One Carbon Metabolism Flashcards

1
Q

Carbon source for SAM?

A

Methionine

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2
Q

Carbon source for Biotin

A

CO2

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3
Q

How many methylation reactions does FH4 participate in

A

Only one methylation reaction, all others use SAM

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4
Q

Biotin is the cofactor for what 3 major carboxylases

A

Pyruvate carboxylase, Acetyl CoA carboxylase, and Propionyl Carboxylase

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5
Q

What is the first enzyme in fatty acid synthesis

A

Acetyl CoA carboxylase

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6
Q

Pyruvate carboxylase is the first enzyme in what metabolic pathway?

A

Gluconeogenesis

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7
Q

One way to get biotin deficiency…

A

eat 20 raw egg whites in one day

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8
Q

In biotin carboxylase, where is biotin attached

A

to the Epsilon amino group of lysine

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9
Q

Biotinidase is present in cells and serum, and it cleaves biotin from

A

proteins

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10
Q

Holocarboxylase synthetase deficiency can result in

A

encephalopathic crisis (brain damage) and hair loss

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11
Q

SAM is synthesized in a reaction involving both

A

Methionine and ATP

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12
Q

SAM is a major carrier of ________ groups in metabolism

A

methyl

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13
Q

Two possible fates of homocysteine

A

Homocysteine is converted to methionine or used in cysteine synthesis

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14
Q

S-Adenosyl homocysteine is hydrolyzed to

A

adenosine and homocysteine

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15
Q

What is the MAJOR carrier of C1 groups in metabolism?

A

Tetrahydrofolate (FH4)

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16
Q

FH4 is a reduced form of

A

folic acid

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17
Q

What can synthesize folate

A

Not humans. Bacteria and higher plants synthesize folate

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18
Q

Why do pregnant women supplement with folic acid?

A

To prevent neural tube defects

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19
Q

Folate is converted to FH4 by 2 reactions with what one enzyme?

A

dihydrofolate reductase in an NADPH dependent reaction

20
Q

What body organ stores about half of the body’s FH4?

21
Q

What form is folate absorbed in by the small intestines?

A

Monoglutamate

22
Q

What two positions do C1 units bind to on FH4?

A

N5 and/or N10

23
Q

Formyl groups bind _____, methyl groups bind ____, while Methenyl and methylene groups form carbon bridges between

A

N10
N5
N5 and N10

24
Q

Most C1 acceptors are intermediates in

A

purine and pyrimidine synthesis

25
All FH4 derivaties are easily interconverted, reversible reactions, except
N-5-methyl-FH4 (energetically stable)
26
Dietary sources of folic acid
Green vegetables, yeast, liver, some fruits
27
oversized RBC precursors in bone marrow (subtype of macrocytic anemia)
Megaloblasts
28
Analog to folic acid which inhibits dihydrofolate reductase which converts dihydrofolate to tetrahydrofolate
Methotrexate
29
Methotrexate stops cell
division of rapidly dividing tumor cells
30
B12 (cobalamin) contains
cobalt
31
What are the two B12 dependent reactions in humans?
Homocysteine-->Methionine Methylmalonyl CoA--> Succinyl CoA (required for BCAA metabolism)
32
What are the basic major sources of B12?
Meat and dairy are the major sources of B12
33
Proteases release B12 from proteins and then it is bound by
haptocorrins (R-binders)
34
Proteases release B12 from haptocorrins and then it is bound to ...
Intrinsic factor
35
Reduced amounts of Intrinsic factor cause
pernicious anemia (common in elderly)
36
What is the most energetically stable form of carbon FH4?
N5-methyl -FH4 (it cannot be converted to other forms of FH4)
37
What cofactor is required for the only reaction that can remove CH3 from N5methylFH4
Vit B12
38
Lack of B12 causes increase in ___________ and reduces pool of ________
homocysteine FH4
39
folate is "trapped" in N5-methyl form
Methyl Trap Hypothesis
40
What cofactor is required in the BCAA degradation reaction of methylmalonyl CoA to succinyl CoA
Vitamin B12
41
too much homocysteine in the blood
Hyperhomocysteinemia
42
_______ in the liver is an alternate pathway for homocysteine to Methionine
Betaine
43
High levels of homocysteine can result from what 3 possibilities?
1) Folate deficiency 2)B12 deficiency 3) mutation in cystathione beta synthase
44
Mutations in cystathione beta synthase cause____________, which is a rare extreme of hyperhomocysteinemia and its symptoms resemble
homocystinuria.... Marfan
45
Major difference between B12 deficiency and folate deficiency
B12 deficiency has neurological symptoms which are not present in folate deficiency
46
Megaloblasts form in the
bone marrow
47
B12 and folate deficiency can both cause
megaloblastic anemia