Citrate

-KetoglutarateSuccinyl CoA

Fumarate

Oxaloacetate

Pyruvate

Acetyl CoA Acetoacetyl CoA

AlanineGlycineCysteineSerineThreonineTryptophan

IsoleucineLeucineTryptophan

GlutamateGlutamineHistidineProlineArginine

LeucineLysinePhenylalanineTyrosineTryptophan

IsoleucineMethionineValine

AspartateAsparagine

TyrosinePhenylalanineAspartate

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

Glutamate FamilyL-Arginine

Ornithine COO-

H3N-C-H

CH2

CH2

COO-

+

COO-NH

CC C

CH2

H2 H2COO-

H3N-C-H

CH2

CH2

CHO

+COO-

H3N-C-H

CH2

CH2

CH2

+

NH3

L-Histidine L-Glutamine

-Kg Glu

L-ProlineGlutamateSemialdehyde

H2ONH4

+

NAD(P)

H2O

L-Glutamate -Ketoglutarate

L-Phenylalanine

CH2C-COO-H

NH3

HO

L-PhenylalanineCH2C-COO-

H

NH3

CH2-COO-

Phenylpyruvate

CH2C-COO-H

OHPhenyllactate

L-Tyrosine Phenylketonuria CH2C-COO-

O

Phenylacetate

CO2

Pyruvate

Alanine

H2O

Phenylalaninehydroxylase

O2

NADH

CH2C-COO-H

NH3

HO

CH2 C-COO-

O

HO

P. 637

Homogentisate

CH2-COO-

HO OH

H

O

COO-

C=CO=C

CH2 CH2-COO-C

H

C=CH

O

COO-O=C

CH2 CH2-COO-C

H

Fumarate + acetoacetate

Fumarylacetoacetate

Maleylacetoacetate

Oxidized

Homogentisate oxidase

AlcaptonuriaO2

L-TyrosineTyrosine amino transferase

Methionine

Homocysteine

P 630

SHCH2

CH2

H3N-CH-COO-

OHCH2

H3N-CH-COO-

SCH2

CH2

H3N-CHCOO-

CH2

CH COO-

NH3+

Cystathione-lyase CH3

CH2

C=OCOO-

CH2-SHCH COO-

NH3+

+

-Ketobutyrate Cysteine

Cysteine is madefrom Methionine

andMethionine goes to Succinyl CoA

-H2O

Cystathionine

Serine

NH4

+

Propionyl CoA

Succinyl CoA

CH3-C-COO-

+NH3

HAlanine

Cysteine

HS-CH2C-COO-

O

CH3C-COO-

O

-Kg

Glutamate

PyruvateSO42-

Sulfate

[S]

-Mercaptopyruvate

H2S

2RSH

HS-CH2-C-COO-

+NH3

H

O2S-CH2-C-COO-

+NH3

H=

Taurine

CO2

O2

Serine-H2O-NH4

Glycine

O3S-CH2-CH2-NH3+=

p625

-NH4+

Sulfinic acid

HO-CH2-C-COO-

+NH3

H

CH2=C-COO-

+NH3

Serinedehydratase-H2O

CH3C-COO-

OCH3-C-COO-

NH2

NH4

PyruvateH2O

Serine

Requires pyridoxal-5-PO4

Leucine Valine Isoleucine

-C-COO-

OCH

R

R’

-C-CO~SCoAO

CHR

R’

Branch chainaminotransferase

-Keto acid dehydrogenase Complex

(Not in liver)

TPPLipoateFADNAD+

HS-CoA

Maple Syrup Urine Disease