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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
In mitochondria In cytosol
B-oxidation, ketogenesis, CAC, CPS1, OTC, pyruvate carboxylation pentose pathway, glycolysis
26
TK activity
protein phosphatases
27
thalassemia
intermedia - Kozak sequence of beta-globin, causing microcytic anemia
28
asparagine
from aspartate, using glutamine
29
essential fructosuria
positive copper reduction test in urine deficient fructokinase
30
Niemann Pick Gaucher Tay Sachs
sphingomyelin - foamy histiocytes, blind glucocerebroside hexoaminidase A - ganglioside Gm2
31
Hurlers
mucopolysaccharidosis heparan and dermatan sulfate high alpha-L-iduronidase deficiency
32
Pompe disease
glycogen storage disease lysosomal a-1,4 glucosidase
33
vitamin E overuse deficiency
hemorrhagic stoke higher mortality infant necrotizing enterocolitis hemolysis, ataxia, neuropathy
34
PFK1 regulation
F6P to F1,6bisP inhibited by ATP, citrate stimulated by AMP, F2,6bisP
35
pyruvate kinase regulation
PEP to pyruvate inhibited by alanine, ATP stimulated by F1,6bisP
36
gluconeogenesis enzymes
pyruvate carboxylase with biotin/ATP -pyruvate to OAA PEPCK with GTP -OAA to PEP F-1,6-bisphosphatase -makes F6P G6Phosphatase
37
pyruvate carboxylase regulation
acetyl CoA stimulates
38
F-1,6-bisphosphatase regulation
AMP, F2,6bisP inhibits | ATP stimulates
39
pyruvate kinase deficiency
hemolytic anemia
40
fructose-2,6-bisphosphate
made by PFK-2 in fed state (insulin) degraded to F6P by FBPase-2 in fasting (glucagon phosphorylates via PKA!) stimulates PFK-1**
41
glycogenolysis regulation
glucagon or epinephrine lead to PKA, which activates glycogen phophorylase kinase activates glycogen phosphorylase** -inhibited by ATP, G6P, glucose
42
glycogenesis
G6P to G1P to UDP-diphosphoglucose via UDP-glucose pyrophosphorylase glycogen synthase** branching enzyme
43
phosphoglucomutase
G1P to G6P in glycogenolysis
44
Pompe disease
infantile is worse, gets HF, cardiomegaly, weakness
45
pyruvate dehydrogenase deficiency
XR or B vitamin low or arsenic poisoning treat with high fat diet, lysine/leucine ketogenic
46
ETC inhibitors
1 - rotenone, amytal, MPP 3 - antimycin A 4 - CO, CN, N3-, H2S ATPase - oligomycin A IMM permeability - 2,4-dinitrophenol, thermogenin, high aspirin
47
Cori cycle
Lactate to liver from muscle gluconeogenesis (3ATP/glucose) glucose to muscle
48
ALT AST
alanine/aKG to pyruvate/glu aspartate/aKG to OAA/glu
49
essential galactosemia
Gal-1-P uridyltransferase deficiency megaly jaundice cataracts
50
HMG CoA synthase
ketogenesis process stimulated by low OAA, high acetylCoA
51
glycogen stores depleted
10-18 hrs
52
ketone bodies appear
48 hrs
53
brain uses ketone bodies predominately by day
day 5
54
percent ketone body use by day 3
60%
55
primary energy source day 2
fatty acids
56
fatty acid synthesis RLS
acetyl CoA carboxylase
57
fatty acid degradation RLS/deficiency
carnitine (palmitoyl) AT1 in mitochondria deficiency causes cytoplasm accumulation weakness, hypotonia, hypoketotic hypoglycemia
58
apolipoproteins
``` chylomicron B48 lipoprotein lipase C2 remnant E VLDL B100 HDL A1 ```
59
LCAT - lecithin chol AT CETB - CE transfer protein SRB1 - scavenger R B1
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
hepatic lipase
IDL to LDL
61
dopamine products
HVA via COMT/MAO NE via vit C
62
Arginine products
creatine urea NO
63
melatonin from
serotonin from tryptophan
64
PKU etiologies
BH4 deficiency | phenylalanine hydroxylase deficiency
65
zinc deficiency
``` wounds acrodermatitis enteropathica hypogonadism infertility anorexia, diarrhea hair loss night vision loss depression ```
66
lead poisoning signs
``` Burton's lines gingiva lines bones, teeth hearing anemia decrease ALA DH basophilic stippling (iron) ringed sideroblasts ```
67
mercury poisoning signs
peeling fingertips kidney, brain
68
low vit A sign
Bitot spots on bulbar conjunctiva
69
high vit A signs
``` dry skin, N/V pseudotumor cerebri liver megaly vision hyperlipidemia alopecia ```
70
deficient if breastfeed
D and K
71
B9
folate deficiency causes depression, diarrhea, megaloblastic, glossitis
72
B5
deficiency has adrenal insufficiency
73
phenytoin inhibits
K and B9
74
INH inhibits
B3 and B6

Biochemistry (3 decks)

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