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Biochemistry > Biochem from DIT > Flashcards

Biochem from DIT Flashcards

1
Q

to make purine

RLS?

A 
glycine - C
glutamine - N
Aspartate - N
THF - C
CO2 - 2

PRPP synthetase, Glutamine PRPP amidotransferase (RLS), IMP dehydrogenase

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2
Q

to make pyrimidine

RLS?

A

carbamoyl P

  • glutamine - NH2
  • CO2 - C=O
  • ATP - P

+ aspartate via CPSII

becomes orotic acid
then UMP to UDP to CTP
or dUDP to dTMP

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3
Q

C minus NH3

A

U

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4
Q

CPSI

A

urea cycle, in mitochondria, needs ammonia

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5
Q

orotic aciduria

A

defect in UMP synthase in pyrimidine synth
causes megaloblastic anemia, orotic acid in urine
NO increase in blood ammonia vs. OTC def

treat with uridine supplements

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6
Q

Hydroxyurea inhibits

A

ribonucleotide reductase

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7
Q

6MP inhibits

A

Glutamine PRPP amidotransferase

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8
Q

mycophenolate inhibits

A

IMP dehydrogenase (IMP to GMP)

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9
Q

Bloom syndrome

A

helicase issue

leukemia, infertility

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10
Q

Ataxia telangectasia

A

dsDNA breaks
low AFP
low IgA
failure in smooth eye pursuit

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11
Q

DNA polymerases

A 
alpha = lAgging
delta = leaDing
beta = BER
gamma = Mitochondria "grandma"
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12
Q

TATA box

A

-25 = Hogness

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13
Q

lac operon

A

glucose removes CAP from CAP site = bad

lactose removes repressor-operator = good

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14
Q

Prader Willi

A

can have OCD, hypogonadism, almond shaped eyes and downturn mouth

diagnose with FISH
treat with GH in short, and restrict food

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15
Q

most common lysosomal storage disease

A

Gaucher

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16
Q

Anode

A

Far end, + charge

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17
Q

Tetrahydrobiopterin needed

thiamine needed

Pyridoxine needed

biotin needed

A

Phenylalanine hydroxylase, tyrosine hydroylase, tryptophan hydroxylase

transketolase, a-ketogluterate DH, pyruvate DH

transaminations, OAA to aspartate

carboxylation

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18
Q

Dopamine hydroxylase deficiency

A

no NE

ptosis, orthostatic HT, hypoglycemia, hypothermia

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19
Q

niacin from

A

tryptophan

so Hartnup disease problem

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20
Q

pentose phosphate pathway

A

NADPH for cholesterol/fatty acid synthesis, glutathione action

ribose 5 phosphate for nucleotides
fructose 6 phosphate for glycolysis

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21
Q

trypsin

A

in SI after duodenal enteropeptidase

activates carboxypeptidase, elastase, chymotrypsin

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22
Q

NAD needed

A

Pyruvate DH
IC dehydrogenase
a-KG DH (thiamine too)
malate DH

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23
Q

Hemoglobin p50

A

26 mm Hg

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24
Q

Carnitine

A

acyl-COA into mitochondria

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25
Q

In mitochondria

In cytosol

A

B-oxidation, ketogenesis, CAC, CPS1, OTC, pyruvate carboxylation

pentose pathway, glycolysis

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26
Q

TK activity

A

protein phosphatases

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27
Q

thalassemia

A

intermedia - Kozak sequence of beta-globin, causing microcytic anemia

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28
Q

asparagine

A

from aspartate, using glutamine

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29
Q

essential fructosuria

A

positive copper reduction test in urine

deficient fructokinase

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30
Q

Niemann Pick

Gaucher

Tay Sachs

A

sphingomyelin - foamy histiocytes, blind

glucocerebroside

hexoaminidase A - ganglioside Gm2

31
Q

Hurlers

A

mucopolysaccharidosis

heparan and dermatan sulfate high

alpha-L-iduronidase deficiency

32
Q

Pompe disease

A

glycogen storage disease

lysosomal a-1,4 glucosidase

33
Q

vitamin E overuse

deficiency

A

hemorrhagic stoke higher mortality
infant necrotizing enterocolitis

hemolysis, ataxia, neuropathy

34
Q

PFK1 regulation

A

F6P to F1,6bisP

inhibited by ATP, citrate
stimulated by AMP, F2,6bisP

35
Q

pyruvate kinase regulation

A

PEP to pyruvate

inhibited by alanine, ATP
stimulated by F1,6bisP

36
Q

gluconeogenesis enzymes

A

pyruvate carboxylase with biotin/ATP
-pyruvate to OAA

PEPCK with GTP
-OAA to PEP

F-1,6-bisphosphatase
-makes F6P

G6Phosphatase

37
Q

pyruvate carboxylase regulation

A

acetyl CoA stimulates

38
Q

F-1,6-bisphosphatase regulation

A

AMP, F2,6bisP inhibits

ATP stimulates

39
Q

pyruvate kinase deficiency

A

hemolytic anemia

40
Q

fructose-2,6-bisphosphate

A

made by PFK-2 in fed state (insulin)

degraded to F6P by FBPase-2 in fasting (glucagon phosphorylates via PKA!)

