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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
Co enz of homocysteine methly transferase
b12 | N5 methly thf
26
catecholamine syn
ad medulla | cns
27
dihydrobiopterin needed in
1. phenylalanine hydroxylase( to tyrosine) | 2. tyrosine hydroxylase ( to dopa and epi)
28
homocystinuria can be caused by which vit def? how to diff them?
1. b6-cystathionine synthetase:inc homo and methionine 2. b9 andb12-inc homo but dec meth diff: methylmalonate inc in ONLY B12
29
where does hb synthesis occur
1. nucleated rbc | 2. liver
30
acute intermittent porphyria which type of genetic ds?
AD
31
what does photosensitivity in porphyria help to diff?
acute intermittent porphyria :absent | porphyria cutanea tarda:present
32
reg of heme synthesis by
its end product heme only
33
role and inh of ferrocheletase
adds Fe2+ to protoporphyrin to make heme | lead(acquired porphyria)
34
port wine urine
ait | pct
35
why barbiturates C/I in porphyria
increasw cyp450 which req heme dec in its conc further decreases repression of heme synthesis
36
why does rbc flouresce in lead piisoning?
due to formation of Znprotoporphyrin | can also occur in severe fe def
37
why there is no photosensitivity in AIP
because precursors do not cause it
38
ring sideroblast are seen in
B6 def | lead poisoning
39
is CO generated in our body?
yes very little during heme catabolism fe and CO are removed to form biliverdin
40
role of UDPglucuronyl tranferase
adds 2 glucuronic acid ti bilirubin to make it water soluble
41
criglar nijjer and gilbert syndrome
rise in indirect bilirubin which is more in former due to defect in UDPgt
42
normal blood bilirubin levels
<1mg/dl all of which is indirect/unconjugated
43
cause of physiological neonatal jaundie
1. hemolysis:HbF has to be replaced by HbA which can interact with 2,3BPG 2. UDP gt is not ready
44
what type of light is used in phototherapy in neonatal jaundice
blue light | not uv light
45
significance of phototherapy
breaks water insoluble bilirubin to water soluble substance which can be easily excreted in urine even before liver does it
46
which type of blilrubin is present in urine
never present if present-pathological it is conjugated/water soluble one
47
b6
ala synthase cystathione synthase dopa decarboxylase
48
megaloblastic anemia seen in
folate b12 b6 ump synthase
49
purine accumulate when
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
pb inhibits
alasynthase | ferrochelatase
51
drugs precipitating AIP
alcohol phenobarbital gresiofulvin inducer of P450 enz which of which heme synthsis enzymes are a part of
52
propionic acid is formed via
aa- valine , isoleucine , methionine, threonine odd no. fatty acids cholesterol converted to methylmalonic acid ...succinyl CoA
53
importance of N-Acetylglutamate
essential activator of carbamoyl phosphate synthase 1 in urea cycle
54
rate limiting step in urea cycle
carbamoyl phosphate synthase 1

Biochemistry (12 decks)

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