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Biochemistry > Proteins > Flashcards

Proteins Flashcards

1
Q

wats defect in hatrtnup ds

A

.defective transport in intestine and kidney of large neutral aa inc tryptophan
.pellegra like symptoms

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2
Q

cystinuria

A

defect in transport in int and kidney of basic a.a.

cysteine exc in urine..forms cystine(disulfide dimer of cysteine)…..renal stones

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3
Q

drug for cystinuria

A

acetazolamide-raise ph of urine(alkaline) in which cystine is more water soluble
diet low in proteins
drink plenty water

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4
Q

transaminases require which coenz

A

pyridoxine(pyridoxal p)

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5
Q

effect of urea on ph

A

neutal

in contrast ,ammonia is toxic and raises the ph

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6
Q

where are the 2 N used in urea cycle to make urea coming from?

A
  1. aspartate
    2.urea
    -glutamate
    -portal blood NH3(some of the glutamine
    and bacteria)
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7
Q

urea cycle begins in

A

liver mitochondria

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8
Q

eg of 2 obligate activators

A
  1. acetylCoA for pyruvate carboxylase-gluconeogenesis

2. N-acetyl glutamte for carbamoyl phosphate syntheyase 1- urea cycle

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9
Q

is arginine essential aa.

A

yes in children because urea cycle doesnt supply much to meet the demands(dividing cells need arginine like for histones) as in adult.

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10
Q

urea cycle products

A

NH3+HCO3+ATP-carbamoyl phosphate-cp+ornithine-citrulline-comes out in cyto-with aspartate ,arginosuccinate-fumarate+arginine-arginine to urea and ornithine

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11
Q

diff bw carbamoyl phosphate synthetase and ornithine transcarbamoylase

A

orotic aciduria presesnt in latter(pyramidime precursor)also X linked
former-AR

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12
Q

cause of pku

A

def of

  1. phenylalanine hydroxylase
  2. tetrahydrobiopterin
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13
Q

features of pku

A

mental retardation
musty odour
microcephaly
diet

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14
Q

diet of pku

A

can be prevented if dx at birth

  1. diet low in phenylalanine ( not absent as it is essential aa) supplemented with tyrosine
  2. avoid aspartame -sweetener
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15
Q

tyrosine fate

A
  1. melanin

2. krebs

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16
Q

albinism def

A

tyrosinase

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17
Q

alkaptonuria def

A

homogentisate oxidase

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18
Q

alcaptonuria features

A

homogenetisic acid acc
dark urine
ochronosis
arthritis

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19
Q

pink or red urine in

A

porphoriya

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20
Q

brown or tea coloured urine

A

hyperbilirubinemia

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21
Q

tender loving care for nancy req by

A
  1. pyruvate dehydrogenase
  2. a-ketoglutarate dehydrogenase
  3. branched chain ketoacid dehydrogenase
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22
Q

maple syrup ds

A 
urine-maple syrup odour
ment retardation
abnormal m tone
ketosis
coma death
23
Q

name enz req B12

A

1.odd C fatty acid metabolism
methylmalonyl CoA mutase
2.a.a.catabolism of methionine
homocysteine methly transferase/methionine synthetase

24
Q

fate of homocysteine

A

.converted back to methionine

.converted to cystathionine

25
Q

Co enz of homocysteine methly transferase

A

b12

N5 methly thf

26
Q

catecholamine syn

A

ad medulla

cns

27
Q

dihydrobiopterin needed in

A
  1. phenylalanine hydroxylase( to tyrosine)

2. tyrosine hydroxylase ( to dopa and epi)

28
Q

homocystinuria can be caused by which vit def? how to diff them?

A
  1. b6-cystathionine synthetase:inc homo and methionine
  2. b9 andb12-inc homo but dec meth
    diff: methylmalonate inc in ONLY B12
29
Q

where does hb synthesis occur

A
  1. nucleated rbc

2. liver

30
Q

acute intermittent porphyria which type of genetic ds?

A

AD

31
Q

what does photosensitivity in porphyria help to diff?

A

acute intermittent porphyria :absent

porphyria cutanea tarda:present

32
Q

reg of heme synthesis by

A

its end product heme only

33
Q

role and inh of ferrocheletase

A

adds Fe2+ to protoporphyrin to make heme

lead(acquired porphyria)

34
Q

port wine urine

A

ait

pct

35
Q

why barbiturates C/I in porphyria

A

increasw cyp450 which req heme
dec in its conc
further decreases repression of heme synthesis

36
Q

why does rbc flouresce in lead piisoning?

A

due to formation of Znprotoporphyrin

can also occur in severe fe def

37
Q

why there is no photosensitivity in AIP

A

because precursors do not cause it

38
Q

ring sideroblast are seen in

A

B6 def

lead poisoning

39
Q

is CO generated in our body?

A

yes
very little during heme catabolism
fe and CO are removed to form biliverdin

40
Q

role of UDPglucuronyl tranferase

A

adds 2 glucuronic acid ti bilirubin to make it water soluble

41
Q

criglar nijjer and gilbert syndrome

A

rise in indirect bilirubin which is more in former due to defect in UDPgt

42
Q

normal blood bilirubin levels

A

<1mg/dl all of which is indirect/unconjugated

43
Q

cause of physiological neonatal jaundie

A
  1. hemolysis:HbF has to be replaced by HbA which can interact with 2,3BPG
  2. UDP gt is not ready
44
Q

what type of light is used in phototherapy in neonatal jaundice

A

blue light

not uv light

45
Q

significance of phototherapy

A

breaks water insoluble bilirubin to water soluble substance which can be easily excreted in urine even before liver does it

46
Q

which type of blilrubin is present in urine

A

never present
if present-pathological
it is conjugated/water soluble one

47
Q

b6

A

ala synthase
cystathione synthase
dopa decarboxylase

48
Q

megaloblastic anemia seen in

A

folate
b12
b6
ump synthase

49
Q

purine accumulate when

A

dying cell normally release dna and rna
digestion of various types of rna(mRNA etc)
whrn conc of free Pi dec(galactosemia, hereditary fructose intolerance, G6PD def)

50
Q

pb inhibits

A

alasynthase

ferrochelatase

51
Q

drugs precipitating AIP

A

alcohol
phenobarbital
gresiofulvin

inducer of P450 enz which of which heme synthsis enzymes are a part of

52
Q

propionic acid is formed via

A

aa- valine , isoleucine , methionine, threonine

odd no. fatty acids

cholesterol

converted to methylmalonic acid …succinyl CoA

53
Q

importance of N-Acetylglutamate

A

essential activator of carbamoyl phosphate synthase 1 in urea cycle

54
Q

rate limiting step in urea cycle

A

carbamoyl phosphate synthase 1

Biochemistry (12 decks)

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