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Biochem metabolism Flashcards

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1
Q

What are the regulators of phosphofructokinase 1 (PFK1)? (two +, two -)

A

+ AMP, fructose 2,6 bisphosphate

  • ATP, citrate
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2
Q

What are the regulators of fructose 1,6-bisphosphatase? (two +, two -)

A

+ ATP, acetyl-CoA

  • AMP fructose 2,6 bisphosphatase
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3
Q

What are the regulators of isocitrate dehydrogenase? (one +, 2 -)

A

+ ADP

  • ATP, NADH
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4
Q

What is the enzyme needed to take glucose to G6P? Other way?

A

Hexokinase

Glucose-6-phosphatase

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5
Q

What is the enzyme needed to take G6P to fructose-6-phosphate?

A

GP isomerase

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6
Q

Where is Hexokinase found in highest quantities? Glucokinase?

A

Hexokinase = most tissues

Glucokinase = Liver and Beta cells of pancreas

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7
Q

What is the equation for Km, if K1 = rate from substrates to enzyme/substrate complex, K2 = rate from complex back to substrates, and K3 = rate from complex to products?

A

Km = (K2+K3)/K1

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8
Q

Which is induced by insulin, hexokinase, or glucokinase?

A

Hexokinase

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9
Q

Which is feedback inhibited by glucose, hexokinase or glucokinase?

A

hexokinase

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10
Q

Which has a gene mutation that is associated with maturity onset DM, hexokinase or glucokinase?

A

Glucokinase

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11
Q

What is the MOA or Arsenic?

A

Blocks ATP generation by phosphoglycerate kinase

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12
Q

What are the two downregulators of hexokinase?

A

G6P

Fructose-6-phosphate

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13
Q

What are the only two amino acids that are strictly ketogenic?

A

Lysine and Leucine

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14
Q

What are the findings of pyruvate dehydrogenase deficiency?

A

Neurological defects, lactic acidosis

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15
Q

What is the treatment for pyruvate dehydrogenase deficiency?

A

Increase ketogenic nutrients

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16
Q

What is the cofactor that is needed to keep glutathione reduced? What happens if this enzyme is impaired?

A

NADH

Hemolytic anemia

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17
Q

What is the enzyme that uses NADH to reduce glutathione? Oxidize it?

A

Glutathione reductase

Glutathione peroxidase

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18
Q

What is the cause of essential fructosuria? Symptoms?

A

Fructokinase does not function properly. Benign loss of fructose in urine.

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19
Q

What is the enzymatic cause of fructose intolerance?

A

Deficiency of aldolase B

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20
Q

What is the effect of galactokinase deficiency?

A

Galactouria, infantile cataracts,

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21
Q

What is the cause of classic galactosemia? Symptoms = ?

A

Absence of galactose-1-phosphate uridyltransferase

Symptoms = Infantile cataracts, MR, jaundice

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22
Q

What is the enzyme that converts glucose to sorbitol? Sorbitol to fructose?

A

Aldose reductase

Sorbitol dehydrogenase

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23
Q

Why is it that DM can damage the eye?

A

Eye only has aldose reductase, therefore there is an accumulation of sorbitol

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24
Q

Which type of aminoacids are needed, L or D?

