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First Aid Behavioral Science > Metabolism > Flashcards

Metabolism Flashcards

1
Q

Metabolism that occurs in Mitochondria

A

beta-oxidation for FA oxidation, acetyl-CoA production, TCA cycle, oxudative phosphorylation

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2
Q

Metabolism in the Cytoplasm

A

Glycolysis, FA synthesis, HMP shunt, protein syn in rER, steroid syn in sER, cholesterol synthesis

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3
Q

Metabolism in both mitochondria and cytoplasm

A

Heme synthesis, Urea cycle, Gluconeogeneis

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4
Q

Kinase

A

Uses ATP to add high-energy phosphate group unto substrate

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5
Q

Phosphorylase

A

Adds inorganic phosphate onto substrate without using ATP

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6
Q

Phosphatase

A

removes phosphate group from substrate

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7
Q

Dehydrogenase

A

catalyzes oxidation-reduction reactions

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8
Q

Hydroxylase

A

adds hydroxyl group onto substrate

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9
Q

Carboxylase

A

Transfers CO2 groups with the help pf biotin

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10
Q

Mutase

A

Relocates a functional group within a molecule

ex is vitB12-dependent methylmalonyl-CoA mutase

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11
Q

Rate-determining enzyme for Glycolysis

A

Phosphofructokinase

inhibited by ATP and citrate

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12
Q

Rate-determining enzyme for Gluconeogenesis

A

Fructose-1,6-Bisphosphatase

inhibited by AMP and F2,6BP

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13
Q

Rate-determining enzyme for TCA cycle

A

isocitrate dehydrogenase

inhibited by ATP and NADH

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14
Q

Rate-determining enzyme for Glycogenesis

A

Glycogen Synthase

inhibited by epinephrine and glucagon

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15
Q

Rate-determining enzyme for Glycogenolysis

A

Glycogen Phosphorylase

inhibited by G6P, insulin and ATP

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16
Q

Rate-determining enzyme for HMP shunt

A

G6PD

inhibited by NADPH

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17
Q

Rate-determining enzyme for de novo pyrimidine synthesis

A

Carbamoyl phosphate synthetase II or CPSII

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18
Q

Rate-determining enzyme for de novo purine synthesis

A

Glutamine-phosphoribosylpyrophosphate (PRPP) amidotransferase
inhibited by AMP, IMP, GMP

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19
Q

Rate-determining enzyme for urea cycle

A

Carbamoyl phosphate synthetase I (CPSI)

Activated by N-acetylglutamate

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20
Q

Rate-determining enzyme for FA synthesis

A

Carnitine acyltransferase I

inhibited by malonyl-CoA

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21
Q

Rate-determining enzyme for Ketogenesis

A

HMG-CoA synthase

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22
Q

Rate-determining enzyme for Cholesterol Synthesis

A

HMG-CoA reductase
inhibited by glucagon and cholesterol
activated by insulin and thyroxine

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23
Q

Galactokinase

A

galactose → galactose-1-phosphate

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24
Q

Galactose-1-phosphate uridyltransferase

A

galactose-1-phosphate → glucose-1-phosphate

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25
Q

Hexokinase/Glucokinase

A

Glucose ↔ Glucose-6-P

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26
Q

G6PD

A

Glucose-6-P→ 6-phosphogluonolactone
HMP shunt
lack of this causes von Gierke’s

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27
Q

Phosphofructokinase

A

rate limiting step in glycolysis
makes fructose-6-P → F1,6P
to reverse this you would need F1,6Bisphosphatase which is rate limiting for gluconeogenesis

