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Metabolism Flashcards

1
Q

Compartmentalization:

-Mitochondrial matrix

A

Mitochondrial matrix:

Citric acid Cycle

Oxidative phosphorylation

Beta oxidation of fatty acids

Ketone body formation

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2
Q

Cytosol cellular compartmentalization of metabolism

A

Glycolysis
PPP
FA synthesis

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3
Q

both mito an cytosol comparmentalization

A

Gluconeogenesis

Urea Synthesis

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4
Q

Products of the citric acid cycle?

A

Succinate
Oxaloacetate
Citrate
Alpha ketoglutarate

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5
Q

Rate determining step of Glycolysis

A

PFK-1

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6
Q

Rate determining step of Gluconeogenesis

A

Fructose 1,6 BP

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7
Q

Rate determining step of TCA cycle

A

Isocitrate Dehydrogenase

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8
Q

Rate determining step of Glycogen Synthesis

A

Glycogen synthase

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9
Q

Rate determining step of Glycogenolysis

A

Glycogen Phosphorylase

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10
Q

Rate determining step of HMP shunt

A

G6PD

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11
Q

Rate determining step of Urea Cycel

A

Carbamoyl phosphate synthetase II

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12
Q

Rate determining step of Fatty acid synthesis

A

Acetyl-CoA carboxylase

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13
Q

Rate determining step of Fatty Acid Oxidation

A

Carnitine Acyltransferase

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14
Q

Rate determining step of Ketogenesis

A

HMG-CoA synthase

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15
Q

Rate determining step of Cholesterol synthesis

A

HMG-CoA reductase

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16
Q

Where is Hexokinase found and Km/Vmax/G6P inhibition

A

Hexokinase:
Muscle

low km high vmax

inihibited by G6P

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17
Q

Where is Glucokinase found and km/Vmax/G6P

A

Glucokinase found in the liver

high km, high vmax, NOT inhibited by G6P

insulin dependent

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18
Q

Pyruvate dehydrogenase Reaction required cofactors

A

NADH, Thiamin pyrophosphate, lipoic acid, FAD, CoASH

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19
Q

Trypsin degrades proteins into which AAs

A

Arginine and Lysine

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20
Q

Pepsin degrades proteins into which AAs

A

Tyrosine
Phenylalanine
Leucine

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21
Q

Chymotrypsin degrades proteins into which AAs

A 
Tryptophan
Phenylalanine
Tyrosine
Methionine
Leucine
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22
Q

Elastase degrades proteins into which AAs

A

Alanine
Glycine
Serine

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23
Q

How do amino acids get into the GI epithelial cell?

A

Sodium coupled transport

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24
Q

How do amino acids get into the bloodstream from the GI epithelial cell?

A

facilitated diffusion

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25
Q

What within lysosomes act on proteins to degrade proteins all the way to free amino acids and how are the enzymes and proteins tagged for the lysosomes?

A

Lysosomal Proteases

Tagged with Mannose-6-phosphate marker

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26
Q

What disease stems from an inability to tag proteins with Mannose 6 phosphate

A

I cell disease

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27
Q

Ketogenic Amino /acids

A

Leucine, lysine

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28
Q

Glucogenic and Ketogenic AAs

A 
Phenylalanine
Tyrosine
Tryptophan
Isoleucine
Threonine
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29
Q

MEtabolic intermediates derived from the carbons from AAs

A

pyruvate

Acetoacteyl CoA

Acetyl CoA

Alpha ketoglutarate

Succinyl CoA

Fumarate

Oxaloacetate

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30
Q

Source of Alanine

A

pyruvate

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31
Q

Source of Aspartic Acid which then creates Asparagine

A

Oxaloacetate

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32
Q

Source of Glutamic acid which then creates Glutamine and Proline

A

alpha ketoglutarate

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33
Q

Glutamic acid creates which Amino Acids

A

Proline, Glutamine

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34
Q

Serine which then creates Glycine source

A

3-phosphoglyceric acid

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35
Q

Source of Cysteine

A

Serine and Methione

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36
Q

Source of tyrosine

A

Phenylalanine

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37
Q

which essential amino acids are needed during periods of growth (children or after traumatic event)

A

Histidine

Arginine

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38
Q

imporant chemical compound on coenzyme A

A

Sulfhydryl end

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39
Q

what is a carrier for free fatty acids?

A

albumin

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40
Q

Free fatty acid is turned into acyl-Coa by?

A

Acyl-CoA synthetase

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41
Q

beta oxidation enzymes/cofactor and respective reaction

A

Acyl coA dehydrogenase w/ FAD–double bond in LFA

Enoyl CoA hydratase–Addition of water (hydroxy compound)

BEta-hydroxyl Acyl CoA Dehydrogenase w/NAD—Oxidation

Beta-Keto ThiolaseCleavage to LFA-2Carbon and Acetyl CoA

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42
Q

What is left after complete Odd Chain Fatty Acid and how is carbon added to that to form methylmalonyl CoA?

A

Propionyl CoA

+ Biotin, CO2 or HC03

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43
Q

Why does the blood pH reduce in Ketocidosis with diabetics?