A

L

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25
What are the three amino acids that are only glucogenic?
M V H
26
What are the two acidic amino acids?
Aspartic acid | Glutamic acid
27
What are the three basic amino acids?
R K H
28
What are the substrates of the urea cycle? (hint: Ordinarily, careless crappers are frivolous about urination)?
``` Ornithine Carbamoyl phosphate Citrulline Argininosuccinate Fumarate Arginine Urea ```
29
Why is it that hyperammonemia leads to inhibition of the TCA cycle?
Loss of alpha-keto glutarate
30
What is the treatment for hyperammonemia? What are the two drugs that can be given?
Limit protein intake Benzoate Phenylbutyrate
31
What is the MOA of lactulose?
Acidify's the Gi tract to trap NH4
32
What is the cause of n-acetylglutamate deficiency (what enzyme is affected)?
A required cofactor for carbamoyl phosphate synthetase I
33
What is the universal NH4 carrier?
Glutamate/ alpha ketoglutarate
34
What is ornithine transcarbamylase deficiency? Mode of inheritance? How is it diagnosed?
XLR loss of ornithine transcarbamylase Orotic acid increased in the blood
35
What is the most common urea cycle disorder (which enzyme is deficient)?
ornithine transcarbamylase deficiency
36
What is the rxn that ornithine transcarbamylase catalyses"
Ornithine to citrulline
37
Orotic acid increased in the blood = ?
Ornithine transcarbamylase deficiency
38
What is the vitamin necessary to convert Y to niacin?
B6
39
What is the vitamin necessary to convert histidine to histamine?
B6
40
What is the enzyme necessary to convert E to GABA?
B6
41
What are the two possible causes of phenylketonuria? (enzyme/cofactor)
Deficiency or defect in phenylalanine hydroxylase or THF cofactor
42
What are the symptoms of phenylketonuria?
MR SZs Musty body odor
43
Musty baby = ?
Phenylketonuria
44
What is the treatment for phenylketonuria?
Decrease F, increase Y.
45
What is the cause of alkaptonuria?
Deficiency of homogentisate oxidase (in degradation of Y to fumarate)
46
What are the symptoms of alkaptonuria?
Dark CT, brown pigmented sclera Urine turns black
47
What are the three causes of homocystinuria?
1. Cystathionine beta-synthase deficiency (CBS) 2. Decreased affinity of cystathionine synthase for PPP 3. Homocysteine methyltransferase deficiency
48
What are the symptoms of homocystinuria?
Increased homocysteine in urine | MR
49
Review methionine to Cysteine pathway.
``` M (M adenosyltransferase) SAM (methyltransferases) Homocysteine (CBS) Cystathionine (Cystathionase) Cysteine ```
50
What are the causes of cystinuria?
Defect in AA transporter for COLA (cysteine, Ornithine, Lys, Arg)
51
What is the cause of maple syrup urine disease? What is the enzyme that is involved?
Blocked degradation of branched AAs (I, L, V) Alpha ketoacid dehydrogenase
52
What are the three AAs that are blocked in maple syrup urine disease?
I L V (I love vermont)
53
What are the symptoms of maple syrup urine disease?
Maple syrup urine Ketoacidosis MR
54
What is the treatment for maple syrup urine disease?
Restrict Leucine, Isoleucine, and valine
55
What is the bond between glycogen monomers (glucose)?/ What about the branches of the polymer?
alpha(1,4) | alpha (1,6) for branches
56
What are the two metabolites that come from the degradation of glycogen?
Glucose-1-phosphate | Glucose-6-phosphate
57
What are the two enzymes that are needed for glycogenolysis?
Glycogen phosphorylase | Debranching enzyme
58
What is the receptor for insulin?
Y kinase dimer
59
What is the receptor for glucagon?
G protein with PKA and calmodulin
60
What are the enzymes responsible for the synthesis of glycogen?
Glycogen synthase | Branching enzymes
61
What are the monomers that are added to a growing glycogen chain?
UDP-glucose
62
What are the two intermediates of glucose to glycogen?
G6P | G1P
63
What is type I GSD? What is the enzyme that is deficient?
Von Gierke---G-6-Phosphatase
64
