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Boards Biochem > Metabolism > Flashcards

Metabolism Flashcards

1
Q

site of FA oxidation (beta oxidation), acetyl-CoA production, TCA cycle, oxidative phosphorylation, ketogenesis

A

mitochondria

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2
Q

site of glycolysis, FA synthesis, HMP shunt, protein synthesis (RER), steroid synthesis (SER), cholest synthesis

A

cytoplasm

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3
Q

site of heme synthesis, urea cycle, gluconeogenesis

A

both mitochondria & cytoplasm

‘HUGs take 2’

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4
Q

phosphofructokinase-1

A

glycolysis

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5
Q

fructose-1,6-bisphosphatase

A

gluconeogenesis

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6
Q

isocitrate dehydrogenase

A

TCA cycle

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7
Q

glycogen synthase

A

glycogenesis

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8
Q

glycogen phosphorylase

A

glycogenolysis

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9
Q

glucose-6-phosphate dehydrogenase (G6PD)

A

HMP shunt

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10
Q

carbamoyl phosphate synthetase II

A

de novo pyrimidine synthesis

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11
Q

glutamine-phosphoribosylpyrophosphate (PRPP) amidotransferase

A

de novo purine synthesis

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12
Q

carbamoyl phosphate synthetase I

A

urea cycle

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13
Q

acetyl-CoA carboxylase

A

fatty acid synthesis

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14
Q

carnitine acyltransferase I

A

fatty acid oxidation

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15
Q

HMG CoA synthase

A

ketogenesis

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16
Q

HMG CoA reductase

A

cholesterol synthesis

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17
Q

what causes glycolysis to produce zero net ATP

A

arsenic

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18
Q

NADH, NADPH, FADH2 carry ____ in activated form

A

electrons

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19
Q

CoA & lipoamide carry ____ in activated form

A

acyl groups

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20
Q

biotin carries ____ in activated form

A

CO2

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21
Q

tetrahydrofolates carry ____ in activated form

A

1-C units

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22
Q

S-adenosylmethionine carries ____ in activated form

A

CH3 groups

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23
Q

TPP carries ____ in activated form

A

aldehydes

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24
Q

universal electron acceptors

A

NAD+ from vitB3

FAD+ from vitB2

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25
Q

NAD+ is used in what type of processes

A

catabolic - carries reducing equivalents away as NADH

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26
Q

NADPH is used in what type of processes

A

anabolic - steroid & fatty acid synthesis as a supply of reducing equivalents
also used in resp burst, cytochrome P450 system, glutatione reductase

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27
Q

locations of hexokinase

A

most tissues except liver and pancreatic beta cells

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28
Q

locations of glucokinase

A

liver & beta cells of pancreas

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29
Q

function of hexokinase

A

sequesters glucose in tissues at low glucose (low insulin)

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30
Q

function of glucokinase

A

stores glucose in liver if high conc of glucose

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31
Q

gene mutation assoc with maturity onset diabetes of young

A

glucokinase

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32
Q

what inhibits lipoic acid causing vomiting, rice water stools, garlic breath?

A

arsenic

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33
Q

what enzyme is responsible for carrying amino groups to the liver from muscle

A

alanine aminotransferase (B6)

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34
Q

what enzyme creates oxaloacetate which replenishes TCA cycle or used in gluconeogenesis

A

pyruvate carboxylase (biotin)

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35
Q

what enzyme transitions from glycolysis to TCA cycle

A

pyruvate dehydrogenasae (B1, B2, B3, B5, lipoic acid)

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36
Q

what enzyme is at end of anaerobic glycolysis (major pathway in RBCs, WBCs, kidney medulla, lens, testes, cornea)

A

lactic dehydrogenase (B3)

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37
Q

glycolysis produces what?

A

glucose to pyruvate

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38
Q

TCA cycle produces what?

A

pyruvate to acetylCoA

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39
Q

cofactors required for alpha ketoglutarate dehydrogenase complex & pyruvate dehydrogenase complex

A

B1, B2, B3, B5, lipoic acid

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40
Q

oxidative phosphorylation poisons- directly inhib e- transport causing decr proton gradient and block ATP synthesis

A

Rotenone, cyanide, antimycin A, CO

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41
Q

oxidative phosphorylation poison- directly inhib mito ATP synthase causing incr proton gradient - no ATP produced

A

oligomycin

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42
Q

oxidative phosphorylation poison- incr perm of membrane causing decr proton gradient and incr O2 consumption - ATP synthesis stops but e- transport continues - produces heat

A

2,4-dinitrophenol (illicit used for wt loss)
ASA (fevers with ASA overdose)
thermogenin in brown fat

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43
Q

enzyme in mito used to convert pyruvate to oxaloacetate

requires biotin & ATP

A

pyruvate carboxylase (gluconeogenesis)

