L-Pipecolic acid

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L-Pipecolic acid
2D structure for L-Pipecolic acid
Chemical Name	(2S)-piperidine-2-carboxylic acid
Chemical Formula	C₆H₁₁NO₂
CAS Number	3105-95-1
Chemical Information	HMDB00716
Biochemical Taxonomy	Amino Acids
Functional Taxonomy	Not Available
Nutritional Taxonomy	Not Available
Metabolic Pathways	Not Available
Biofluid Location	Blood
Tissue Location	Liver
Normal Biofluid Concentrations	Blood: 10.0 (0.0-20.0) uM
Normal Tissue Concentrations	Not Available
Diseases / Conditions Related to Nutrition	Neonatal ALD Zellweger syndrome
Other (Monogenic Disorders)	Not Available
Abnormal Biofluid Concentrations	Blood (Neonatal ALD): 65.0 +/- 63.0 uM Blood (Zellweger syndrome): 9.7 +/- 3.1 uM
Abnormal Tissue Concentrations	Not Available
Physiological Processes	Not Available

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Introduction

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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 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)