Amino Acid Reactions

Ketogenic Only

Leucine and Lysine (L’s)

Gluconeogenic Only

All except for L’s and FITTT

Gluconeogenic and Ketogenic

(FITTT) Phenylalanine, Isoleucine, Tryptophan, Tyrosine, Threonine

Leu, Ile, Val

Acetyl CoA to the TCA

Acetoacetate

Ketone Body to TCA

Propionyl CoA

Succinyl CoA to TCA

Dioxigenase

Adds 2 O2 to split the ring

TCA

SAM

Important Methyl Donor

Black Urine Disease

Absence of oxidase: Inability to break down rings.

Maple Syrup Urine Disease

Can’t degrade branched AA (Val, Ile, Leu)

Phenylketonuria (PKU)

Absence of Phenylalanine hydrolase or tetrahydrobiopterin

Heme goes to

Bilirubin