Showing chemical card for L-Pipecolic acid (CFc000000567)
Record Information
Version
1.0
Creation Date
2022-08-28 10:25:12 UTC
Update Date
2022-09-13 18:44:59 UTC
Chemfont ID
CFc000000567
Molecule Identification
Common Name
L-Pipecolic acid
Definition
L-pipecolic acid is a normal human metabolite present in human blood, where is present as the primary enantiomer of pipecolic acid. L-pipecolic acid is a cyclic imino acid (contains both imino (>C=NH) and carboxyl (-C(=O)-OH) functional groups) produced during the degradation of lysine, accumulates in body fluids of infants with generalized genetic peroxisomal disorders, including Zellweger syndrome (OMIM 214100 ), neonatal adrenoleukodystrophy (OMIM 202370 ), and infantile Refsum disease (OMIM 266510 ). L-pipecolic acid levels are also elevated in patients with chronic liver diseases. L-pipecolic acid is the substrate of delta1-piperideine-2-carboxylate reductase (EC 1.5.1.21) in the pathway of lysine degradation (PMID: 2717271 , 8305590 , 1050990 ).
Belongs to the class of organic compounds known as l-alpha-amino acids. These are alpha amino acids which have the L-configuration of the alpha-carbon atom.