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28
Q

Adolase B

A

found in liver

A is in muscle

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29
Q

Pyruvate Kinase

A

PEP to pyruvate

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30
Q

Pyruvate dehydrogenase

A

requires thiamine cofactor TPP

Pyruvate → acetyl-CoA

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31
Q

Pyruvate Carboxylase

A

Requires Biotin

Pyruvate → Oxaloacetate

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32
Q

Isocitrate Dehydrogenase

A

Rate limiting step in TCA,

Isocitrate → alpha-ketoglutarate

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33
Q

Ornithine Transcarbamylase

A

Requires TTP

alpha-ketoglutarate → succinyl-CoA

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34
Q

ATP production in the heart and liver

A

net 32 ATP, malate-aspartate shittle

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35
Q

ATP production in the muscle

A

net 30 ATP, uses glycerol-3-phosphate shuttle

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36
Q

ATP production in Anaerobic Glycolysis

A

net 2 ATP per glucose molecule

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37
Q

Phosphoryl groups

A

carried by ATP

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38
Q

Electrons

A

carried by NADH, NADPH, FADH2

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39
Q

Acyl groups

A

carried by CoA, lipoamide

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40
Q

CO2 carried by

A

Biotin

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41
Q

1 carbon units

A

carried by tetrahydrofolates

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42
Q

CH3 groups

A

carried by SAM

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43
Q

Aldehydes

A

Carried by TPP

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44
Q

Product of HMP Shunt

A

NADPH

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45
Q

Use of NADPH

A

anabolic processes like steroid and FA synthesis
respiratory burst
cytochrome P450 system
glutathione reductase

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46
Q

Low Km

A

High affinity

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47
Q

Low Vmax

A

Low capacity

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48
Q

Glucokinase

A

induced by insulin, high Km, found in liver and beta-cells of pancreas

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49
Q

Mutated in MODY

A

Glucokinase

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50
Q

hexokinase

A

Low Km, Low Vmax, in most tissues except liver and beta cells of pancreas
inhibited by glucose-6-P

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51
Q

Enzymes that require ATP in glycolysis

A

hexokinase/glucokinase

phosphofructokinase-1

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52
Q

Enzymes that produce ATP in glycolysis

A

Phosphoglycerate kinase

Pyruvate Kinase

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53
Q

Purely ketogenic amino acids

A

Lysine and Leucine

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54
Q

Pyruvate dehydroxylase complex deficiency

A

causes a build up or pyruvate that gets shunted to lactate via LDH and alanine via ALT
neuro issues, lactic acidosis, increased serum alanine in infancy

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55
Q

Tx for Pyruvate dehydroxylase complex deficiency

A

increase ketogenic nutrients, high fat content of increase lysine and leucine

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56
Q

Complex I of ETC

A

uses NADH electrons and pumps out hydrogen into intermembrane space of mitochondria
inhibited by Rotenone

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57
Q

Complex II of ETC

A

uses FADH2 electons

succinate dehydroxynase

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58
Q

Complex III of ETC

A

take electrons from CoQ and pumps hydrogen into intermembrane space
inhibited by Antimycin A

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59
Q

Complex IV

A

takes electrons from cytochrome C, makes water
pumps hydrogen into the intermembrane space
inhibited by cyanide and CO

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60
Q

ATP synthase

A

makes ATP and pushes H into mitochondria matrix

inhibited by Oligomycin

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61
Q

Uncoupling Agents

A

2,4-Dinitophenol, aspirin, thermogenin in brown fat
increases permeability of mitochondrial membrane
decreases hydrogen gradient and increases oxygen consumption
products heat

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62
Q

Glucose-6-phosphatase

A

occurs in ER to make glucose

an irreversible enzyme

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63
Q

Odd-chain FA

A

yield 1 propionyl-CoA during metabolism, which can enter the TCA cycle as succinyl CoA
can also undergo gluconeogenesis

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64
Q

Even-chain FA

A

can’t produce glucose, only yield acetyl-CoA

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65
Q

Purpose of HMP Shunt

A

provides a source of NADPH which is required to reduce glutathione inside RBCs to prevent destruction and for FA and cholesterol biosynthesis

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66
Q

Sites of HMP Shunt

A

Lactating mammary glands, adrenal cortex for steroid syn, RBCs

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67
Q

Oxidative HMP shunt Enzyme

A

Glucose-6-dehydrogenase, rate limiting step the make G6P into 2NADPH, CO2, and ribulose-5-P

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68
Q

Nonoxidative HMP shunt enzyme

A

phosphopentose isomerase, transketolase (req B1)

makes ribulose5P into ribose5Pi, G3P and F6P

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69
Q

NADPH oxidase deficiency

A

chronic granulomatous disease

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70
Q

Steps of Oxidative Respiratory Burst

A

NADPH oxidase, Superoxide dismutase, myeloperoxidase, glutathione peroxidase (req selenium), glutathione reductase (req selenium), G6PD