stimulates PFK-1**

41
Q

glycogenolysis regulation

A

glucagon or epinephrine lead to PKA, which activates glycogen phophorylase kinase

activates glycogen phosphorylase**
-inhibited by ATP, G6P, glucose

42
Q

glycogenesis

A

G6P to G1P to UDP-diphosphoglucose via UDP-glucose pyrophosphorylase

glycogen synthase**
branching enzyme

43
Q

phosphoglucomutase

A

G1P to G6P in glycogenolysis

44
Q

Pompe disease

A

infantile is worse, gets HF, cardiomegaly, weakness

45
Q

pyruvate dehydrogenase deficiency

A

XR or B vitamin low or arsenic poisoning

treat with high fat diet, lysine/leucine ketogenic

46
Q

ETC inhibitors

A

1 - rotenone, amytal, MPP

3 - antimycin A
4 - CO, CN, N3-, H2S

ATPase - oligomycin A

IMM permeability - 2,4-dinitrophenol, thermogenin, high aspirin

47
Q

Cori cycle

A

Lactate to liver from muscle
gluconeogenesis (3ATP/glucose)
glucose to muscle

48
Q

ALT

AST

A

alanine/aKG to pyruvate/glu

aspartate/aKG to OAA/glu

49
Q

essential galactosemia

A

Gal-1-P uridyltransferase deficiency

megaly
jaundice
cataracts

50
Q

HMG CoA synthase

A

ketogenesis

process stimulated by low OAA, high acetylCoA

51
Q

glycogen stores depleted

A

10-18 hrs

52
Q

ketone bodies appear

A

48 hrs

53
Q

brain uses ketone bodies predominately by day

A

day 5

54
Q

percent ketone body use by day 3

A

60%

55
Q

primary energy source day 2

A

fatty acids

56
Q

fatty acid synthesis RLS

A

acetyl CoA carboxylase

57
Q

fatty acid degradation RLS/deficiency

A

carnitine (palmitoyl) AT1 in mitochondria

deficiency causes cytoplasm accumulation
weakness, hypotonia, hypoketotic hypoglycemia

58
Q

apolipoproteins

A 
chylomicron B48
lipoprotein lipase C2
remnant E
VLDL B100
HDL A1
59
Q

LCAT - lecithin chol AT

CETB - CE transfer protein

SRB1 - scavenger R B1

A

nascent HDL to HDL-2/3 (less protein, more CE)

gives CE from HDL-2/3 to VLDL/IDL/LDL
gives TAG in exchange

liver scavenger R for HDL-2/3

60
Q

hepatic lipase

A

IDL to LDL

61
Q

dopamine products

A

HVA via COMT/MAO

NE via vit C

62
Q

Arginine products

A

creatine
urea
NO

63
Q

melatonin from

A

serotonin from tryptophan

64
Q

PKU etiologies

A

BH4 deficiency

phenylalanine hydroxylase deficiency

65
Q

zinc deficiency

A 
wounds
acrodermatitis enteropathica
hypogonadism
infertility
anorexia, diarrhea
hair loss
night vision loss
depression
66
Q

lead poisoning signs

A 
Burton's lines gingiva
lines bones, teeth
hearing
anemia
decrease ALA DH
basophilic stippling
(iron) ringed sideroblasts
67
Q

mercury poisoning signs

A

peeling fingertips

kidney, brain

68
Q

low vit A sign

A

Bitot spots on bulbar conjunctiva

69
Q

high vit A signs

A 
dry skin, N/V
pseudotumor cerebri
liver megaly
vision
hyperlipidemia
alopecia
70
Q

deficient if breastfeed

A

D and K

71
Q

B9

A

folate

deficiency causes depression, diarrhea, megaloblastic, glossitis

72
Q

B5

A

deficiency has adrenal insufficiency

73
Q

phenytoin inhibits

A

K and B9

74
Q

INH inhibits

A

B3 and B6

Biochemistry (3 decks)

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