chpt 10 a biochem Flashcards

1
Q

biotin needed for.. (3 enzyme and pathways)

A

(1) pyruvate carboxylase (gluconeogenesis), (2) acetyl CoA carboxylase (fatty acid synthesis), (3) propionyl coA carboxylase (synthesis of odd-carbon fatty acids, valine, methionine, isoleucine, and threonine – VOMIT)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

carboxylases require (3)

A

ABC enzymes: ATP, biotin, and CO2

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

biotin deficiency

A

(1) hypoglycemia in between meals (fasting) (

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

biotin deficiency caused by? presentation

A

most common cause: excessive raw egg consumption. associated w. hair loss, bowel inflammation, muscle pain

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

thiamine (B1) used by..

A

dehydrogenase (energy production): pyruvate dehydrogenase, alpha-ketoglutarate dehydrogenase (TCA)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

PDH

A

pyruvate –> acetyl CoA

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

dehydrogenases are tightly associated w/ …

A

energy production

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

tissue that utilize glucose as source of energy (glycolysis, PDH, TCA and electron transport chain)

A

thiamine deficiency: CNS (pyruvate dehydrogenase) and cardiac tissue (lots of ATP via TCA,

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

most common cause of thiamine deficiency? other causes?

A

(1) alcoholism (interrupts absorption of thiamine itself) –> wernicke: ataxia, opthalmoplegia and korsakoff: confabulation (2) diet: beri-beri (neuro-like wernicke & korsakoff) – husked (white) rice.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

confabulation

A

inventing something to justify something you believe is true, when you can’t remember well

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

wet beri beri

A

thiamine deficiency: cardiac tissue needs ATP (absence: swell, organomegaly –> dilated cardiomyopathy: systolic failure –> CHF)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

transketolase requires… for…

A

HMP shunt. only requires thiamine.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

(1) PDH, (2) alpha ketoglutarate dehydrogenase, and (3) branched chain ketoacid dehydrogenase requires

A

5 cofactors. thiamine, lipoate, coA, fad, nad

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

test for thiamine deficiency?

A

transketolase deficiency. transketolase only requires thiamine for fxn

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

branched chain ketoacid dehydrogenase

A

metabolism of valine, isoleucine, and leucine –> maple syrup urine disease

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

Niacin B3 needed for

A

NAD(H) and NADP(H) used by dehydrogenases..

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

niacin deficiency seen in..

A

eldery (tea and toast diet) and malnourished

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

niacin deficiency results in..

A

no source of electrons for electron chain transport. 4Ds: diarrhea, dementia, dermatitis, death

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

pellagra can also be related to what deficiency?

A

tryptophan deficiency (in corn), which supplies a portion of niacin requirement (along w/ making 5-HT)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

Hartnup disease

A

genetic disease associated w/ decreased reabsorption of tryptophan by kidney.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

folic acid comes from..

A

leafy vegetables

22
Q

folic acid required for..

A

thymidylate synthase: cannot transfer methyl group to U to make T (no DNA synth). THF needed for enzymes in purine synthesis: cannot make A, G (no DNA or RNA synth)

23
Q

folate deficiency is caused by? results in?

A

alcoholics & pregnancy. stores depleted in 3 months. pregnancy – neural tube defect: spinal bifida, anencephaly.

