L-pipecolic acid oxidase (PIPOX)

L-pipecolic acid oxidase (PIPOX) is a peroxisomal flavin-dependent enzyme responsible for the oxidation of L-pipecolic acid as part of the L-lysine catabolic pathway, and it also metabolizes sarcosine[2][3]. The enzyme is encoded by the PIPOX gene and is essential for degrading L-pipecolic acid in humans, as its deficiency leads to the accumulation of this metabolite, particularly in patients with peroxisomal biogenesis disorders such as Zellweger syndrome[3]. PIPOX is structurally and functionally most similar to monomeric sarcosine oxidases, and has no significant sequence similarity to D-amino acid oxidases[3]. It is localized to the peroxisome, confirmed by its peroxisomal targeting signal[2][3]. Currently, there are no known therapeutic drugs that target PIPOX directly. However, loss of its activity is clinically relevant for the diagnosis of peroxisomal disorders; thus, measuring L-pipecolic acid levels can serve as a biomarker for disease[3].

Other names

Peroxisomal sarcosine oxidaseL-pipecolate oxidaseL-pipecolic acid oxidasePSOpipecolic acid oxidasesarcosine oxidase

Enzyme catalyzes the oxidation of L-pipecolate and sarcosine.