PIPOX oxidises PPCA to P6C

Stable Identifier
R-HSA-6783880
Type
Reaction
Species
Homo sapiens
Compartment
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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

Mihalik, SJ, Dodt, G, McCabe, K, Gould, SJ, Reimann, SA, Kim, DG, Reuber, BE

Biochem J 2000
24043460 Lysine metabolism in mammalian brain: an update on the importance of recent discoveries

Cooper, AJ, Jamie, JF, Hallen, A

Amino Acids 2013
Participants
Participates
Catalyst Activity

L-pipecolate oxidase activity of PIPOX [peroxisomal matrix]

Orthologous Events
Cross References
Rhea
Authored
Reviewed
Created
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