VITAMIN

Disorders Of Methionine And Cysteine Metabolism

Cystathionine

Cysteine

dietary deficiency;
processing or
attachment to enzyme

Cystathionase
Cystathionine -synthase (requires pyridoxal phosphate)
CH3 group adenosine
donation

X defect

HOMOCYSTINURIA
Link to atherosclerosis?

S-Adenosylmethionine

Homocysteine-SH CH3-B12

Methionine
adenosyltransferase
ATP

Methionine-S-CH3

Methionine synthase

X deficiency
THF

X defect

X defect
Hydroxy B12

X deficiency

N5-CH3 THF

MTHFR
defect 7

S-Adenosylmethionine Homocysteine-SH methyl-B12

THF
4

CYTOPLASM

Methionine synthase
ATP

Methionine-SCH3

in methyl trap
with B12 deficiency

N5-methyl THF

1
Hydroxy B12

From diet

2 Adenosyl B12

Succinyl CoA

Methylmalonyl CoA
mutase

Methylmalonyl
CoA
Methylmalonyl

CoA

Propionyl CoA carboxylase

TCA
cycle

biotin
MITOCHONDRION

Propionyl CoA

Figure 5. Metabolism of cobalamin and associated disorders

(homocystinuria and methylmalonic aciduria)

Leucine
-Ketoglutarate

Valine

Isoleucine

Glutamate

Transaminase

Branched-chain -ketoacid
CoA + NAD+

CO2 + NADH

Branched-chain ketoacid
dehydrogenase

CoA derivatives of each branched-chain ketoacid

biotin

3 Acetyl CoA
from leucine

Propionyl CoA
biotin

i
Succinyl CoA

Methylmalonyl CoA
adenosyl B12

Figure 6. Pathways for the catabolism of leucine, isoleucine and valine