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71
Q

Pyocyanin

A

P. aeruginosa tonxin that generates ROS to kill competing microbes

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72
Q

Lactoferrin

A

protein in secretory fluid and neutrophils that inhibits microbial growth viw iron chelation

73
Q

Heinz Bodies

A

G6PD deficiency, precipitated and oxidized Hb

74
Q

Bite Cells

A

G6PD deficiency,phagocytic removal of Heiz bodies by splenic macrophages

75
Q

Agents that exacerbate G6PD deficiency

A

fava beans, sulfonamides, primaquine, antiTB drugs

they are oxidizing agents that lead to hemolytic anemia

76
Q

G6PD deficiency genetics

A

X-linked recessive, most common human enzyme deficiency

primarily in blacks

77
Q

Defect in fructokinase

A

Essential fructosuria

auto recessive, see fructose in blood and urine otherwise fairly benign

78
Q

Hereditary deficiency in aldolase B

A

Fructose Intolerance - stay away from honey, juice and fruit
Autosomal recessive
Fructose-1-P accumulates
hypoglycemia, jaundice, cirrhosis, vomiting

79
Q

infantile cataracts, failure to track objects, don’t develop a social smile

A

Galactokinase deficiency
galactitol accumulates
auto recessive

80
Q

Classic Galactosemia

A

absence of galactose-1P uridyltransferase
galactitol accumulates in lens of eye
failure to thrive, jaundice, hepatomegaly, infantile cataracts, intellectual disability

81
Q

E. coli sepsis

A

classic galactosemia can lead to neonatal E. coli sepsis

82
Q

Areas with aldose reductase

A

Schwann cells, retina, kidneys

need NADPH to make slucose into sorbitol

83
Q

Osmotic Diarrhea

A

Lactase deficiency

also gets bloating cramosm and flatulancea

84
Q

Glucogenic Amino Acids

A

methionine, Valine, Histidine

85
Q

Acidic Amino Acids

A

aspartic acid, glutamic acid

negatively charged at body’s pH

86
Q

Basic Amino Acids

A

arginine, lysine, histidine

has no charge at body’s pH

87
Q

Amino acids required during periods of growth

A

arginine and histidine

88
Q

Amino Acids increased in histones which bind negatively charged DNA

A

Arginine and lysine

89
Q

Ornithine Transcarbamylase

A

ornithine into citrulline

requires carbamoyl phsophatase

90
Q

Arginase

A

makes arginine and H2O into urea to be excreted by kidneys

91
Q

Amino Acids that transport ammonia

A

Alanine and Glutamate

92
Q

Ammonia intoxication

A

tremor (asterixis), slurred speech, somnolence, vomiting, cerebral edeam and blurred vision

93
Q

Too much ammonia inhibits

94
Q

Traps NH4+ in GI tract for excretion

A

Lactulose, used to tx hyperammonemia

95
Q

Binds to amino acids in diet for excretion

A

Benzoate or Phenylbutyrate (tx hyperammonemia)

96
Q

Required cofactor for carbamoyl phosphate synthetase I

A

N-acetylglutamate

97
Q

N-acetylglutamate Deficiency

A

hyperammonemia (tremor, slurred speech, cerebral edema)

increased ornithine with normal urea cycle enzymes

98
Q

increase orotic acid in blood and urine

A

Ornithine Transcarbamylase Deficiency
X-linked recessive, excess ammonia
tremor, slurred speech, cerebral edema
orotic acid is part of pyrimidine pathway

99
Q

Use of SAM

A

NE → Epinephrine

100
Q

makes melatonin

A

Tryptophan→ serotonin → melatonin

101
Q

Amino acid for porphyrin/heme

102
Q

Amino Acid for GABA and Glutathione

103
Q

Amino Acid for Creatine, Urea, Nitric Oxide

104
Q

Deficient in Phenylalanine hydroxylase

105
Q

Deficient in Homogentisate oxidase

A

Alkaptonuria

degradative pathway of tyrosine to fumarate

106
Q

Deficient in Tyrosinase

107
Q

Inhibited by Carbidopa

A

DOPA decarboxylase to inhibit DA production

108
Q

musty body odor, fair skin, eczema

A

PKU (Auto recessive)

