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Step 1 Review > Metabolism High Yield > Flashcards

Metabolism High Yield Flashcards

1
Q

Where does beta oxidation occur?

A

mitochondira

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2
Q

Where does glycolysis occur?

A

cytoplasm

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3
Q

Where does HMP shunt occur?

A

cytoplasm

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4
Q

Where does acetyl-CoA production occur?

A

Mitochondria

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5
Q

Where does Heme synthesis occur?

A

Both mitochondria and cytoplasm

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6
Q

Where does urea cycle occur?

A

mitochondria and cytoplasm

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7
Q

Where does TCA cycle occur?

A

Mitcochondria

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8
Q

What type of enzyme adds inorganic phosphate onto substrate with NO use of ATP?

A

Phosphorylase

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9
Q

What type of enzyme calalyzes oxidation reduction reactions?

A

dehydrogenase

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10
Q

What type of enzyme transfers CO2 groups with the help of biotin?

A

Carboxylase

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11
Q

What enzyme relocates a functional group within a molecule?

A

Mutase

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12
Q

What is the rate determining enzyme of glycolysis?

A

PFK1

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13
Q

How does F26BP alter activity of glycolysis?

A

increased activity of PFK1

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14
Q

How does citrate alter the activity of glycolysis?

A

increased activity of citrate

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15
Q

What is the rate determining enzyme of gluconeogenesis?

A

F16BPase

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16
Q

What upregulates F16BPase? What downregulates it?/

A

Upregulate? ATP and acetyl coA

Downregulate- AMP, F26BP

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17
Q

What is the rate determining enzyme of TCA cycle?

A

Isocitrate dehydrogenase

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18
Q

What inhibits isocitrate dehydrogenase?

A

NADH and ATP

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19
Q

What is the rate determining enzyme of glycogenesis?

A

glycogen synthase

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20
Q

What is the rate determining enzyme of glycogenolysis?

A

glycogen phosphorylase

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21
Q

What is the rate determining enzyme of HMP shunt?

A

G6PD

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22
Q

What enhances activity of G6PD?

A

NADP+

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23
Q

What decreases activity of G6PD?

A

NADPH

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24
Q

What is the rate determining enzyme of de novo pyrimidine synthesis?

A

Carbamoyl phosphate synthetase II

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25
Q

What is the rate determining enzyme of de novo purine synthesis?

A

PRPP aminotransferase

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26
Q

What downregulates PRPP aminotransferase activity?

A

AMP IMP, GMP

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27
Q

What is the rate determining enzyme of urea cycle?

A

carbamoyl phosphate synthetase I

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28
Q

What is the rate determining enzyme of fatty acid synthesis?

A

acetyl coA carboxylase

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29
Q

What is the rate determining enzyme of fatty acid oxidation?

A

Carnitine acyltransferase I

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30
Q

What downregulates the activity of carnitine aceyltransferase I?

A

malonyl- CoA

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31
Q

What is the rate determining enzyme of ketogenesis?

A

HMG- CoA synthase

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32
Q

What is the rate determining enzyme of cholesterol synthesis?

A

HMG- CoA reductase

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33
Q

What type of processes is NAD+ used in?

A

CATABOLIC

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34
Q

What type of processes is NADPH used in?

A

Anabolic processes

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35
Q

What pathway produces NADPH?

A

HMP shunt

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36
Q

Where is glukokinase found?

A

LIVER

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37
Q

Where is hexokinase found?

A

most tissues except the liver and beta pancreatic cells

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38
Q

Which has a lower Km… hexokinase or glucokinase?

A

Hexokinase

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39
Q

Which has a higher Vmax…hexokinase or glucokinase?

A

Glucokinase

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40
Q

Which is inducable via insulin…hexokinase or glucokinase?

A

glucokinase

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41
Q

Which is inhibited by G6P…hexokinase or glucokinase?

A

Hexokinase

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42
Q

Which steps of glycolysis produces ATP?

A

Phosphogylcerte kinase

Pyruvate kinase

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43
Q

What inhibits pyruvate kinase?

A

Alanine and ATP

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44
Q

What enzyme is active in the fasting state…FBPase2 or PFK-2?

A

FBPase2–> converts F26BP back to F6P

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45
Q

What enzyme is active in the fed state… FBPase2 or PFK-2?

A

PFK-2–> converts F6P to F26BP

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46
Q

What are the 5 cofactors needed for pyruvate dehydrogenase complex?

A

B1, B2 B3, B5, Lipoic acid

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47
Q

What inhibits lipoic acid?

A

Arsenic

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48
Q

What is the treatment for pyruvate dehydrogenase complex deficiency?

