PRODUCE DA PROTEIN

Question 1

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

Answer 1

isovaleryl CoA dehydrogenase

argininosuccinate synthase, saccharopine dehydrogenase- yes

Question 2

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

Answer 2

met

arg

Question 3

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

Answer 3

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

Question 4

amino acid that prefer transamination as their major means of catabolism

Answer 4

valine

Question 5

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

Answer 5

alanine
cysteine
glycine

Question 6

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

Answer 6

Tryptophan

Question 7

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

Answer 7

glutaminase

Question 8

2 pathways linked in the krebs cycle:

Answer 8

urea cycle

citric acid cycle

Question 9

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

Answer 9

(CPS) Carbamoyl phosphate synthase reaction

(ASS) Argininosuccinate synthetase reaction

Question 10

culprit in the development of coma in severe liver disease

Answer 10

depletion of α-ketoglutarate

Question 11

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

Answer 11

tyrosine

Question 12

causes of PKU except

Answer 12

deficiency of NADH

DEFICIENCY OF Phe monooxygenase and dihydrobiopterin reductase- yes

Question 13

true regarding glycine

Answer 13

glycine is a component of creatine, glutathione and adenine

Question 14

true regarding creatine synthesis

Answer 14

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

Question 15

intracellular protein degradation is made possible by

Answer 15

the use of lysosomes and ubiquitin

Question 16

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

Answer 16

long half lives

Question 17

true regarding meisters cycle

Answer 17

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

Question 18

major enzyme involved in the synthesis of dispensable amino acids

Answer 18

aminotransferase

Question 19

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

Answer 19

alanine
serine
asparagine

Question 20

true regarding oxidative deamination

Answer 20

it always leads to the release of free ammonia

Question 21

true regarding transamination

Answer 21

it always leads to the synthesis of new amino acids

Question 22

entry point of ammonia in the urea cycle

Answer 22

synthesis of carbamoyl phosphate

Question 23

products of decarboxylation of amino acids

Answer 23

GABA
nitric oxide
epinephrine

Question 24

committed steps in the urea cycle

Answer 24

synthesis of urea and ornithine, citrulline, carbamoyl phosphate

Question 25

deficiency of carbamoyl phosphate synthetase 1 leads to

Answer 25

hyperanumonemia type 1

Question 26

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

Answer 26

curcinoid syndrome, hartnups disease, MSUD

Question 27

as amino acids are degraded, their carbons are converted to

Answer 27

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

Question 28

true regarding the catabolism of tryptophan

Answer 28

alanine can be synthesized from the catabolism of tryptophan

Question 29

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

Answer 29

cysteine, alanine, glycine

Question 30

true regarding homocysteine

Answer 30

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

Question 31

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

Answer 31

benzoic acid

Question 32

coenzyme needed in conversion of | glutamic acid 🡆 GABA

Answer 32

pyridoxal PO4

Question 33

transmethylation reactions where S-adenosylmethionine is needed as methyl donor

Answer 33

synthesis of choline

Question 34

true regarding carnosine

Answer 34

used to enhance copper uptake | it can act as neurotransmitter

Question 35

differences between carbamoyl phosphate synthetase 1 and carbamoyl phosphate synthetase 2

Answer 35

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

Question 36

true regarding gamma-aminobutyric acid

Answer 36

deficiency leads to convulsive seizures

Question 37

true regarding the catabolism of branched-chain amino acids

Answer 37

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

Question 38

all dispensable amino acids

Answer 38

can undergo gluconeogenesis can undergo transamination can become limiting amino acids

Question 39

true regarding the kreb-henseleit cycle

Answer 39

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

Question 40

In the treatment of ammonia intoxication..

Answer 40

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

Question 41

true regarding the catabolism of methionine

Answer 41

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

Question 42

true regarding the catabolism of amino acids

Answer 42

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

Question 43

true regarding negative nitrogen balance

Answer 43

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

Question 44

what happens when amino acids undergo catabolism?

Answer 44

alanine 🡆 pyruvate tryptophan 🡆 serotonin phenylalanine 🡆 tyrosine

Question 45

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

Answer 45

maple syrup urine disease | blue diaper syndrome

Question 46

true regarding catabolism of amino acids

Answer 46

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

Question 47

substances derived from the catabolism of amino acids

Answer 47

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

Question 48

indispensible amino acids that can be converted to nonessential amino acids

Answer 48

lysine

Question 49

hyperammonemia is a manifestation of the following clinical conditions

Answer 49

hyperlysinemia, citrullinemia, HHH syndrome

Question 50

glycine is a component of the following substances

Answer 50

heme, creatinine, choline

Question 51

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

Answer 51

alanine, threonine, serine

Question 52

amino acids that undergo non-oxidative deamination

Answer 52

serine, tyrosine

Question 53

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

Answer 53

glutamic acids

Question 54

regarding tyrosinemia type 1

Answer 54

it can be diagnosed thru liver biopsy

Question 55

true regarding MSUD

Answer 55

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

Question 56

negative nitrogen balance can be brought about by

Answer 56

prolonged illness

Question 57

amino acids that are interconvertable

Answer 57

glycine and serine

Question 58

true regarding transamination

Answer 58

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

Question 59

intermediate derived form the catabolism of leucine

Answer 59

a-ketoisocaproic acid, isovaleryl CoA

Question 60

reaction that converts glutamic acid to GABA

Answer 60

decarboxylation

Question 61

aldolase, enolase, aconitase are proteins with what half lives

Answer 61

long ones nigga