PIPOX oxidises PPCA to P6C

Stable Identifier
R-HSA-6783880
Type
Reaction [transition]
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
24043460 Lysine metabolism in mammalian brain: an update on the importance of recent discoveries

Hallen, A, Jamie, JF, Cooper, AJ

Amino Acids 2013
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
Participants
Participant Of
Catalyst Activity
Catalyst Activity
Title
L-pipecolate oxidase activity of PIPOX [peroxisomal matrix]
Physical Entity
Activity
Orthologous Events
Cross References
Rhea
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Reviewed
Created