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)

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2
Q

carboxylases require (3)

A

ABC enzymes: ATP, biotin, and CO2

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3
Q

biotin deficiency

A

(1) hypoglycemia in between meals (fasting) (

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4
Q

biotin deficiency caused by? presentation

A

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

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5
Q

thiamine (B1) used by..

A

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

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6
Q

PDH

A

pyruvate –> acetyl CoA

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7
Q

dehydrogenases are tightly associated w/ …

A

energy production

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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,

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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.

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10
Q

confabulation

A

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

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11
Q

wet beri beri

A

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

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12
Q

transketolase requires… for…

A

HMP shunt. only requires thiamine.

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13
Q

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

A

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

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14
Q

test for thiamine deficiency?

A

transketolase deficiency. transketolase only requires thiamine for fxn

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15
Q

branched chain ketoacid dehydrogenase

A

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

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16
Q

Niacin B3 needed for

A

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

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17
Q

niacin deficiency seen in..

A

eldery (tea and toast diet) and malnourished

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18
Q

niacin deficiency results in..

A

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

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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)

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20
Q

Hartnup disease

A

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

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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
Q

cyanocobalamin (B12) deficiency caused by…

A

stored for decades. hard to be deficient w/ diet

26
Q

B12 enzymes (2)

A

homocysteine methytransferase & methylmalonyl CoA mutase

27
Q

homocysteine methytransferase

A

untraps inactive folate from storage (N5-methyl TF) , allows it to be active.

28
Q

methylmalonyl CoA mutase

A

VOMIT pathway (odd chain fatty acid, valine, methionine, threonine). these metabolites accumulate in B12 deficiency

29
Q

methylmalonyl CoA mutase

A

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
Q

subacute combined degeneration of spinal cord

A

B12 deficiency, HIV, and Friedrich’s ataxia (dorsal column and lateral column)

31
Q

most common cause of B12 deficiency

A

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
Q

2 uses of pyridoxine (B6)

A

used pyridoxal-P (PLP) used for EVER (1) transaminase (aminotransferase): AST (GOT), ALT (GPT). and (2) delta-aminolevulinate synthase

33
Q

job of transaminase

A

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
Q

high transaminase (AST/ALT) activity results in

A

low B6 levels.

35
Q

2 most common cause of B6 deficiency

A

1: alcoholic cirrhosis 2: iatrogenic: isoniazid (bc hepatotoxic)

36
Q

vitamin B6 is important for

A

detox ammonia and synthesis of heme

37
Q

role of B6 deficiency w/ delta-aminolevulinate synthase

A

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
Q

riboflavin B2. FAD (FADH2)

A

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
Q

specific riboflavin deficiency?

A

unlikely, usu a part of general malnourishment. presents w/ (1) corneal neovascularization, (2) cheilosis / stomatitis (3) MAGENTA-COLOURED tongue – GLOSSITIS

40
Q

vitamin C deficiency arises from

A

deficiency in citrus fruits and green vegetables

41
Q

enzymes related to ascorbate (2)

A

(1) prolyl and lysyl hydroxlases: collagen synthesis (2) dopamine hydroxylase: catecholamine synthesis, absorption of iron in GI tract

42
Q

vitamin C deficiency

A

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
Q

prolyl and lysyl hydroxlase VS. lysine hydroxylase

A

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
Q

vitamin deficiency related increase in bleeding time?

A

vitamin C deficiency –> defects in collagen (proylyl and lysyl hydroxylase) affects ability of vWF to bind platelets. vitamin K… EDIT

45
Q

vitamin C deficiency on catecholamine synthesis

A

vitamin C required for dopamine hydroxylase, needed for dopamine –> NE (dopamine hydroxylase). problems w/ CNS and sympathetic system

46
Q

vitamin C on iron absorption

A

vegetables – heme of plant as Fe3+, in stomach need H+ to convert Fe3+ –> Fe2+ (absorbed in

47
Q

pantothenic acid B5

A

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
Q

pantothenic acid needed to make

A

CoA.

49
Q

vitamin D (cholecalciferol) fxn

A

fxn: in response to hypocalcemia, helps normalize serum Ca2+ levels & phosphate

50
Q

vitamin A (carotene)

A

retinoic acid and retinol –> growth regulators, esp in epithelium. retinal –> impt for rod and cone cells for vision

51
Q

vitamin K (from menaquinione, bacteria; phytoquinone, plants)

A

carboxylation of glutamic acid residues in many Ca2+- binding proteins, importantly coagulation factors II, VII, IX, and X, protein C and S

52
Q

E (alpta-tocopherol)

A

antioxidant in the lipid phase. protects membrane lipids from peroxidation