NBS2 Exam 1: Biochem "Other" AA metabolism Flashcards

1
Q

What are the three prodcuts of arginine metabolism?

A

ornithine –>Petrescine, NO and Creatinine, glutamic acid, proline

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2
Q

What enzyme converts arginine to petrucine?

A

ornithine decarboxylase converts ornithine to petrucine

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3
Q

what inhibits ornithine decarboxylase

A

eflorcine.

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4
Q

What does petrucine do?

A

It stabalizes DNA

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5
Q

What does efloricine do?

A

it blocks ornithine carboxylase which destroys the tyranosomes of african sleeping sickness. It alo can help with hituisim (growth of hair on a females face)

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6
Q

What does creatinine have to do with arginine?

A

You get creatinine from arginine which you use to measure GFR and kidney f(x)

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7
Q

how do we produce serine?

A

we divert from glycolysis (from G3P) using transaminase

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8
Q

serine can be broken down into what products?

A

glycine and pyruvate

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9
Q

The conversion of serine to glycine involves the use of what intermediate and what enzyme

A

Serine hydroxy methyltransferase which uses Tetrahydrafolate, It gains one carbon in the rxn. This can then be used to make purines or thymdine.

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10
Q

What happens in the breakdown of Glycine

A

It is broken down using glycine cleavage complex, which adds one carbons to THF, thus promoting nucelotide synthesis.

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11
Q

what happens when glycine cleavage complex is broken?

A

Glycine accumulates and is an inhibitory neutotransmitter. Severe mental disability, death frequntly during infancy

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12
Q

trace out serine metabolism

A

serine comes from G3P, it is broken down to glycine by serine hydroxy methyl transferase and creates a N5N10Folate in the process. Glycine can be broken down to oxaloacete (kidney stones) or is combusted to create more N5N10Folate leading to pyrimidine synthesis

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13
Q

histidine metabilism is important why? (tell me about its metabolism)

A

Histidine is broken down into FIGLU. FIGLU can only be broken down further if folate is added. When folate is added, it adds a C in order to produce N5N10THF for 1 carbon metabolism and the synthetis of nucelic acids

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14
Q

if you have increased FIGLU

A

your folate stores may have been depleted

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15
Q

what AA break down and add to the folate pool?

A

Serine, glycine, histidine and tryptophan

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16
Q

What is the folate trap?

A

The folate trap is a result of having the folate intermediates converted to methyl THF. Methyl THF is not in equilibrium with the rest of the methyl products. You need to use homocysteine and B12 to convert Methyl THF to THF. If you dont have B12, your folate stores will get trapped as methyl THF and you will lose your ability to make folate, thus inhibiting the ability to synthesize DNA. This leads to macrocytic anemia

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17
Q

Why is B12 important in relation to folate

A

need B12 to convery Methyl THF to THF, which i used in 1 carbon metabolism to synthesize DNA

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18
Q

B12 deficiency leads to

A

macrocytic anemia due to the deplation of the folate stores. You may also get homocysteneuria since homocysteine cannot be converted back to methionine int he remethylation pathway

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19
Q

first step in the break down of methionine?

A

You add 3 ATP to methionine and get s-adenosyl methionine,

20
Q

s-adenosyl methionine is used in

A

Is a carbon donor and is important in the methylation of histones

21
Q

S adenosyl methioine (from methionine) is broken down into

A

s adenosyl homocysteine

22
Q

s adenosyl homocysteine is broken down into

A

homocysteine

23
Q

Homocysteine reacts with what amino acid?

24
Q

when homocysteine reacts with serine, what enzyme do they use and what do they form?

A

cystathionine synthase converts homocysteine and serine into cystathione.

25
What cofactor does cystathioine synthase use?
PLP
26
what TCA cycle intermediate des methionine eventually break down into?
succinyl CoA
27
cystathionine is broken down into what amino acid?`
cysteine
28
deficinecy in cystathionine synthase will cause
homocysteneuria: the accumulation of homocysteine .
29
the system that is mot important to consider in relation to homocysteneuria
the carviovascular system
30
describe the remethylation pathway
homocysteine is converted back to methionine by putting a methyl group on it using B12 which ges its carbon from THF
31
in homocysteneuria will see
increase homocysteneruia and methionine
32
how do you treat homocysteneuria?
supplement with PLP/B6 and B12 and folic acid. Decrease methionine in diet.
33
What is betaine used for?
Betaine boosts the remethylation pathway to make methionine from homocysteine by serving as a methyl group donor.
34
what are the branched chain AA?
Valine, isoleucine and leucine `
35
Explain MSUD
MSUD is the inability to break down branched chian alpha keto acids due to a defect in branched chain alpha amino dehydrogenase
36
How are branch chain alpha keto acids broken down?
by branched chain alpha amino dehydrogenase
37
how do you treat MSUD?
consume low levels of valine, leucine and isoleucine
38
What is the cofactor used with branched chain alpha amino dehydrogenase
thiamine
39
path of branched chain AA
Valine isolecuine and leucine -->brnahces chain AA --> propionyl --> Methylmalonyl coA --> succinyl CoA
40
proprionic acidemia is due to what
the inability to break the propinionyl coA from branched chain AA into methyl malonyl CoA. Problem in Propionyl CoA. Or due to a B12 deficiency causing the enzyme to not work.
41
what funnels into propionyl coA?
VOMIT: valine, odd chain aa, methionine, isoleucine, threonine
42
what cofactors are used for propionyl coA carboxylase?
biotin, HCO3- and ATP
43
multiple carboxylase defiiency
when you have decreased biotin: effect every carboxylase that uses biotin
44
why is glycine increased in proprionic acidosis
metabolic chaos theory. proprionic acid will inhibit the glycine cleavage complex which combusts glycine for its 1-carbon metabolism power
45
why is ammonia increased in proprionic acidemia
Proprionic acid inhibits CPS1 activity
46
how do you treat proprionic acidosis
give carnitine to react with acetly CoA, the complex is excreted . Can also kill bactria in the gut to decrease the amount of proprionic acidin the gut.