alcoholics: megaloblastic anemia & homocysteinuria– DVT and atherosclerosis

24
Q

3 major vitamins needed to lower levels of homocysteine

A

folate, B12, B6

25
cyanocobalamin (B12) deficiency caused by...
stored for decades. hard to be deficient w/ diet
26
B12 enzymes (2)
homocysteine methytransferase & methylmalonyl CoA mutase
27
homocysteine methytransferase
untraps inactive folate from storage (N5-methyl TF) , allows it to be active.
28
methylmalonyl CoA mutase
VOMIT pathway (odd chain fatty acid, valine, methionine, threonine). these metabolites accumulate in B12 deficiency
29
methylmalonyl CoA mutase
source of succinyl-CoA (neeedd for TCA, therefore related to ATP) --> explains why B12 has neuro symptoms but folate does not. subacute combined degeneration of spinal cord
30
subacute combined degeneration of spinal cord
B12 deficiency, HIV, and Friedrich's ataxia (dorsal column and lateral column)
31
most common cause of B12 deficiency
pernicious anemia. other: aging, poor nutrition, bacterial overgrowth of terminal ileum, crohn's disease, pancreatic problems (proteases in pancreas needed to separate B12 from R-binders protect from acid), rarely vegans, diphylobothrium latum
32
2 uses of pyridoxine (B6)
used pyridoxal-P (PLP) used for EVER (1) transaminase (aminotransferase): AST (GOT), ALT (GPT). and (2) delta-aminolevulinate synthase
33
job of transaminase
DETOXIFY ammonia (can be used as a strong base, can dissolve lipids of membranes. detoxed by liver. when liver damage, have high transaminase (release from liver & try to ensure that ammonia is not free to enter brain)
34
high transaminase (AST/ALT) activity results in
low B6 levels.
35
2 most common cause of B6 deficiency
1: alcoholic cirrhosis 2: iatrogenic: isoniazid (bc hepatotoxic)
36
vitamin B6 is important for
detox ammonia and synthesis of heme
37
role of B6 deficiency w/ delta-aminolevulinate synthase
heme synthesis decrease, hemoglobin decrease, microcytic anemia, less iron use. iron build-up in precursors --> sidereoblastic anemia. cheilosis/stomatitis (cracking or scaling of the lip borders and corners of the mouth)
38
riboflavin B2. FAD (FADH2)
dehydrogenases (1) succinate dehydrogenase (creb cycle) (2) fatty acyl CoA dehydrogenase (beta-oxidation) (3) glycerol phosphate shuttle / dehydrogenase. bring electrons from cytoplasm into mitochondrial. ENERGY production
39
specific riboflavin deficiency?
unlikely, usu a part of general malnourishment. presents w/ (1) corneal neovascularization, (2) cheilosis / stomatitis (3) MAGENTA-COLOURED tongue -- GLOSSITIS
40
vitamin C deficiency arises from
deficiency in citrus fruits and green vegetables
41
enzymes related to ascorbate (2)
(1) prolyl and lysyl hydroxlases: collagen synthesis (2) dopamine hydroxylase: catecholamine synthesis, absorption of iron in GI tract
42
vitamin C deficiency
scurvy: poor wound healing (granulation tissue = type 3 collagen, easy bruising (perifollicular hemorrhage), bleeding gums, increased bleeding time** bc/ collagen = a, painful glossitis, anemia.
43
prolyl and lysyl hydroxlase VS. lysine hydroxylase
prolyl & lysyl hydroxlase: hydroxylated in collagen, allow to crosslink to eachother to make stable collagen fibrils. occurs in ER. VERSUS: lysl oxidase, cross-links collagen, extracellular, uses Cu2+ as cofactor.
44
vitamin deficiency related increase in bleeding time?
vitamin C deficiency --> defects in collagen (proylyl and lysyl hydroxylase) affects ability of vWF to bind platelets. vitamin K... EDIT
45
vitamin C deficiency on catecholamine synthesis
vitamin C required for dopamine hydroxylase, needed for dopamine --> NE (dopamine hydroxylase). problems w/ CNS and sympathetic system
46
vitamin C on iron absorption
vegetables -- heme of plant as Fe3+, in stomach need H+ to convert Fe3+ --> Fe2+ (absorbed in
47
pantothenic acid B5
deficiency is RARE. cannot live. needed for fatty acid metabolism (either fatty acid coenzyme A whether build synthase or break down w/ beta hydroxylation). PDH and TCA.
48
pantothenic acid needed to make
CoA.
49
vitamin D (cholecalciferol) fxn
fxn: in response to hypocalcemia, helps normalize serum Ca2+ levels & phosphate
50
vitamin A (carotene)
retinoic acid and retinol --> growth regulators, esp in epithelium. retinal --> impt for rod and cone cells for vision
51
vitamin K (from menaquinione, bacteria; phytoquinone, plants)
carboxylation of glutamic acid residues in many Ca2+- binding proteins, importantly coagulation factors II, VII, IX, and X, protein C and S
52
E (alpta-tocopherol)
antioxidant in the lipid phase. protects membrane lipids from peroxidation