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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
Homocystinuria
Defect in methionine degradation | Char: ectopia lentis, faluty bone, mr,thrombi,MII,stroke in the young
26
cystinuria
defect of renal tubular aa transporter for cystine, ornithine lysine and arg in the PCT Can lead to staghorn calculi
27
MSUD
deficiency of a-ketoacid dehydrogenase complex (blocked degradation of branched amino acids)
28
Porphyrins
cyclic compounds formed by the linkage of 4 pyrrole rings through methyne (-HC) bridges
29
form complexes with the metal ions bound to the nitrogen atom of the pyrrole rings
porphyrins
30
formation of a-Aminolevulinic acid
Glycine + succinyl CoA Enzyme: ALA syntahse Co-factor: pyridoxine
31
Formation of heme
introduction of iron into protoporphyrin IX occurs spontaneously, but the rate is enhanced by ferrochelatase
32
enzymes in formation of bilirubin
HEme oxygenase system of REC
33
enzyme in formation of bilirubin diglucoronide
Bilirubin Glucoronyltransferase
34
lead inactivates what enzymes
ala Dehydratase, ferrochelatase
35
Clinical symptoms of porphyria
photosensitivity, neyropsychiatric symptoms, abdominal pain
36
used to measure bilirubin in serum
Van Der Bergh Reaction
37
indirect hyperbilirubinemia
hemolytic anemias, crigler najar syndrome I and 2, gilbert syndrome
38
direct hyperbilirubinemia
Biliary tree obstruction, dubin-johnson syndrome, rotor syndrome
39
second messenger of insulin
Tyrosine kinase
40
Second messenger of glucagon
cAMP
41
stimulus of secretion of insulin
- ingestion of glucose and amino acids | - Cholecystokinin and gastric-inhibitory polypeptide
42
Stimulus for secretion for glucagon
potential hypoglycemia
43
glucagon has similiar effect with
epinephrine, cortisol, GH
44
glucagon pathway decreased
glycogenesis
45
insulin pathway decreased
gluconeogenesis, glycogenolysis,beta oxidation
46
Estimated average requirement
Avg daily nutrient intake level estimated to meet hte requirement of 50% of healthy individuals in a particular life stage and gender group
47
Recmmended daily allowance
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
Estimated Energy requirement
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
kwashiorkor vs marasmus
Protein vs calorie deprivation presents 1 year after vs less than 1 year edema vs no edema
50
1st sign of Vit A deficiency
loss of sensitivity to green light
51
2 forms of VItamin D
Cholecalciferol D3 and ERgocalciferol D2
52
least toxic of the FAt soluble vitamins
Vitamin E
53
Functions of vit K
coenzyme for y-carboxulation of glutamate residue in clotting factors and other proteins (1972,protein C and S)
54
Thiamine is coenzyme for
pyruvate dehydrogenase, a-ketoglutarate dehydrogenase, branched chain amino acid dehydrogenase, transketolases
55
B1 deficiency
infantile beriberi vs adult beriberi | Wernicke enceph and korsakoff sydrome
56
B2
ETC carriers in redox reactions
57
inhibits lipolysis in adipose tissue and is thus used to treat hyperlipidemia
Niacin (B3)
58
Pellagra (3Ds) ,vitamin deficiency?
diarrhea, dermatitis, dementia, vit B3 (niacin)
59
burning foot syndrome
B5: panthothenic acid
60
only water soluble vitamin with toxicity
b6
61
Active form of B9
tetrahydrofolate
62
biotin is coenzyme for
Pyruvate carboxylase, ACetyl CoA carboxylase, propionyl CoA carboxylase
63
test for B1 deficiency
Erythrocyte Transketolase activity assay
64
test for B6 deficiency
erythrocyte transaminase assay
65
test for B12 deficiency
schiling test, methylamalonic acid test
66
test for folate deficiency
Formiminoglutamic acid (FIGlu) excretion test
67
ferritin
storage form of iron in liver,spleen, BM,intestinal mucosa,etc More abundant when the tissue stores are low
68
hemosiderin
denatured derivative of ferritin, predominates when tissue stores are high
69
transferrin
iron transport protein in plasma
70
acrodematitis enterohepatica
dermatitis, diarrhea and alopecia due to impaired intestinal zinc absorption
71
copper is bound to
Ceruplasmin (60%) | albumin and histidine
72
menkes syndrome
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
Wilson Disease
HEpatolenticular degeneration, blockage of copper excretion is locked (hence accumulation)
74
Treatment for wilson disease
D-penicillamine
75
psychosis and parkinonsm
manganese madness
76
keshan disease
low selenium content with cardiomyopathy
77
Lesch-nyhan Syndrome
Deficiency of HGPRT, gout self mutilation,MR
78
Severe Combined deficiency
Adenosine deaminiase deficiency, lymphocytopenia
79
Von Gierke disease
GLucose 5 phosphatase deficiency
80
B-hydroxybutyric aciduria
Dihydropyrimidine dehydrogenase deficiency

biochem boards (3 decks)

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