Md2 Block4 Biochem L 39 Metabolism Of Amino Acids Carbon Skeleton Part B

Front 1

Amino acids that form pyruvate

1 Alanine

Back 1

Ala loses amino group by transamination  pyruvate

Hint 1

Front 2

Amino acids that form pyruvate

Serine

Back 2

Ser  pyruvate by serine dehydratase

Ser  glycine and N5, N10-methylenetetrahydrofolic
acid by serine hydroxymethyltransferase

Hint 2

Front 3

Amino acids that form pyruvate

3 Glycine

Back 3

Glycine  Ser by addition of methylene group from N5, N10-methylenetetrahydrofolic acid

Glycine  CO2 + NH4+ by the glycine cleavage complex

Front 4

Nonketogenic

deficiency of =

Back 4

glycine cleavage complex

info:
Glycine is a major inhibitory neurotransmitter

Front 5

Amino acids that form pyruvate

4 Cystine

Back 5

Cystine reduced to cysteine using NADH + H+

Cysteine -- desulfuration --- pyruvate

Hint 5

Front 6

Amino acids that form pyruvate

5 Threonine

Back 6

converted to pyruvate or a-ketobutyrate, which forms succinyl CoA

Hint 6

Front 7

Amino acids that form fumarate

Phenylalanine and Tyrosine
Degradation thereof =

Back 7

Front 8

Diseases associated with tyrosine metabolism

Albinism
deficiency of tyrosinase

Back 8

PKU
deficiency of phenylalanine hydroxylase (classic PKU) or tetrahydrobiobterin reductase (malignant PKU

Hint 8

Alkaptonuria
deficiency of homogentisate oxidase

Front 9

Amino acids that form Succinyl CoA
1Methionine

Back 9

Met  S-adenosylmethionine (SAM)

SAM is a major methyl-group donor in 1-C metabolism

Hint 9

Front 10

Resynthesis of methionine
requires vitamin B12

Back 10

Front 11

Hyperhomocysteinemia

Back 11

homocystinuria occurs due to deficiency of cystathione synthase

Front 12

Hyperhomocysteinemia
4 dz s/s

Back 12

- lens dislocation after age 3 as well as other ocular abnormalities

- osteoporosis develops during childhood, mental retardation

- lipid deposits form atheromas

- homocysteine can have other effects: lipid oxidation and platelet aggregation leads to fibrosis and calcification of atherosclerotic plaques

Front 13

Diseases associated with methionine metabolism

Back 13

Front 14

Amino acids that form Succinyl CoA


Valine and isoleucine

Back 14

branched chain amino acids

methylmalonyl CoA mutase requires B12

Maple Syrup Urine Disease (MSUD) due to a deficiency in branched chain a-keto acid dehydrogenase

Hint 14

Front 15

Diseases associated with threonine metabolism

Back 15

Front 16

Amino acids that form Succinyl CoA

Threonine

Back 16

can also be converted to pyruvate

Hint 16

Front 17

Amino acids that form Acetyl CoA or Acetoacetyl CoA

4 amino acids form acetyl CoA or acetoacetyl CoA directly without forming pyruvate:

Back 17

leucine 
isoleucine 
lysine
phenylalanine and tyrosine also give rise to

Hint 17

acetoacetate

acetyl CoA (also propionyl CoA)

acetyl CoA

acetoacetate during their catabolism