109
Q

Avoid aspartame

A

PKU because it contains phenylalanin

110
Q

Brown pigmented sclera, urine turns black on prolonged exposure to air

A

Alkaptonuria (ochronosis)

111
Q

Cystathionine Synthase deficiency

A

Homocystinuria

112
Q

Tx of Homocystinuria from cystathione synthase deficiency

A

decrease methionine, increase cysteine, increase B12 and folate in diet

113
Q

Decreased affinity of cystathionine synthase for pyridoxal phosphate

A

Homocystinuria, tx with increase Vit B6 and cysteine

114
Q

Homocysteine methyltransferase deficiency

A

Homocystinuria, tx with increase methionine in diet

115
Q

Findings for homocystinuria

A

increase homocysteine in urine, dumb, osteoporosis, tall stature, kyphosis, lens subluxation (down and in), thrombosis, and atherosclerosis

116
Q

Hexagonal Cystine Stones

A

Cystinuria

Tx K-citrate and acetazolamide

117
Q

Urinary cyanide-nitroprusside test

A

Diagnostic for Cystinuria

118
Q

Hereditary defect in Cystinuria

A

Auto recessive

renal PCT and intestinal AA transporter for Cysteine, Ornithine, Lysine and Arginine (COLA)

119
Q

Branched Amino Acids

A

Maple Syrup Urine Disease
I Love Vermont Maple Syrup
Isoleucine, leucine and valine

120
Q

Limit Dextran

A

1-4 residues remaining on a branch after glycogen phosphorylase has already shortened it

121
Q

Von Gierke Disease

A

deficient in glucose-6-phosphatase, aut recessive

severe fasting hypoglycemia, increased blood lactate, increased glycogen in liver

122
Q

Tx of Von Gierke Dx

A

frequent oral glucose/cornstarch

AVOID fructose and galactose

123
Q

Cardiomyopathy early death glycogen storage dx

A

Pompe Disease, deficient in lysosomal alpha1,4-glucosidase

124
Q

Cori Disease

A

Lack debranching enzyme alpha-1,6-glucosidase

fasting hypoglycemia

125
Q

McArdle Disease

A

deficient in myophosphorylase, increase glycogen in muscle
problem with beginning of exercise
red urine with strenuous exerise

126
Q

Muscle Carnitine Deficiency

A

muscle pain later with exercise because fatty acid oxidation is used AFTER glycogen for muscle energy
FA in muscle biopsy

