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biochem boards > Day 2- Biochem Top Notch > Flashcards

Day 2- Biochem Top Notch Flashcards

1
Q

first phase of AA Catabolism

A
  • deamination

- forming ammonia and a-ketoacid

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2
Q

what can be used for 2nd phase of AA catabolism

A

intermediates of energy producing -metabolic pathways (glycolysis, krebs cycle)

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3
Q

Ureotelic

A

water soluble urea

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4
Q

Transamination: AA transfer their a-amino ketoglutarate, proper pairing with enzyme

A 
1. Alanine aminotransferase 
Alanine: - pyruvate
SGPT-ALT
2. Aspartate amino transferase
aspartate - oxaloacetate
SGOT- AST
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5
Q

oxidative deamination enzyme

A

Glutamate dehydrogenase

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6
Q

Transport of ammonia from peripheral tissues through glutamine through which enzyme

A

glutamine synthase

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7
Q

urea cycle occurs where

A

both mitochondria and cytosol,happens at the liver only

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8
Q

substrates for urea cycle

A

NH3, aspartate,CO2

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9
Q

RAte limiting step of Urea cycle?

A

NH3 + CO2 -> Carbamoyl phosphate

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10
Q

Urea cycle: Enzyme of rate - limiting step

A

Carbamoyl phosphate synthetase 1

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11
Q

Hereditary Hyperammonemia:

A

Most common: ornithine transcarbomylase defiiency

CArbamoyl Phosphate synthase-I deficiency

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12
Q

Purely Ketogenic AA

A

Lysine, leucine

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13
Q

Glucogenic and ketogenic AA

A

Phenylalanine tyrosine isoleucine tweetophan

TRY PHilippine ISlands TY

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14
Q

Specialized product of arginine

A

Creatinine, polyamines, NO

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15
Q

Specialized product of Cysteine

A

Coenzyme A, Taurine, gluthathione

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16
Q

Specialized product of Glutamate

A

GABA,glutathione

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17
Q

Specialized product of Glycine

A

heme,purines,craetine,glutathione, bile acids

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18
Q

Specialized product of Histidine

A

histamine

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19
Q

Methionine

A

S- adenosylmethionine,

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20
Q

Serine

A

SPhingosine, purines,thymine

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21
Q

Tryptophan

A

Serotonin, niacin, melatonin

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22
Q

Tyrosine

A

catecholamines, thyroid hormones, melanin

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23
Q

Phenylketonuria

A

decrease in phenylalamine hydroxylase or tertahydrobiopterin

char: Severe MR, fair skin,eczema

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24
Q

Alkaptonuiria:

A

deficiencyt of homogentisate oxidase in the degradative pathway of tyrosine,build of homogenistic acid

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25
Q

Homocystinuria

A

Defect in methionine degradation

Char: ectopia lentis, faluty bone, mr,thrombi,MII,stroke in the young

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26
Q

cystinuria

A

defect of renal tubular aa transporter for cystine, ornithine lysine and arg in the PCT
Can lead to staghorn calculi

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27
Q

MSUD

A

deficiency of a-ketoacid dehydrogenase complex (blocked degradation of branched amino acids)

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28
Q

Porphyrins

A

cyclic compounds formed by the linkage of 4 pyrrole rings through methyne (-HC) bridges

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29
Q

form complexes with the metal ions bound to the nitrogen atom of the pyrrole rings

A

porphyrins

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30
Q

formation of a-Aminolevulinic acid

A

Glycine + succinyl CoA
Enzyme: ALA syntahse
Co-factor: pyridoxine

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31
Q

Formation of heme

A

introduction of iron into protoporphyrin IX occurs spontaneously, but the rate is enhanced by ferrochelatase

