1. Citrate  -Ketoglutarate Succinyl CoA Fumarate Oxaloacetate Pyruvate Acetyl CoAAcetoacetyl CoA Alanine Glycine Cysteine Serine Threonine Tryptophan Isoleucine Leucine Tryptophan Glutamate Glutamine Histidine Proline Arginine Leucine Lysine Phenylalanine Tyrosine Tryptophan Isoleucine Methionine Valine Aspartate Asparagine Tyrosine Phenylalanine Aspartate

2. Classification of Amino Acids Amino acids that can form gluconeogenic precursors are called “glucogenic” (pyruvate,  -ketoglutarate, succinyl-CoA, fumarate, or OAA) Amino acids that form acetyl-CoA or acetoacetate are called “ketogenic” Many are BOTH

3. Glutamate Family L-Arginine Ornithine COO - H 3 N-C-H CH 2 COO - + N H C CC CH2H2 H2H2 H2H2 H 3 N-C-H CH 2 CHO + COO - H 3 N-C-H CH 2 + NH 3 L-Histidine L-Glutamine  -Kg Glu L-Proline Glutamate Semialdehyde H2OH2O NH 4 + NAD(P) H2OH2O L-Glutamate  -Ketoglutarate

4. L-Phenylalanine CH 2 C-COO - H NH 3 HOHO L-Phenylalanine CH 2 C-COO - HNH 3 CH 2 -COO - Phenylpyruvate CH 2 C-COO - H OH Phenyllactate L-Tyrosine Phenylketonuria CH 2 C-COO - O Phenylacetate CO 2 Pyruvate Alanine H2OH2O Phenylalanine hydroxylase O2O2 NADH

5. CH 2 C-COO - H NH 3 HO CH 2 C-COO - O HO P. 637 Homogentisate CH 2 -COO - HOOH H O COO - C=C O=C CH 2 CH 2 -COO - C H C=C H O COO - O=C CH 2 CH 2 -COO - C H Fumarate + acetoacetate Fumarylacetoacetate Maleylacetoacetate Oxidized Homogentisate oxidase Alcaptonuria O2O2 L-Tyrosine Tyrosine amino transferase

6. Methionine Homocysteine P 630 SH CH 2 H 3 N-CH-COO - OH CH 2 H 3 N-CH-COO - S CH 2 H 3 N-CH COO - CH 2 CHCOO - NH 3 + Cystathione  -lyase CH 3 CH 2 C=O COO - CH 2 -SH CHCOO - NH 3 + +   -Ketobutyrate Cysteine Cysteine is made from Methionine and Methionine goes to Succinyl CoA -H 2 O Cystathionine Serine NH 4 + Propionyl CoA Succinyl CoA

7. CH 3 -C-COO - + NH 3 H Alanine Cysteine HS-CH 2 C-COO - O CH 3 C-COO - O  -Kg Glutamate Pyruvate SO 4 2- Sulfate [S]  -Mercaptopyruvate H2SH2S 2RSH HS-CH 2 -C-COO - + NH 3 H O 2 S-CH 2 -C-COO - + NH 3 H = Taurine CO 2 O2O2 Serine -H 2 O -NH 4 Glycine O 3 S-CH 2 -CH 2 -NH 3 + = p625 -NH 4 + Sulfinic acid

8. HO-CH 2 -C-COO - + NH 3 H CH 2 =C-COO - + NH 3 Serine dehydratase -H 2 O CH 3 C-COO - O CH 3 -C-COO - NH 2 NH 4 Pyruvate H2OH2O Serine Requires pyridoxal-5-PO 4

9. LeucineValineIsoleucine -C-COO - O CH R R’ -C-CO~SCoA O CH R R’ Branch chain aminotransferase  -Keto acid dehydrogenase Complex (Not in liver) TPP Lipoate FAD NAD + HS-CoA Maple Syrup Urine Disease