A

beta hydroxybutyric acids are moderately strong acids and require buffers, when buffering capabilities are surpassed, pH lowers

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44
Q

What is the principal control of Fatty acid oxidation via Acetyl CoA Carboxylase

A

Acetyl CoA carboxylase (key enzyme) stimulated by insulin

inhibited by Glucagon and high levels of Acyl CoA

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45
Q

What is the principal control of Fatty acid oxidation via Carnitine Palmitoyl-Transferase

A

Palmitoyl-transferase inhibited by increased levels of Malonyl CoA so the transfer of fatty acids into the mitochondrion slows down

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46
Q

how is biotin attached typically

A

lysine residue zzz

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47
Q

What cousin of NAD is required for FA biosynthesis?

A

NADPH from the pentose phosphate pathway

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48
Q

How is pyruvate transported from the mitochondrial matrix to the cytosol so it can be used for fatty acid synthesis?

A

broken down into oxaloacetate (CO2 input) and acetyl coA then forms citrate which enters in the cytsol, acetyle coa given off and oxaloacetate turns into malate, gives off NADPH and CO2 and goes back into Pyruvate

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49
Q

What stimulates acetyl coa carboxylase?

A

insulin (citrate) stimulates protein phosphatase which removes a phosphate from Acetyl Coa Carboxylase thus activating it

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50
Q

what inhibits acetyl coa?

A

Glucagon (and palmitoyl coA) stimulates cAMP thus stimulating cAMP dependent protein kinase which stimulates AMPKK which somehow makes ATP phosphorylate Acetyl CoA carboxylase

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51
Q

what prosthetic group is contained within acyl carrier protein?

A

Pantotheine

52
Q

What do desaturase do?

A

oxidative, add double bonds, require O2 and NADH

53
Q

Essential fatty acis

A

Linoleic
Linolenic
Arachidonic Acid

54
Q

Essential fatty acids are required in the synthesis of?

A

Prostaglandin
Thromboxane
Leukotriene
Lipoxin

Structural lipids of the cell

Structure of the Mito membrane

55
Q

why are essential fatty acids unable to be made endogenously?

A

we can’t add the second or third double bond to oleic acid to make the linolenic and linoleic

56
Q

Phosphatidic acid is phosphorylated glycerol that is dephosphorylated to create?

A

Diacylglycerol

and then Triacylglycerides and Phospholipids

57
Q

unsaturated ether linkage at C1 Phospholipids called?

A

Plasmologens

58
Q

Thinking about MS, white matter contains what phospholipids that are lost in MS resulting in white matter resembling grey matter?

A

Sphingomyelins (also ethanolamine plasmalogen)

59
Q

Where are sphingolipids degraded?

60
Q

What is the basis of sphingolipids?

61
Q

Deficiency of Hexosaminidase A?

62
Q

Cherry red spot on retina, muscle atrophy, paralysis, seizures, Ashkenzi Jews, Cajun populations

63
Q

Beta Glucocerebrosidase deficiency

A

Gaucher’s

64
Q

bone marrow transplant is curative, no brain involvement in type I, hepato/splenomegaly, death by 3mos

A

Gaucher’s, beta glucocerebrosidase deficiency

65
Q

sphingomyelinase defiency

A

Niemann-Pick

66
Q

lack of muscle coordination, learning problems, brain degeneration, feeding and swallowing difficulties, slurred speech, spastic, hepato/splenomegaly, cherry red halo around retina

A

Niemann-Pick

67
Q

NADPH donate

A

electrons in reductive synthetic reactions

68
Q

NAD _____Acceptor

A

electron acceptor in oxidative degradative reactions

69
Q

NADH feeds electrons into the ______ and produce ATP

70
Q

How is fatty acids transported into the mitochondrial matrix for beta oxidation?

71
Q

Where does FA synthesis take place?

A

Cytosol, transported via citrate

72
Q

Glucose to G6P is an example of a _______ cycle

A

Futile cycle

73
Q

net yield of ATP from glycolysis

74
Q

Key intermediates n TCA

A

oxaloacetate, citrate, succinate, alpha ketoglutarate

also precursors of AAs feed into other cycles

75
Q

Glutamate dehydrogenase reaction generates?

A

NH3——> Carbamoyl Phosphate + Ornithine—-> Citrulline

76
Q

The urea cycle creates which intermediate in TCA which produces Asparate that then feeds back into the Urea Cycle at Argininosuccinate

77
Q

Which metabolic pathways is found exclusively in the cytosol?

A

Glycolysis
PPP
FA synthesis

78
Q

Which metab pathway is in both the mito and the cytosol?

A

gluconeogenesis

79
Q

The Pyruvate dehydrogenase reaction converts Pyruvate to _________

A

Acetyl CoA

80
Q

What cofactors are required for the pyruvate dehydrogenase reaction?

A

NADH
Thiamin pyrophosphate lipoic acid
FAD
CoASH

81
Q

In times of fasting, Acetyl CoA is converted into Acetoactyl CoA to produce?

