AA

Question 1

What do you make a-KG from?`

Answer 1

glutamate + H20 –> glu DH

Question 2

What do you make alanine from?

Answer 2

pyruvate + glutamate

Question 3

What do you make aspartate from?

Answer 3

OAA + glutamate

Question 4

What do you make asparagine from?

Answer 4

aspartate + glutamine

Question 5

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

Answer 5

3 phosphoglyerate; glutamate is involved

Question 6

What do you make glycine from?

Answer 6

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

Question 7

What do you make cysteine from?

Answer 7

methionine

Question 8

What other AA is utilized to make cysteine?

Answer 8

serine

Question 9

What are important intermediates of cysteine?

Answer 9

SAM; homocysteine; cystathione

Question 10

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

Answer 10

cystathionine B-synthase

Question 11

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

Answer 11

cystathionase

Question 12

How do we make tyrosine?

Answer 12

from phenylalanine

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

Question 13

How do we convert DHBtn back to THBTN?

Answer 13

DHBTN reductatase; NADH gets oxidized

Question 14

Succinyl CoA?

Answer 14

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

Question 15

What is familial hyperinsulinemic hypoglycemia type 6?

Answer 15

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

Question 16

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

Answer 16

carboxylase requires biotin

Question 17

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

Answer 17

mutase –> requires B12

Question 18

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

Answer 18

acidemias

Question 19

B12 traveling pathway?

Answer 19

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

Question 20

What results from a defect in cystathionine B-synthase?

Answer 20

homocystinuria

Question 21

What is pernicious anemia?

Answer 21

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

Question 22

What is megoloblastic anemia?

Answer 22

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

Question 23

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

Answer 23

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

Question 24

How do you convert homocysteine to methionine?

Answer 24

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

Question 25

What 2 enzymes require B12?

Answer 25

mutase in production of succinyl coA and methionine synthase; in production of methionine from homocysteine

Question 26

Valine is broken down into what product?

Answer 26

succinyl Coa

Question 27

What enzymes in the muscle and liver break down valine?

Answer 27

BCAT in muscle | BCKDH in liver

Question 28

Isoleucine and leucine can be broken down into what?

Answer 28

1. isoleucine --> acetyl-cOA +propionyl CoA | 2. lecuine_ acetyl CoA + acetoacetate

Question 29

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

Answer 29

acetoacetate and fumurate

Question 30

Tyrosinemia II is due to what?

Answer 30

defect in Tyrosine AT; keratitis, painful skin lesions

Question 31

What is alkaptonuria from?

Answer 31

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

Question 32

What disease results form a defect in homogentisate oxidase?

Answer 32

alkaptonuria

Question 33

What is tyrosinemia I do to?

Answer 33

defect in fumarylacetetone hydrolase; accumulation of succinylacetone

Question 34

Accumulation of succinylacetone is seen in what disease?

Answer 34

tyrosinemia I

Question 35

Children can't synthesize what AA very well?

Answer 35

arginine