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44
Q

enzyme in cytosol used to convert oxaloacetate to phosphoenolpyruvate
requires GTP

A

phosphoenolpyruvate carboxykinase (gluconeogenesis)

45
Q

enzyme in cytosol used to convert fructose 1,6 bisphosphate to fructose 6 phosphate

A

fructose 1,6 bisphosphatase (gluconeogenesis)

46
Q

enzyme in ER used to convert glucose 6 phosphate to glucose

A

glucose 6 phosphatase (gluconeogenesis)

47
Q

denatured Hb precipitates in RBCs due to oxidative stress

seen in G6PD deficiency

A

heinz bodies

48
Q

result of phagocytic removal of Heinz bodies by splenic macrophages
seen in G6PD deficiency

A

bite cells

49
Q

defect in fructokinase, autosomal recessive, benign, fructose in blood & urine

A

essential fructosuria

50
Q

deficiency of aldolase B, autosomal recessive
fructose-1-phosphate accumulates causing decr in avail phosphate
sympt present with fruit, juice, honey consumption

A

fructose intolerance

51
Q

accumulation of galacitol
autosomal recessive
infantile cataracts, failure to track objects

A

galactokinase deficiency

52
Q

accumulation of galactitol and other toxic substances
autosomal recessive
FTT, jaundice, hepatomegaly, infantile cataracts, intellect disability, E. coli sepsis in neonates

A

classic galactosemia

53
Q

what enzyme converts glucose to sorbitol and galactose to galactitol

A

aldose reductase

54
Q

what tissues have both aldose reductase & sorbitol dehydrogenase

A

liver, ovaries, seminal vesicles

55
Q

which tissues have only aldose reductase

A

schwann cells, retina, kidneys, lens

56
Q

methionine, valine, histidine

A

glucogenic essential a.a.

57
Q

isoleucine, phenylalanine, threonine, tryptophan

A

glucogenic/ketogenic essential a.a.

58
Q

leucine, lysine

A

ketogenic essential a.a.

59
Q

aspartic acid, glutamic acid

A

acidic a.a. (neg charge at body pH)

60
Q

arginine, lysine, histidine

A

basic a.a.

61
Q

required a.a. during periods of growth

A

arginine, histidine

62
Q

a.a. high in histones which bind neg charged DNA

A

arginine, lysine

63
Q

tremor (asterixis), slurring of speech, somnolence, vomiting, cerebral edema, blurry vision

A

ammonia intoxication

64
Q

excess NH4 depletes what enzyme?

A

alpha ketoglutarate - leads to inhib of TCA cycle

65
Q

tx for hyperammonemia

A

lactulose- acidify GIT & trap NH4
Rifaximin- decr colonic ammoniagenic bacteria
benzoate/phenylbutyrate- bind a.a. and excrete

66
Q

absence of N-acetylglutamate leads to…

A

hyperammonemia

it is a cofactor for carbamoyl phosphate synthetase I

67
Q

neonate poor resp & body temp regulation, poor feeding, develop delay, intellect disability

A

N-acetylglutamate deficiency

68
Q

most common urea cycle disorder, x linked recessive, interferes w/ body ability to eliminate ammonia
evident in 1st days of life
excess carbamoyl phosphate is converted to orotic acid

A

ornithine transcarbamylase deficiency

69
Q

intellectual disability, musty body odor, growth retardation, seizures, fair skin, eczema

A

phenylketonuria (deficient phenylalanine hydroxylase)

70
Q

autosomal recessive blocked degradation of branched a.a. (isoleucine, leucine, valine) d/t low alpha ketoacid dehydrogenase

A

maple syrup urine disease

71
Q

congenital deficiency of homogentisate oxidase in degradative pathway of tyrosine to fumarate creating pigment forming homogenistic acid accumulates in tissue
autosomal recessive
brown sclera and CT, black urine in air, arthralgias

A

alkaptonuria (ochronosis)

72
Q

high homocysteine in urine, intellect disability, osteoporosis, marfanoid habitus, kyphosis, lens subluxation, thrombosis, atherosclerosis
autosomal recessive

A

cystathionine synthase deficiency
decreased affinity of cystathionine synthase for pyridoxal phosphate
homocysteine methyltransferase deficiency

73
Q

hereditary defect of renal PCT and intestinal a.a. transporter that prevents reabsorpt of cysteine, ornithine, lysine, arginine
autosomal recessive

A

cystinuria

74
Q

severe fasting hypoglycemia, high glycogen in liver, high lactate in blood, high TG’s, high uric acid, hepatomegaly
autosomal recessive deficient glucose 6 phosphatase

A

Von Gierke disease (type I)

75
Q

cardiomegaly, hypertrophic cardiomyopathy, exercise intolerance, systemic findings lead to early death
autsomal recessive deficient lysosomal alpha 1,4 glucosidase

