detoxification mechanism in our body

A

benzoic acid

32
Q

coenzyme needed in conversion of

glutamic acid 🡆 GABA

A

pyridoxal PO4

33
Q

transmethylation reactions where S-adenosylmethionine is needed as methyl donor

A

synthesis of choline

34
Q

true regarding carnosine

A

used to enhance copper uptake

it can act as neurotransmitter

35
Q

differences between carbamoyl phosphate synthetase 1 and carbamoyl phosphate synthetase 2

A

CPS 1 requires an activator, CPS 2 does not require an activator

36
Q

true regarding gamma-aminobutyric acid

A

deficiency leads to convulsive seizures

37
Q

true regarding the catabolism of branched-chain amino acids

A

it takes place primarily in the brain muscles, the first reaction involved is a transaminidation

38
Q

all dispensable amino acids

A

can undergo gluconeogenesis
can undergo transamination
can become limiting amino acids

39
Q

true regarding the kreb-henseleit cycle

A

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

40
Q

In the treatment of ammonia intoxication..

A

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

41
Q

true regarding the catabolism of methionine

A

leads to the formation of a substance that is “corrosive” to the lining of the arteries
[carnosine]

42
Q

true regarding the catabolism of amino acids

A

niacin (B3) is needed as coenzyme in the oxidative deamination of glutamine

43
Q

true regarding negative nitrogen balance

A
  1. indicates that protein turnover is very fast
  2. it occurs during recuperation from an illness
  3. indicates a rapid degradation of amino acids
44
Q

what happens when amino acids undergo catabolism?

A

alanine 🡆 pyruvate
tryptophan 🡆 serotonin
phenylalanine 🡆 tyrosine

45
Q

inborn error/s of protein metabolism that is/are manifested by mental retardation

A

maple syrup urine disease

blue diaper syndrome

46
Q

true regarding catabolism of amino acids

A

oxidative deamination, non-oxidative deamination and transamination removes the a-amino group
decarboxylation leads to the production of amines

47
Q

substances derived from the catabolism of amino acids

A

the brains natural tranquillizer is derived from from glutamic acid
a neurotransmitter that is a vasoconstrictor
a smooth muscle contractant derived from arginine

48
Q

indispensible amino acids that can be converted to nonessential amino acids

A

lysine

49
Q

hyperammonemia is a manifestation of the following clinical conditions

A

hyperlysinemia, citrullinemia, HHH syndrome

50
Q

glycine is a component of the following substances

A

heme, creatinine, choline

51
Q

amino acids that is/are first converted to pyruvate before they can undergo gluconeogenesis

A

alanine, threonine, serine

52
Q

amino acids that undergo non-oxidative deamination

A

serine, tyrosine

53
Q

in most cases of transamination the new amino acid that is formed is

A

glutamic acids

54
Q

regarding tyrosinemia type 1

A

it can be diagnosed thru liver biopsy

55
Q

true regarding MSUD

A

the characteristic odor of the urine is due to the excretion of keto acids derived from valine, leucine and isoleucine

56
Q

negative nitrogen balance can be brought about by

A

prolonged illness

57
Q

amino acids that are interconvertable

A

glycine and serine

58
Q

true regarding transamination

A

it involves the transfer of the guanido group of arginine to glycine

59
Q

intermediate derived form the catabolism of leucine

A

a-ketoisocaproic acid, isovaleryl CoA

60
Q

reaction that converts glutamic acid to GABA

A

decarboxylation

61
Q

aldolase, enolase, aconitase are proteins with what half lives

A

long ones nigga