What is type II GSD? What is the enzyme that is deficient? Symptoms?
Pompe's disease Acid maltase Pompe's trashes the pump (heart, liver, muscle failure)
65
What is type III GSD? What is the enzyme that is deficient?
Cori disease | Debranching enzyme
66
What is type V GSD? What is the enzyme that is deficient? Symptoms?
McArdle's disease Muscle glycogen phosphorylase Muscle weakness
67
What is the enzyme that is deficient in Hunter's syndrome? Mode of inheritance?
Iduronate sulfatase--XLR
68
What is the enzyme that is deficient in Hurler's syndrome? Mode of inheritance?
alpha-L-iduronidase (AR)
69
What is the enzyme that is deficient in fabry's disease? Symptoms? What accumulates?
alpha-galactosidase Peripheral neuropathy Ceramide trihexoside
70
What is the enzyme that is deficient in Gaucher's disease? Symptoms? What accumulates?
Glucocerebrosidase Crumpled tissue paper macrophages Glucocerebroside
71
What is the enzyme that is deficient in Niemann-Pick's disease? Symptoms? What accumulates?
Sphingomyelinase Sphingomyelin Progressive neurodegeneration (No Man picks his nose with his sphinger
72
What is the enzyme that is deficient in Tay-Sach's disease? Symptoms? What accumulates?
Hexosaminidase A GM2 ganglioside Progressive neurodegeneration (tay sax lacks hexosaminidase)
73
What is the enzyme that is deficient in Krabbe disease? Symptoms? What accumulates?
galactocerebrosidase Galactocerebroside Peripheral neuropathy, MR
74
What is the enzyme that is deficient in Metachromatic leukodystrophy? Symptoms? What accumulates?
Arylsulfatase A Cerebroside sulfate Central and peripheral demyelination
75
What is carnitine deficiency?
Inability to transport LCFAs into mito, causing weakness, hypotonia
76
What is acyl-coa dehydrogenase deficiency?
Inability to transport carnitine inside the mito, d/t lack of carrier.
77
What is the enzyme that transports FAs into the mitochondria? What is the intermediate produce between the two?
Carnitine palmitoyl transferase I and II Acyl-carnitine
78
What are FAs metabolized to, and is measured in DKA?
beta-hydroxybutyrate
79
What is the rate limiting step of Cholesterol synthesis? (name the enzyme involved)
HMG-CoA reductase
80
What is the MOA of statins?
Inhibit HMG-CoA reductase
81
What is the enzyme that breaks down dietary triglycerides in the small intestines?
Pancreatic lipase
82
What is the enzyme that degrades Tgs circulating in chylomicrons and VLDLs?
Lipoprotein lipase (LPL)
83
What is the function of apoprotein A?
activators of LCAT
84
What is the function of apoprotein B?
Structural
85
What is the function of lipoprotein C?
Modulate the function of LPL
86
What is the function of lipoprotein E?
Message
87
What is the lipase that degrades TGs stored in adipocytes?
Hormone sensitive lipase
88
What is the function of LCAT?
Catalyzes esterification of cholesterol
89
Can the body breakdown cholesterol?
No
90
What is the enzyme that transports cholesterol out of a cell into HDL, in order to form LDL? What is the enzyme that takes the cholesterol, and transfers to HDL particles?
ABCA-1 LCAT
91
What is the function of LDL?
Transports cholesterol from the Liver to body
92
What is the function of HDL?
Transports cholesterol from the body to liver
93
What is the effect of EtOH on HDL synthesis?
Increases
94
What is Type I hypercholesterolemia?
AR lipoprotein lipase deficiency
95
What is type II hypercholesterolemia?
AD absent LDL receptors
96
What is type IV hypercholesterolemia?
AD hepatic overproduction of VLDLs
97
Which type of hypercholesterolemia: increased chylomicrons, TG, and cholesterol
I
98
Which type of hypercholesterolemia: Increased LDLs, cholesterol
II
99
Which type of hypercholesterolemia: increase VLDL, Tgs
IV
100
Which type of hypercholesterolemia: hyperchylomicronemia
I
101
Which type of hypercholesterolemia: familial hypercholesterolemia
IIa
102
Which type of hypercholesterolemia: hypertriglyceridemia
IV

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