A

increase intake of ketogenic nutrients (high fat or increased lysine and leucine)

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49
Q

What are the symptoms of pyruvate dehydrogenase complex deficiency?

A

Lactic acidosis, neuro defects and increased serum alanine

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50
Q

What 4 molecules does pyruvate lead to?

A

1- Alanine
2- Oxaloacetate
3- Acyl- CoA
4- Lactate

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51
Q

How many molecules of NADH, FADH2, CO2 and GTP produces per acetyl CoA?

A

NADH- 3
FADH2- 1
CO2- 2
GTP- 1

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52
Q

Order of molecules in the TCA cycle?

A 
Citrate
Isocitrate
alpha- Ketogluteate
Succinyl- CoA
Succinate
Fumarate
Malate
Oxaloacetate
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53
Q

What complex in the ETC does FAD2 transfter electrons to?

A

Complex II (lower energy than NADH transfer (complex I)

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54
Q

What does rotenone block?

A

complex I

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55
Q

What does cyanide block?

A

Complex IV

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56
Q

What does antimycin A block?

A

Complex III

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57
Q

What does CO block?

A

complex IV

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58
Q

What does oligomycin blocl?

A

complex V

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59
Q

Name 2 uncoupling agents?

A

2,4- dinitrophenol

Aspirin

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60
Q

Name the enzyme:

Pyruvate–> OAA

A

Pyruvate carboxylase

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61
Q

Name the enzyme:

OAA–> PEP

A

Phosphoenolpyruvvate carboxykinase

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62
Q

Name the enzyme:

F16BP–> F6P

A

F16BPase

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63
Q

What gluconeogenesis enzyme do muscles lack?

A

G6Pase

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64
Q

Deficiency in enzymes of gluconeogenesis results in…

A

hypoglycemia

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65
Q

Does HMP shunt produce ATP?

A

NO!!!

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66
Q

Which phase of the HMP shunt produces NADPH?

A

Oxidtive/ irreversible

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67
Q

Aside from producing NADPH, what are the other benefit if the HMP shunt?

A

Produces glycolytic intermediates and ribose for nucleotide synthesis

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68
Q

What enzyme is defieicny in essential fructosuria?

A

Fructokinase

AR

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69
Q

What enzyme is deficienct in fructose intolerance?

A

Aldolase B

AR

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70
Q

What accumulates in fructose intolerance?

A

F1P

this leads to decrease in phosphate (inhibition of glycogenolysis and gluconeogenesis)

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71
Q

What is the treatment for fructose intolerance?

A

Decrease intake fructose and sucrose

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72
Q

What enzyme is deficient in galactose deficiency?

A

Galactokinase (cannot convert galactose to Galactose 1 Phosphate)

73
Q

What molecule increases in galactokinase deficiency?

A

Galactitol–> leads to infantile cataracts

74
Q

What enzyme is deficient in classic galactosemia?

A

Galactose 1 phosphate uridyltransferase

75
Q

What is the treatment for classic galactosemia?

A

exclude galactose and lactose from diet

76
Q

What is sorbitol metabolized to?

A

Fructose

77
Q

What enzyme produces sorbitol from glucose?

A

Aldolase reductase

78
Q

What results from increased sorbitol wthin cells?

A

Osmotic damage (cataracts, retinopathy, peripheral neuropathy)

79
Q

What is lactose metabolized into?

A

Glucose + galactose

80
Q

Name the essential gluconeogenic amino acids?

A

Methionine
Valine
Histidine

81
Q

What are the ketogenic essential amino acids?

A

Leucine

Lysine

82
Q

What are the essential amino acids that are both ketogenic and glucogenic?

A

Isoleucine
Phenylalanine
Threonine
Tryptophan

83
Q

Which amino acids are negatively charged at body pH?

A

Aspartic acid

Glutamic acid

84
Q

Which amino acids are positively charged at the body pH?

A

Arginine
Lysine
Histidine

85
Q

In the transport of ammonia…amino acids transfer their NH3 to what compound?

A

Glutamate which then transfers it to alanine

86
Q

What is the cahill cycle?

A

Alaine (muscle)–> alanine (liver)–> pyruvate–> glucose (liver)–> glucose (muscle)–> pyruvate–> alanine (muscle)

87
Q

What is the cori cycle?

A

Lactate (muscle–> lactate (liver)–> pyruvate–> glucose (liver)–> glucose (muscle)–> pyruvate –> lactate (muscle)

88
Q

What is the most common deficiency of the urea cycle?

A

Ornithine transcarbamylase

89
Q

NAG synthase deficiency affects which enzyme in the urea cycle?

A

Carbamoyl phosphate synthas I

90
Q

Where are the enzymes carbamoyl phosphate synthase and ornithine transcarbamylase located?

