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Learning to Love Biochemistry > Biochem Exam 2 > Flashcards

Biochem Exam 2 Flashcards

(56 cards)

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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

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A

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

24
Q

Sanfilippo’s (ABCD)

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A

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

25
Crigler-Najjar/Gilbert syndromes
Glycoconjugation defect | Buildup of bilirubin due to defect in UGT preenting the necessary conjugation with glucaronic acid for bili excretion
26
NABQI
causes liver damage, free radical buildup when UDP-glucaronylation pathway is overwhelmed and acetaminophen is oxidized p450 to NABQI
27
DGAT inhibitor
inhibits triacylglycerol synthesis
28
Isocitrate DH
regulates CAC stim. by high ADP inhibited by high NADH
29
Alpha-KGDH complex
regulates CAC | inhibited by high NADH or succ.-CoA
30
PDH kinase
High PDH kinase inactivates phospho-PDH
31
PDH phosphatase
High pDH phosphatase causes active PDH complex
32
Cyanide
Inhibits Complex IV of ETC | Blocks electron flow to oxygen in matrix
33
CO
Inhibits Complex IV of ETC | Blocks electron flow to oxygen in matrix
34
2,4 DNP
Uncoupler of ETC | Bypass the ATP synthase, providing alternate route for H+ to flow back to matrix
35
UCPs
Uncoupling proteins inhibit ETC Bypass the ATP synthase, providing alternate route for H+ to flow back to matrix Pesticides
36
High aspirin
Uncoupler of ETC | Bypass the ATP synthase, providing alternate route for H+ to flow back to matrix
37
Atractyloside
Inhibits adenine nucleotide translocase which resides in the inner membrane ad exchanges mitochondrial ATP for cystolic ADP
38
Main regulation of Glycolysis
PFK1
39
PFK1
Increased PFK1 regulates glycolysis | F26BP causes acceleration of glycolysis
40
Hexokinase
Regulates glycolysis by its product, Glu-6-P, acetyl CoA, and glucagon Hexokinase stimulates insulin
41
Pyruvate kinase
Regulates glycolysis turns PEP to pyruvate Stimulates fructose-1,6-BP and insulin PK is inhibited by ATP
42
cAMP
turns on PFKII -> reduces F2,6BP -> reduces glycolysis
43
Pyruvate carboxylase
Regulates gluconeogenesis | turns pyruvate to OAA, inhibited by insulin
44
PEP carboxykinase
OAA to PEP Inhibited by insulin and AMP Stim. by glucagon and cAMP
45
Main regulating step of gluconeogenesis
F16BP
46
F16BP
Regulates gluconeogenesis F16BP to F6P, is inhibited by F26BP, AMP Stim. by citrate
47
Glucose-6-phosphatase
G6P to glucose inhibited by insulin stimulated by glucagon
48
Acetyl CoA
High acetyl CoA stim. gluconeogensis and inhibits glycolysis Low acetyl CoA stimulates glycolysis and inhibits gluconeogensis Acetyl CoA inhibits PDH Stimulates P- carboxylase
49
G6PDH
Main regulation of PPP Reduces G6P to phosphoglucolactone Inhibited by NADPH
50
Main regulation of PPP
G6PDH
51
Glucokinase
Regulates glycogen synthesis Glucose -> G6P Stimulated by insulin NOT inhibited by end products
52
Glycogen synthase
Adds glucose to glycogen Inhibited by glucagon, Pi Stimulated by insulin
53
Glycogen phosphorylase
G-1-P units from glycogen Inhibited by insulin Stimulated by glucagon, epi
54
Glu-6-phosphatase
G6P -> glucose inhibited by Insulin Stim. by glucagon, epi
55
Insulin
Inhibits glycogen synthase, glucose-6-phosphatase, pyruvate carboxylase, PEP carboxykinase, Stimulates glycogen phosphorylase, Glu-6-phosphatase, Glucokinase Stimulated by hexokinase
56
Malonyl CoA
inhibits FA synthesis by inhibiting uptake of FA into mitochondria

Learning to Love Biochemistry (1 decks)

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