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Learning to Love Biochemistry > Biochem Exam 2 > Flashcards

Biochem Exam 2 Flashcards

1
Q

PDH Deficiency

A

Causes chronic lactic acidosis in kids (fatal)

Neuro probs

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2
Q

Thiamine (B1) Deficiency

A

Alcoholics, poor diet, nausea, constipation, appetite loss

Beri-beri

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3
Q

Advanced deficiency syndromes

A

Alcoholics, Wernicke-Korsakoff syndrome

confusion, ataxia, loss of eye coordination

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4
Q

Beri-beri

A

Dry: neuromuscular, no fluid retention
Wet: peripheral edema, cardiac failure

Thiamine deficiency affects transketolase of PPP
Also affects PDH, alpha-KGDH (alcohol abuse)

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5
Q

CAC enzyme defects

A

lactic acidosis

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6
Q

Pyruvate carboxylase deficiency

A

lactic acidosis

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7
Q

Ketosis/ketoacidosis

A

High ketone bodies, excessive acetyl-CoA
Starvation (low carb/Atkins diets) => ketosis
DM => ketoacidosis

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8
Q

Arsenate

A

looks like phosphate and binds to form 3-phosphoglycerate using phosphoglycerate kinase but no energy output means cells starve

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9
Q

Essential fructosuria

A

no fructokinase, no uptake, benign

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10
Q

Hereditary Fructose Intolerance

A

Deficient in aldolase B so fructose-P accumulates in liver

Phosphate depletes, then can’t break down glycogen so it accumulates

Solution: avoid fructose and sucrose

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11
Q

Galactosemia

A

Gal-P accumulates in liver/CNS/kidney causing liver failure
Galactose-1-phosphate uridyl transferase deficiency s most common/severe
Galactokinase deficiency
UDP-gal-epimerase deficiency

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12
Q

G6PD deficiency

A

Lowered PPP activity -> low NADPH levels -> deprive RBCs of antioxidants
Pt sensitive to H2O2 and Fava beans

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13
Q

Fructose-1,6-Bisphosphatase Deficiency

A

Fasting hypoglycemia and metabolic acidosis
High lactate/pyruvate/ketones
Low glucose

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14
Q

Pompe Disease

A

Glycogen buildup, GSD II

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15
Q

Glycogen phosphorylase deficiency

A

Cannot break down glycogen

Muscle: GSD V = McArdle
Liver: GSD VI = Hers

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16
Q

McArdle

A

Muscle glycogen phosphorylase deficiency; cannot breakdown glycogen
GSD V

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17
Q

Hers

A

Liver glycogen phosphorylase deficiency; cannot breakdown glycogen
GSD VI

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18
Q

GSD III

A

Cori
Problem with debranching enzyme
No phosphorylase

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19
Q

GSD I

A

Von Gierke

G6 phosphatase cannot form glucose from Glu-6P

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20
Q

GSD II

A

Pompe

Alpha-glucosidase cannot make glucose from lysosome/glycogen

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21
Q

Mucopolysaccharidoses

A

problem with degradation of proteoglycans so they accumulate in lysosomes over years

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22
Q

Hunter’s

A

Defective Iduronate synthase causing accumulation of dermatan sulfate and heparin sulfate
Causes skeleton abnormalities and MR

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23
Q

Hurler-Scheie

A

Defective alpha-idonurase
Causing skeletal abn and MR
Treatment is laronidase to replace alpha-Iduronoidase

24
Q

Sanfilippo’s (ABCD)

A

Defective heparin sulfate causing accumulation of heparin sulfate
Mild physical defects, severe MR

25
Q

Crigler-Najjar/Gilbert syndromes

A

Glycoconjugation defect

Buildup of bilirubin due to defect in UGT preenting the necessary conjugation with glucaronic acid for bili excretion

26
Q

NABQI

A

causes liver damage, free radical buildup when UDP-glucaronylation pathway is overwhelmed and acetaminophen is oxidized p450 to NABQI