A

pompe disease (type II)

76
Q

milder form of type I glycogen storage disease with normal blood lactate level
autosomal recessive deficient debranching enzyme (alpha 1,6 glucosidase)

A

Cori disease (type III)

77
Q

high glycogen in muscle but muscle can’t break it down - painful muscle cramps, myoglobinuria (red urine), arrhythmia
autosomal recessive deficient skel muscle glycogen phosphorylase (myophosphorylase)

A

McArdle disease (type V)

78
Q

periph neuropathy, angiokeratomas, CV/renal disease

X linked recessive deficient alpha galactosidase A

A

Fabry disease

79
Q

most common lysosomal storage disease
hepatosplenomegaly, pancytopenia, osteoporosis, aseptic necrosis of femur, bone crises, lipid laden macrophages
autosomal recessive deficient glucocerebrosidase

A

Gaucher disease

80
Q

progressive neurodegen, hepatosplenomegaly, foam cells, cherry red spot on macula
autosomal recessive deficient sphingomyelinase

A

Niemann-Pick disease

81
Q

progressive neurodegen, developemental delay, cherry red spot on macula, lysosomes with onion skin
autosomal recessive deficient hexosaminidase A

A

Tay Sachs

82
Q

periph neuropathy, develop delay, optic atrophy, globoid cells
autosomal recessive deficient galactocerebrosidase

A

Krabbe disease

83
Q

central + periph demyelination, ataxia, dementia

autosomal recessive deficient arylsulfatase A

A

metachromatic leukodystrophy

84
Q

develop delay, gargoylism, airway obstruction, corneal clouding, hepatosplenomegaly
autosomal recessive deficient alpha L iduronidase

A

Hurler syndrome

85
Q

mild Hurler + aggression and no corneal clouding

X linked recessive deficient iduronate sulfatase

A

Hunter syndrome

86
Q

increased incidence of what lysosomal storage diseases in ashkenazi jews?

A

Tay sachs, Niemann pick, Gaucher

87
Q

inherited defect in transport of LCFAs into mitochondria causing toxic accumulation
weakness, hypotonia, hypoketotic hypoglycemia

A

systemic 1’ carnitine deficiency

88
Q

autosomal recessive disorder of FA oxidation
accumulation of 8-10C fatty acyl carnitines in blood
hypoketotic hypoglycemia
vomiting, lethargy, sz, coma, liver dysfunction

A

medium chain acyl-CoA dehydrogenase deficiency

89
Q

degrades dietary TG’s in small intestine

A

pancreatic lipase

90
Q

degrades circulating TG’s in chylomicrons and VLDLs

found on vascular endothelial surface

A

lipoprotein lipase

91
Q

degrades TG’s remaining in IDL

A

hepatic TG lipase

92
Q

degrades TG’s stored in adipocytes

A

hormone sensitive lipase

93
Q

catalyzes esterification of cholesterol

A

LCAT

94
Q

mediates transfer of cholesterol esters to other lipoprotein particles

A

Cholesterol ester transfer protein CETP

95
Q

mediates remnant uptake

A

apoE

96
Q

activates LCAT

A

apoA-I

97
Q

lipoprotein lipase cofactor

A

apoC-II

98
Q

mediates chylomicron secretion

A

apoB-48

99
Q

binds LDL receptor

A

apoB-100

100
Q

transports cholest from liver to tissues

A

LDL

101
Q

transports cholest from periphery to liver

A

HDL

102
Q

delivers dietary TG’s to peripheral tissue, delivers cholest to liver in form of chylomicron remnants (depleted of TGs)
secreted by intestinal epithel cells

A

chylomicrons

103
Q

delivers hepatic TG’s to peripheral tissue

secreted by liver

A

VLDL

104
Q

formed in degradation of VLDL

delivers TGs and cholest to liver

A

IDL

105
Q

delivers hepatic cholest to peripheral tissues
formed by hepatic lipase modification of IDL in peripheral tissue
taken up by target cells via endocytosis

A

LDL

106
Q

mediates reverse cholest transport from periph to liver
repository for apoC & E (needed for chylomicron and VLDL metabolism)
secreted from liver and intestine
alcohol increases its synthesis

A

HDL

107
Q

autosomal recessive lipoprotein lipase deficiency or altered apoC-II
pancreatitis, hepatosplenomegaly, xanthomas, no incr risk for atherosclerosis

A

familial dyslipidemia I- hyperchylomicronemia

108
Q

autosomal dominant absent/defective LDL receptors

accelerated atherosclerosis, xanthomas, corneal arcus

A

familial dyslipidemia II- familial hypercholesterolemia

109
Q

autosomal dominant hepatic overproduction of VLDL

can cause acute pancreatitis

A

familial dyslipidemia III- hypertriglyceridemia

Boards Biochem (6 decks)

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