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32
Q

enzymes in formation of bilirubin

A

HEme oxygenase system of REC

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33
Q

enzyme in formation of bilirubin diglucoronide

A

Bilirubin Glucoronyltransferase

34
Q

lead inactivates what enzymes

A

ala Dehydratase, ferrochelatase

35
Q

Clinical symptoms of porphyria

A

photosensitivity, neyropsychiatric symptoms, abdominal pain

36
Q

used to measure bilirubin in serum

A

Van Der Bergh Reaction

37
Q

indirect hyperbilirubinemia

A

hemolytic anemias, crigler najar syndrome I and 2, gilbert syndrome

38
Q

direct hyperbilirubinemia

A

Biliary tree obstruction, dubin-johnson syndrome, rotor syndrome

39
Q

second messenger of insulin

A

Tyrosine kinase

40
Q

Second messenger of glucagon

A

cAMP

41
Q

stimulus of secretion of insulin

A
  • ingestion of glucose and amino acids

- Cholecystokinin and gastric-inhibitory polypeptide

42
Q

Stimulus for secretion for glucagon

A

potential hypoglycemia

43
Q

glucagon has similiar effect with

A

epinephrine, cortisol, GH

44
Q

glucagon pathway decreased

A

glycogenesis

45
Q

insulin pathway decreased

A

gluconeogenesis, glycogenolysis,beta oxidation

46
Q

Estimated average requirement

A

Avg daily nutrient intake level estimated to meet hte requirement of 50% of healthy individuals in a particular life stage and gender group

47
Q

Recmmended daily allowance

A

Avg daily ditary intake levl that is sufficient to meet the requirments of >95% of all individuals in a life stage and gender group

48
Q

Estimated Energy requirement

A

average dietary energy intake predicted to maintain an energy balance in a healthy adult of a defined age,gender and height whose weight and level of physical activity are consistent with good health

49
Q

kwashiorkor vs marasmus

A

Protein vs calorie deprivation
presents 1 year after vs less than 1 year
edema vs no edema

50
Q

1st sign of Vit A deficiency

A

loss of sensitivity to green light

51
Q

2 forms of VItamin D

A

Cholecalciferol D3 and ERgocalciferol D2

52
Q

least toxic of the FAt soluble vitamins

A

Vitamin E

53
Q

Functions of vit K

A

coenzyme for y-carboxulation of glutamate residue in clotting factors and other proteins (1972,protein C and S)

54
Q

Thiamine is coenzyme for

A

pyruvate dehydrogenase, a-ketoglutarate dehydrogenase, branched chain amino acid dehydrogenase, transketolases

55
Q

B1 deficiency

A

infantile beriberi vs adult beriberi

Wernicke enceph and korsakoff sydrome

56
Q

B2

A

ETC carriers in redox reactions

57
Q

inhibits lipolysis in adipose tissue and is thus used to treat hyperlipidemia

A

Niacin (B3)

58
Q

Pellagra (3Ds) ,vitamin deficiency?

A

diarrhea, dermatitis, dementia, vit B3 (niacin)

59
Q

burning foot syndrome

A

B5: panthothenic acid

60
Q

only water soluble vitamin with toxicity

A

b6

61
Q

Active form of B9

A

tetrahydrofolate

62
Q

biotin is coenzyme for

A

Pyruvate carboxylase, ACetyl CoA carboxylase, propionyl CoA carboxylase

63
Q

test for B1 deficiency

A

Erythrocyte Transketolase activity assay

64
Q

test for B6 deficiency

A

erythrocyte transaminase assay

65
Q

test for B12 deficiency

A

schiling test, methylamalonic acid test

66
Q

test for folate deficiency

A

Formiminoglutamic acid (FIGlu) excretion test

67
Q

ferritin

A

storage form of iron in liver,spleen, BM,intestinal mucosa,etc
More abundant when the tissue stores are low

68
Q

hemosiderin

A

denatured derivative of ferritin, predominates when tissue stores are high

69
Q

transferrin

A

iron transport protein in plasma

70
Q

acrodematitis enterohepatica

A

dermatitis, diarrhea and alopecia due to impaired intestinal zinc absorption

71
Q

copper is bound to

A

Ceruplasmin (60%)

albumin and histidine

72
Q

menkes syndrome

A

x-linked recessive. deficiency of an ATP dependent membrane transporter for copper
presents as growth retardation, MR,seizures, arterial aneurysms,brittle hair, bone diminerlization

73
Q

Wilson Disease

A

HEpatolenticular degeneration, blockage of copper excretion is locked (hence accumulation)

74
Q

Treatment for wilson disease

A

D-penicillamine

75
Q

psychosis and parkinonsm

A

manganese madness

76
Q

keshan disease

A

low selenium content with cardiomyopathy

77
Q

Lesch-nyhan Syndrome

A

Deficiency of HGPRT, gout self mutilation,MR

78
Q

Severe Combined deficiency

A

Adenosine deaminiase deficiency, lymphocytopenia

79
Q

Von Gierke disease

A

GLucose 5 phosphatase deficiency

80
Q

B-hydroxybutyric aciduria

A

Dihydropyrimidine dehydrogenase deficiency

biochem boards (3 decks)

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