A

Mitochondria

91
Q

What amino acid is the backbone of urea?

A

Aspartate

92
Q

What is the treatment for hyperammonemia?

A

Lactulose (ascidify the GI and trap NH4 for excretion

93
Q

Tyrosine is a derivative of which amino acid?

A

phenylalanine

94
Q

Niacin is a derivative of which amino acid?

A

Tryptophan

95
Q

Serotonin is a derivative in which amino acid?

A

Tryptophan

96
Q

Prophyrin and heme is a derivative of which amino acid?

A

Glycine

97
Q

Glutathione is a derivative of which amino acids?

A

glutamate

98
Q

Urea is a derivative of which amino acid?

A

arginine

99
Q

NO is a derivative of which aminoa cid?

A

argining

100
Q

NE/ DA/ Epi are derivatives of which amino acids?

A

Phenylalanine

101
Q

Cofactor for dopamine–> NE?

A

vit C

102
Q

Cofactor for NE–> Epi?

A

SAM

103
Q

What phenylalanine derivative is melatin made from? What enzyme is responsible?

A

Made from DOPA

Tryosinase!

104
Q

What is deficient in malignant PKU?

A

tetrahydrobiopterin cofactor

105
Q

“musty body odor”

A

PKU

106
Q

What enzyme is deficient in maple syrup urine disease?

A

alpha ketoacid dehydrogenase

107
Q

“burnt sugar smelling urine”

A

Maple syrup urine disease

108
Q

What amino acids are to be avoided in maple syrup urine disease?

A

isoleucine
Leucine
Valine
(branched amino acids)

109
Q

“black urine when exposed to air”

A

Alkaptonuria

110
Q

What is deficient in alkaptonuria?

A

Homogentisate oxidase

111
Q

What pathway is defective in alkaptonura?

A

Tyrosine–> fumarate

112
Q

What are the symptoms of homocystinuria?

A

1- intellectual disability
2- marfanoid habitus
3- lens sublixation
4- atherosclerosis

113
Q

What amino acids cannot be reabsorbed in the PCT in cystinuria?

A

COLA

Cystine
Onithine
Lysine
Arginine

114
Q

What type of receptor is the insulin receptor?

A

tyrosine kinase

115
Q

What type of receptor is glucagon receptor?

A

increased cAMP–> increased PKA–> increased glycogen phosphoryalase kinase

116
Q

What organ maintains blood sugar at appropriate levels?

A

Hepatocytes

117
Q

What enzyme breaks alpha 1,4 linkages?

A

glycogen phosphorylase

118
Q

What are the two debranching enzyme?

A

4alpha D glucanotransferase

alpha-1,6- glucosidase

119
Q

Is glycogen phosphorylase active when it is dephosphorylated or phosphorylated?

A

Phosphorylated

120
Q

What enzyme deficiency leads to severe fasting hypoglycemia, increased glycogen in liver, increased blood lactate, increased triglycerides, increased uric acid and hepatomegaly?

A

Glucose 6 phosphatase

von gierke disease

121
Q

What enzyme deficeicny leads to cardiomegaly, hypertrophic cardiomyopathy, exercise intolerance, systemic findings leading to early death?

A

Lysosomal alpha 1,4 glucosidase (acid maltase)

Pompe disease (type II)

122
Q

What enzyme deficiency leads to is a milder form of type I glycogen storage disease with normal blood lactate levels?

A

Debranching enzyme (alpha 1,6 glucosidase

Cori Disease (type III)

123
Q

What enzyme deficiency leads to increased glycogen storave in muscles but muscles cannot break it down leading to painful cramps, red urine after strenuous exercise?

A

Skeletal muscle glycogen phosphorylase (myophosphorylase)

McArdle disease (Type V)

124
Q

How is McArdle disease treated?

A

B6

125
Q

Deficient enzyme in fabry disease?

A

alpha- galactosidase A

126
Q

Deficient enzyme in gaucher disease?

A

Glucocerebrosidase

127
Q

Deficient enzyme in Niemann pick disease?

A

Shingomyelinase

128
Q

Deficient enzyme in Tay sachs disease?

A

Heosaminidase A

129
Q

Deficient enzyme in Krabbe disease?

A

Galactoerebrosidase

130
Q

What enzyme is deficiient in Metachromatic leukodystrophy?

A

Arylsulfatase A

131
Q

Deficient enzyme in Hurler syndroe?

A

alpha- L idurinidase

132
Q

What is the deficient enzyme in Hunter syndrome?

A

Iduronate sulfatase

133
Q

Name the lysosomal storage disease:

progressive neurodegeneration, foam cells, “cherry red” macula, hepatosplenomegaly

A

Neiman Pick

134
Q

What builds up in nieman pick?