127
Q

deficient in alpha-galactosidase A

A

Fabry Disease X-linked recessive

128
Q

Accum ceramide trihexoside

A

Fabry Disease

129
Q

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

A

Fabry Disease X-linked recessive

130
Q

crumpled tissue paper

A

Gaucher Disease, auto recessive

131
Q

Accum Glucocerebroside

A

Gaucher Disease, auto recessive

132
Q

Deficient in Glucocerebrosidase of beta-glucosidase

A

Gaucher Disease, auto recessive

133
Q

Cherry-red spot on macula, foam cells

A

Niemann-Pick Disease, Auto recessive

134
Q

Cherry-red spot on macula, lysosomes with onion skin

A

Tay-Sachs, Auto recessive

135
Q

Deficient in Sphingomyelinase thus accumulate sphingomyelin

A

Niemann-Pick Disease, Auto recessive

hepatosplenomegaly, progressive neurodegen

136
Q

Deficient in Hexosaminidase A and accumulates GM2 ganglioside

A

Tay-Sachs, Auto recessive

Progressive neurodegeneration

137
Q

Globoid Cells

A

Krabbe Disease, Autosomal Recessive

138
Q

Deficient in Galactocerebrosidase

A

Krabbe Disease, Autosomal Recessive

developmental delay, optic atrophy, periph neuropathy

139
Q

Deficient in Arylsulfatase A

A

Metachromatic Leukodystrophy, autosomal recessive

accum cerebroside sulfate

140
Q

Central and peripheral demyelination with ataxia and dementia

A

Metachromatic Leukodystrophy, autosomal recessive

accum cerebroside sulfate

141
Q

Deficient in alpha-L-iduronidase

A

Autosomal recessive Hurler Disease

142
Q

corneal clouding, gargoylism, airway obstruction, mucopolysaccharidoses

A

Autosomal recessive Hurler Disease

143
Q

Deficiency in Iduronate sulfatase

A

Hunter Syndrome - X-linked recessive

No corneal clouding, aggressive behavior

144
Q

Hypoketotic Hypoglycemia

A

Carnitine deficiency

inability to transfer LCFAs into the mitochondria

145
Q

Ketone Bodies

A

Prolonged Starvation and Diabetic Ketoacidosis

Oxaloacetate is depleted for gluconeogenesis

146
Q

Protein and Carbohydrates

147
Q

Fat

148
Q

Alcohol

149
Q

Fed State (after a meal)

A

glycolysis and aerobic respiration

insulin stim storage of lipids, proteins and glycogen

150
Q

Fasting (between meals)

A

glucagon, adrenaline stimulates use of fuel reserves
hepatic glycogenolysis(major)
FFA release from fat (minor)

151
Q

Starvation (1-3days)

A

Blood glucose maintained by hepatic glycogenolysis, FFA from adipose, muscle and liver shift fuel from glucose to FFA

152
Q

Glycogen Stores Depleted

A

After 1 day of starvation

153
Q

Starvation (>3days)

A

Ketones major source of fuel for brain

154
Q

Lecithin-cholesterol acyltransferase LCAT

A

esterfies 2/3 of palsma cholesterol

155
Q

S/E of statins

A

Rhabdomyolysis and Hepatotoxicity

must check LFTs and Creatine Kinase levels

156
Q

Pancreatic Lipase

A

degradation of dietary TGs in small intestine

157
Q

Lipoprotein Lipase

A

degradation of TG circulating in chlyomicrons and VLDLs

found on vascular endothelial surface

158
Q

Hepatic TG Lipase

A

degrades TG remaining on IDL

159
Q

Hormone sensitive Lipaase

A

degrades TG stored in Adipocytes

160
Q

ApoE

A

mediated remnant uptake

found on chylomicron, its remnant, VLDL, IDL and HDL

161
Q

ApoA-I

A

Activates LCAT

on chylomicron and HDL

162
Q

ApoC-II

A

LPL Cofactor

on chlymicron, VLDL and HDL

163
Q

ApoB-48

A

Mediates Chylomicron Secretion

On chylomicron remnant and on Chylomircon

164
Q

ApoB-100

A

Binds LDL-receptor

on VLDL, IDL, LDL

165
Q

Transports cholesterol from liver to tissues

166
Q

Transports cholesterol from periphery to liver

167
Q

Composition of Lipoproteins

A

cholesterol, TGs, phospholipids

168
Q

Fx of Chylomicron

A

Delivers dietary TG to peripheral tissue.

Delivers cholesterol to liver in form of chylomicron remnants

169
Q

Fx of IDL

A

degraded VLDL, delivers TGs and cholesterol to liver

170
Q

Fx of VLDL

A

delivers hepatic TGs to peripheral tissue, secreted by liver

171
Q

Fx of HDL

A

acts as repository for apoC and ApeE which are needed for chylomicron and VLDL metabolism
alcohol increases synthesis, secreted from both liver and intestine

172
Q

Increased Chyomicrons, TG and cholesterol in blood

A

Hyper-chylomicronemia

Autosomal recessive

173
Q

LPL deficiency fo altered apoC-II, get eruptive/pruritic xanthomas

A

Hyper-chylomicronemia

Autosomal recessive

174
Q

Increased LDL and cholesterol in blood

A

Type IIa, Autosomal Dominant

Familial Hypercholesterolemia

175
Q

Absent fo defective LDL receptors

A

Type IIa, Autosomal Dominant

Familial Hypercholesterolemia

176
Q

Accelerated atherosclerosis, Achilles xanthomas and corneal arcus

A

Type IIa, Autosomal Dominant

Familial Hypercholesterolemia

177
Q

Increase VLDL and TG in blood

A

Type IV, Autosomal Dominant

Hyper-triglyceridemia

178
Q

Overproduction of VLDL

A

Type IV, Autosomal Dominant

Hyper-triglyceridemia

179
Q

Shaggy’s Best Friend

A

Scooby-Doo

First Aid Behavioral Science (4 decks)

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