Reactome | PIPOX oxidises PPCA to P6C

PIPOX oxidises PPCA to P6C

Stable Identifier

R-HSA-6783880

Type

Reaction [transition]

Species

Homo sapiens

Compartment

peroxisomal matrix

Synonyms

L-pipecolate + O2 => H(+) + H2O2 + L-1-piperideine-6-carboxylate

ReviewStatus

5/5

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Peroxisomal sarcosine oxidase (PIPOX aka L-pipecolate oxidase) is an important enzyme in the pipecolate pathway of lysine degradation. The saccharopine pathway is the predominant lysine degradative pathway in extracerebral tissues, whereas the pipecolate pathway predominates in adult brain. PIPOX can mediate the oxidation of L-pipecolate (PPCA) to (S)-1-piperideine-6-carboxylate (P6C), an intermediate in equilibrium with alpha-aminoadipate delta-semialdehyde (AAS), which connects the two degradative pathways. Patients lacking PIPOX accumulate PPCA which can lead to psychiatric and neurological disorders (Dodt et al. 2000, Hallen et al. 2013).

Literature References

PubMed ID	Title	Journal	Year
10642506	L-Pipecolic acid oxidase, a human enzyme essential for the degradation of L-pipecolic acid, is most similar to the monomeric sarcosine oxidases Dodt, G, Kim, DG, Reimann, SA, Reuber, BE, McCabe, K, Gould, SJ, Mihalik, SJ	Biochem J	2000
24043460	Lysine metabolism in mammalian brain: an update on the importance of recent discoveries Hallen, A, Jamie, JF, Cooper, AJ	Amino Acids	2013