27
Q

DGAT inhibitor

A

inhibits triacylglycerol synthesis

28
Q

Isocitrate DH

A

regulates CAC
stim. by high ADP
inhibited by high NADH

29
Q

Alpha-KGDH complex

A

regulates CAC

inhibited by high NADH or succ.-CoA

30
Q

PDH kinase

A

High PDH kinase inactivates phospho-PDH

31
Q

PDH phosphatase

A

High pDH phosphatase causes active PDH complex

32
Q

Cyanide

A

Inhibits Complex IV of ETC

Blocks electron flow to oxygen in matrix

33
Q

CO

A

Inhibits Complex IV of ETC

Blocks electron flow to oxygen in matrix

34
Q

2,4 DNP

A

Uncoupler of ETC

Bypass the ATP synthase, providing alternate route for H+ to flow back to matrix

35
Q

UCPs

A

Uncoupling proteins
inhibit ETC
Bypass the ATP synthase, providing alternate route for H+ to flow back to matrix
Pesticides

36
Q

High aspirin

A

Uncoupler of ETC

Bypass the ATP synthase, providing alternate route for H+ to flow back to matrix

37
Q

Atractyloside

A

Inhibits adenine nucleotide translocase which resides in the inner membrane ad exchanges mitochondrial ATP for cystolic ADP

38
Q

Main regulation of Glycolysis

39
Q

PFK1

A

Increased PFK1 regulates glycolysis

F26BP causes acceleration of glycolysis

40
Q

Hexokinase

A

Regulates glycolysis by its product, Glu-6-P, acetyl CoA, and glucagon

Hexokinase stimulates insulin

41
Q

Pyruvate kinase

A

Regulates glycolysis
turns PEP to pyruvate
Stimulates fructose-1,6-BP and insulin
PK is inhibited by ATP

42
Q

cAMP

A

turns on PFKII -> reduces F2,6BP -> reduces glycolysis

43
Q

Pyruvate carboxylase

A

Regulates gluconeogenesis

turns pyruvate to OAA, inhibited by insulin

44
Q

PEP carboxykinase

A

OAA to PEP
Inhibited by insulin and AMP
Stim. by glucagon and cAMP

45
Q

Main regulating step of gluconeogenesis

46
Q

F16BP

A

Regulates gluconeogenesis
F16BP to F6P, is inhibited by F26BP, AMP
Stim. by citrate

47
Q

Glucose-6-phosphatase

A

G6P to glucose
inhibited by insulin
stimulated by glucagon

48
Q

Acetyl CoA

A

High acetyl CoA stim. gluconeogensis and inhibits glycolysis

Low acetyl CoA stimulates glycolysis and inhibits gluconeogensis

Acetyl CoA inhibits PDH
Stimulates P- carboxylase

49
Q

G6PDH

A

Main regulation of PPP
Reduces G6P to phosphoglucolactone
Inhibited by NADPH

50
Q

Main regulation of PPP

51
Q

Glucokinase

A

Regulates glycogen synthesis
Glucose -> G6P
Stimulated by insulin
NOT inhibited by end products

52
Q

Glycogen synthase

A

Adds glucose to glycogen
Inhibited by glucagon, Pi
Stimulated by insulin

53
Q

Glycogen phosphorylase

A

G-1-P units from glycogen
Inhibited by insulin
Stimulated by glucagon, epi

54
Q

Glu-6-phosphatase

A

G6P -> glucose
inhibited by Insulin
Stim. by glucagon, epi

55
Q

Insulin

A

Inhibits glycogen synthase, glucose-6-phosphatase, pyruvate carboxylase, PEP carboxykinase,

Stimulates glycogen phosphorylase, Glu-6-phosphatase, Glucokinase

Stimulated by hexokinase

56
Q

Malonyl CoA

A

inhibits FA synthesis by inhibiting uptake of FA into mitochondria

Learning to Love Biochemistry (1 decks)

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