A

betahydroxybutryate (ketone bodies)

82
Q

the net flow of carbons from Acetyl CoA?

83
Q

What is the beignning of the PPP?

A

G6P—–>6phosphgluconate

84
Q

Glucose produces Glucose 6 phosphate which can be convereted into?

85
Q

Pyruvate in anaerobic conditions produces _______

A

Lactate which is converted back to pyruvate

86
Q

Pyruvate also transaminated to create

87
Q

irreversible reaction of Pyruvate

A

Acetyl CoA

88
Q

If Pyruvate + Biotin

A

Oxaloacetate

89
Q

Importan branchpoints

A

G6P

Pyruvate

90
Q

NADP+ increase and which pathway is stimulated?

A

Pentose Phosphate Pathway

91
Q

How is Phosphofructokinase regulated

A

Activated by F26BP

Inhibited by ATP and citrate

92
Q

Principal control step in Gluconeogenesis?

A

Fructose 1,6 Bisphosphate to

Fructose 6 Phosphate

93
Q

Glucose 6 phosphotase can lead to inability to convert F6P to G6P and so a failure o the last step in gluconeogenesis leading to

A

poorly controlled blood sugar levels (hypoglycemia)

94
Q

ATP allosterically inhibits

A

glycolysis

95
Q

ATP activates

A

gluconeogenesis

96
Q

the principal controlled step in glycolysis occurs at

A

Phosphofructokinase 1

97
Q

Gluconeogenisis controlled step enzyme?

A

Fructose 1,6, Bisphosphatase

98
Q

Liver glycogen levels are lowest at?

99
Q

Glucoses linked 1,x

A

linked 1,4 in glycogens

100
Q

The ends of glycogen branches can be reduced at the non-reducing ends by_____ plus ______and this creates ______

A

Glycogen Phosphorylase plus debranching enzyme (alpha 1,6 glucosidase)

creating Glucose 1 phosphate and some free glucose

101
Q

where is most of our glycogen stored?

102
Q

Glycogen synthesis carried our by ______ and ______

A

Glycogen synthase and branching enzyme

103
Q

How does the liver differ in terms of the mechanism by which it releases a form of glucose from glycogen?

A

glucose can be released into the blood

104
Q

how does insulin affect cAMP levels in cells?

A

decreases cAMP levels, this affects the kinase cascade resulting in dephosphorylation of kinase which keeps the glycogen synthase dephosphorylated and thus activated

105
Q

is glycogen synthase active or inactive when phosphorylated?

A

it is more active when dephophorylated (so decreased levels of cAMP = more active= more glycogen synthesis)

106
Q

Is Glycogen Phosphorylase active or inactive when phosphorylated?

A

active when phosphorylated (so increased levels of cAMP means more active glycogen phosphorylase= more glycogen breakdown and more glucose available

107
Q

Glucose and alanine cycle differ in that

A

Alanine carries a nitrogen group that can be excreted

108
Q

Principal controlled step in fatty acid synthesis occurs at

A

Acetyl CoA to Malonyl CoA via

Acetyl CoA Carboxylase + Biotin

109
Q

Glutamate + Glutamate Decarboxylase =

110
Q

GABA degraded to create?

111
Q

Tyrosine can be created into what neurotransmitter precursor?

112
Q

DOPA can be fully metabolized to

A

Epi and Norepi

113
Q

Tyrosine also precursor

A

Thyroid Hormone

114
Q

Tryptophan precursor for what NT?

A

Serotonin which can turn into melatonin

115
Q

Arginine turns into ornithine then _____ and is also a precursor for

A

Glutamate

Putrescine, spermidine, spermine.

116
Q

which enzyme that’s part of the debranching enzyme generates free glucose during the enzymatic breakdown of glycogen in the skeletal muscles.

A

alpha 1,6-amyloglucosidase

117
Q

disorder characterized by hypoglycemia, hyperlipidemia, lactic acidosis and ketosis, convulsions and glycogen accumulation in liver and kidneys

A

gkycogen storage disease: Von Gierkeon of

118
Q

glucagon increases….

A

glycogenolysis

gluconeogenesis

mobilization of fatty acids

119
Q

insulin dependent Glut transporter

A

Glut-4 in the muscle heart and adipose

120
Q

insulin dependent Glut transporter

A

Glut-4 in the muscle heart and adipose

121
Q

Insulin binds a membrane receptor which stimulates what pathway?

A

Tyrosine kinase pathway

122
Q

Alanine and glutamine released by skeletal muscles in post-absorptive humans. they carry

123
Q

which tissues exclusively depend on glucose?

A

RBCs
Lymphocytes
Renal Medulla
retina

brain (can also utilize ketone bodies)

124
Q

Skeletal muscle and adipose tissue use Glut #

125
Q

Glucose as sole energy source

126
Q

what happens during starvation re:Fatty acids, Gluconeogenesis, Glycogen synthesis, glycogenolysis, and ketone bodies

A

Fatty acids are released from adipose tissue

gluconeogenesis occurs in the liver

glycogen synthesis decreases

glycogenolysis increases in liver and muscle

ketone bodies synthesized by the liver

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