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Section 3 > AA > Flashcards

AA Flashcards

1
Q

What do you make a-KG from?`

A

glutamate + H20 –> glu DH

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2
Q

What do you make alanine from?

A

pyruvate + glutamate

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3
Q

What do you make aspartate from?

A

OAA + glutamate

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4
Q

What do you make asparagine from?

A

aspartate + glutamine

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5
Q

What do you make serine from? What AA is involved?

A

3 phosphoglyerate; glutamate is involved

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6
Q

What do you make glycine from?

A

Serine + THF –> serine hydroxymethyltransferase –> glycine + N5, N10 THF metheylene + H20

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7
Q

What do you make cysteine from?

A

methionine

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8
Q

What other AA is utilized to make cysteine?

A

serine

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9
Q

What are important intermediates of cysteine?

A

SAM; homocysteine; cystathione

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10
Q

What enzyme do we use to convert homocysteine + serine –> cystathionine?

A

cystathionine B-synthase

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11
Q

How do we convert cystathione _ a-ketobutyrate _ NH4 –> cysteine?

A

cystathionase

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12
Q

How do we make tyrosine?

A

from phenylalanine

phenylalanine + O2 + THBtn –> phenylalnine hydroxylase –> tyrosine + DHBTN

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13
Q

How do we convert DHBtn back to THBTN?

A

DHBTN reductatase; NADH gets oxidized

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14
Q

Succinyl CoA?

A
  1. Propionyl-COA –> carboxylase –> D-methylmalonly COA
  2. D –> racemase –> L
  3. L methylmalonyl COA –> B12 mutase –> succinyl coa
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15
Q

What is familial hyperinsulinemic hypoglycemia type 6?

A

mutation in glutamate DH, so GDH is not inhibited and keeps on breaking down AAS

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16
Q

So, to make succinyl Coa you first need to convert propionly-COA –> D-methylmalonyl -COA by what enzyme? What does this enzyme require?

A

carboxylase requires biotin

17
Q

L methylmaylonlyl-COA –> succinyl CoA requires what enzyme and WHAT VITAMIN?!?!?!

A

mutase –> requires B12

18
Q

Defects in carboxylase, racemase, and mutase will lead to what?

A

acidemias

19
Q

B12 traveling pathway?

A
  1. released from foot from chewing and gastic acid
  2. binds to R binding proteins
  3. travels to duodenum where R portins are degraded and binds to intrinsic factor proteins
  4. intrinsic/B12 complex and protesase resitsant and taken up in the ileum by receptor mediated enocytosis
  5. b12 is liberated from intrinic factor and binds to transcobalimin
  6. transcobalinin/b12 realsed into blood
  7. stored in liver
20
Q

What results from a defect in cystathionine B-synthase?

A

homocystinuria

21
Q

What is pernicious anemia?

A

lack of b12 or inability to absorb B12 from lack of intrinic factor for example

22
Q

What is megoloblastic anemia?

A

B12 defiency results in accumulation of N5 methyl THF, and decreases concentrations of more oxidized forms of THF; large cells

23
Q

N5, N10 methlene THF is required for synthesis of what?

A

thymidine and purine rings; so a lack of this will prevent DNA replication

24
Q

How do you convert homocysteine to methionine?

A

homocysteine + N5 methyl THF –> methionine synthase –> methionie + THF

25
Q

What 2 enzymes require B12?

A

mutase in production of succinyl coA

and methionine synthase; in production of methionine from homocysteine

26
Q

Valine is broken down into what product?

A

succinyl Coa

27
Q

What enzymes in the muscle and liver break down valine?

A

BCAT in muscle

BCKDH in liver

28
Q

Isoleucine and leucine can be broken down into what?

A
  1. isoleucine –> acetyl-cOA +propionyl CoA

2. lecuine_ acetyl CoA + acetoacetate

29
Q

Phenylalanine is converted into Tyrosine which is broken down finally into what 2 products?

A

acetoacetate and fumurate

30
Q

Tyrosinemia II is due to what?

A

defect in Tyrosine AT; keratitis, painful skin lesions

31
Q

What is alkaptonuria from?

A

defect in homogentisate oxidase; buildup of homogentisate and urine is black

32
Q

What disease results form a defect in homogentisate oxidase?

A

alkaptonuria

33
Q

What is tyrosinemia I do to?

A

defect in fumarylacetetone hydrolase; accumulation of succinylacetone

34
Q

Accumulation of succinylacetone is seen in what disease?

A

tyrosinemia I

35
Q

Children can’t synthesize what AA very well?

A

arginine

Section 3 (2 decks)

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