A

Sphingomyelin

135
Q

Name the lysosomal storage disease:

central and peripheral demyelination with ataxia and dementia

A

Metachromatic leukodystrophy

136
Q

What builds up in metachromatic leukodystrophy?

A

cerebroside sulfate

137
Q

Name the lysosomal storage disease:

peripheral neuropathy of hands/ feet, angiokeratomas, CV and renal disease

A

Fabry disease

138
Q

What builds up in fabry disease?

A

ceramide trihexoside

139
Q

Name the lysosomal storage disease:

progressive neurodegeneration, delayed development, cherry red macula, lysosomes with onion skin

A

Tay sachs disease

140
Q

What builds up in Tay sachs?

A

GM2 ganglioside

141
Q

Name the lysosomal storage disease:

hepatosplenimegaly, pancytopenia, osteoporiss, necrosis of femus, lipid laden macrophages”

A

gaucher disease

142
Q

What builds up in gaucher disease?

A

glucocerebroside

143
Q

Name the lysosomal storage disease:

“peripheral neuropathy, developmental delay, optic atrophy, globoid cells

A

Krabbe disease

144
Q

What builds up in krabbe disease?

A

Galactocerbroside

145
Q

Name the lysosomal storage disease:

mild developmental delay, mild airway obstruction, mild hepatosplenomegaly, agressive behavior

A

hunter syndrome

146
Q

Name the lysosomal storage disease:

developpmental delay, gargoylism, airway obstruction, corneal clouding, hepatosplenomegaly

A

Hurler syndrome

147
Q

Inherited defect in transport of LCFAs in the mitochondria?

A

Systemic primary carnitine deficiency

148
Q

Fatty acid synthesis requires the transport of _____ from the mitochondria to the cytosol.

A

Citrate

149
Q

“inability to breakdown fatty acids into acetyl coA leading to accummulation of 8- 10 carbon fatty acyl in the blood”

A

Medium chain acyl CoA dehydrogenase deficiency

150
Q

What leads to the production of ketone bodies?

A

Excess acetyl- CoA (glucose and FFA are shunted to ketone formation)

151
Q

What maintains blood glucose levels in fasting states between meals?

A

hepatic glycogenolysis (major)

152
Q

What maintains blood glucose levels during starvation between 1-3 days?

A

Hepatic glycogenolysis, adipose release of FFA, muscle and liver, hepatic gluconeogenesis from peripheral lactate and alanine

153
Q

How long does it take for glycogen to be depleted?

A

1 day

154
Q

What maintains blood glucose levels during starvation after day 3?

A

adipose stores (ketone bodies from acyl coA)

then after adipose stores are depleted protein degredation accelerates

155
Q

What induces the enzyme HMG- CoA reductase?

A

insulin

156
Q

What esterifies plasma cholesterol?

A

LCAT

157
Q

What cells release VLDL?

A

Hepatocytes

158
Q

What converts chylomiccrons to chylomicron remnants?

A

LPL

159
Q

What converts VLDL–> IDL?

A

LPL

160
Q

What converts IDL–> LDL?

A

Hepatic TG lipase

161
Q

What cells release HDL?

A

Liver and intestine

162
Q

What enzyme makes nascent HCL mature?

A

LCAT

163
Q

What is the function of Mature HDL?

A

Transfer of cholesterol esers to VLDL, IDL, and LDL via CETP (cholesterol ester transfer protein)

164
Q

What is the function of ApoE?

A

mediates remnant uptake

165
Q

What molecules have ApoE?

A 
Chylomicron
Chylomicron remnant
VLDL
IDL
HDL
166
Q

What is the function of Apo A-I?

A

Activates LCAT

167
Q

What molecules have Apo A-I?

A

Chylomicron

HCL

168
Q

What is the function of Apo C-II?

A

LPL cofactor

169
Q

What molecules have Apo C-II?

A

Chylomicron
VLDL

HDL

170
Q

What is the function of Apo B-48?

A

Mediates chylomicron secretion

171
Q

What molecules have Apo B-48?

A

Chylomicron

Chylomicron remnant

172
Q

What is the function of Apo B-100?

A

Binds LDL receptor

173
Q

What molecules have Apo B-100?

A

VLDL
IDL
LDL

174
Q

Which molecule acts as a reserviour of Apo E and C-II?

A

HDL

175
Q

What secretes HDL?

A

liver and intestine

176
Q

Which familiar hyperlipidemia has absent LPL or altered Apo C-II?

A

Type I

177
Q

Which familiar hyperlipidemia has absent or defective LDL receptor?

A

Type II

178
Q

Which familial hyperlipidemia has hepatic overproduction of VLDL?

A

Type IV

Step 1 Review (62 decks)

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