PIPOX - pipecolic acid and sarcosine oxidase Gene

Also Known as LPIPOX

Species: Homo sapiens

Gene Type: protein coding
Gene ID: 51268

About PIPOX

Cytogenetic location: 17q11.2 Genomic coordinates (GRCh38): 17:29,043,141-29,057,216 (from NCBI)

This gene has 10 transcripts (splice variants) and 203 orthologues. Biased expression in liver (RPKM 113.3) and kidney (RPKM 56.6).

Summary

Enables L-pipecolate oxidase activity and sarcosine oxidase activity. Involved in L-lysine catabolic process to acetyl-CoA via L-pipecolate. Located in peroxisome. [provided by Alliance of Genome Resources, Apr 2022]

PIPOX Products (1)

mRNA Protein Name
NM_016518.3 NP_057602.2 peroxisomal sarcosine oxidase
Molecular Function GO Annotation Evidence References Source
enables L-pipecolate oxidase activity IDA
IDA: Inferred from direct assay
10642506 GOA
enables protein binding IPI
IPI: Inferred from physical interaction
21044950 GOA
enables sarcosine oxidase activity IDA
IDA: Inferred from direct assay
10642506 GOA
Biological Process GO Annotation Evidence References Source
involved in L-lysine catabolic process to acetyl-CoA via L-pipecolate IDA
IDA: Inferred from direct assay
10642506 GOA
Cellular Component GO Annotation Evidence References Source
located in peroxisome IDA
IDA: Inferred from direct assay
10642506 GOA
EXP: Inferred from Experiment IDA: Inferred from direct assay IPI: Inferred from physical interaction IMP: Inferred from mutant phenotype IGI: Inferred from genetic interaction IEP: Inferred from expression pattern

PIPOX Protein Structure

DAO

DAO: FAD dependent oxidoreductase (9 - 363)

  • 0
  • 100
  • 200
  • 300
  • 390 a.a.
Protein Preferred Names Protein Names

peroxisomal sarcosine oxidase

  • L-pipecolate oxidase

PIPOX Protein-protein interaction Information

Type
Protein Name Protein ID Interactor Interactor Species Interactor ID Detection Method References
Intra
PIPOX Q9P0Z9 POT1 Homo sapiens Q9NUX5 21044950
Intra
PIPOX Q9P0Z9 POT1 Homo sapiens Q9NUX5 21044950
Cross: Cross-species interaction Intra: Intraspecies interaction

Related Diseases

Diseases Alias
Peroxisome Biogenesis Disorder 1b
  • Peroxisome Biogenesis Disorder

  • Infantile Refsum Disease

  • Infantile Phytanic Acid Storage Disease

  • PBD1B

  • Refsum Disease, Infantile

  • Adrenoleukodystrophy, Autosomal Neonatal

  • Ird

  • Mild Pbd-Zsd

  • Mild Peroxisome Biogenesis Disorder-Zellweger Spectrum Disorder

  • Pbd-Zsd

  • Peroxisome Biogenesis Disorder Spectrum

  • Peroxisome Biogenesis Disorder-Zellweger Spectrum Disorder

  • Autosomal Neonatal Adrenoleukodystrophy

  • Refsum Disease Infantile

  • Peroxisome Biogenesis Disorders

  • Peroxisome Biogenesis Disorder, Type 1b

Zellweger Syndrome
  • Cerebrohepatorenal Syndrome

  • Zellweger Leukodystrophy

  • Zs

  • Congenital Iron Overload

  • Chr

  • Zws

  • Severe Pbd-Zsd

  • Severe Peroxisome Biogenesis Disorder-Zellweger Spectrum Disorder

Hyperlysinemia, Type I
  • Hyperlysinemia

  • Lysine Intolerance

  • Alpha-Aminoadipic Semialdehyde Synthase Deficiency

  • Lysine:Alpha-Ketoglutarate Reductase Deficiency

  • L-Lysine:Nad-Oxido-Reductase Deficiency

  • Lysine Alpha-Ketoglutarate Reductase Deficiency

  • Alpha-Aminoadipic Semialdehyde Deficiency Disease

  • Lysine Alpha-Ketoglutarate Reductase Deficiency Disease

  • Saccharopinuria

  • Hyperlysinemia Type I

  • Hyperlysinemias

  • L-Lysine Nad-Oxido-Reductase Deficiency

  • Familial Hyperlysinemia

  • Saccharopine Dehydrogenase Deficiency Disease

  • Hyperlysinemia, 1

  • HYPLYS1

  • Saccharopine Dehydrogenase Deficiency

Epilepsy, Pyridoxine-Dependent
  • Pyridoxine-Dependent Epilepsy

  • PDE

  • Pyridoxine Dependency With Seizures

  • Vitamin B6-Dependent Seizures

  • EPD

  • Aasa Dehydrogenase Deficiency

  • Antiquitin Deficiency

  • Pyridoxine Dependency

  • Glutamate Decarboxylase Deficiency

  • Pyridoxine-Dependent Seizures

  • Deficiency Of Glutamate Decarboxylase

Glutaric Acidemia I
  • Glutaryl-Coa Dehydrogenase Deficiency

  • GA1

  • Glutaric Acidemia Type 1

  • Glutaric Aciduria 1

  • Glutaric Aciduria Type 1

  • Glutaric Acidemia Type I

  • Glutaric Aciduria, Type 1

  • Glutaric Aciduria I

  • Ga I

  • Glutaricaciduria, Type I

  • Glutaryl-Coenzyme A Dehydrogenase Deficiency

  • Glutaric Academia Type 1

  • Glutaric Aciduria Type I

  • Ga-1

  • Gcdh Deficiency

  • Ga 1

  • Glutaric Acidemia 1

  • Gcdhd

  • Glutaric Aciduria, Type I

  • Glutaricaciduria I

  • Ga-I

  • Glutaricaciduria, Type 1

Peroxisomal Biogenesis Disorder
  • Zellweger Spectrum Disorders

  • Peroxisome Biogenesis Disorder-Zellweger Syndrome Spectrum

  • Disorders Of Peroxisome Biogenesis

  • Zellweger Spectrum

  • Zellweger Syndrome Spectrum

  • Peroxisomal Biogenesis Disorders

  • Pbd, Zss

  • Pbd-Zsd

  • Pbd-Zss

  • Pbd-Zellweger Spectrum Disorder

  • Peroxisomal Biogenesis Disorders, Zellweger Syndrome Spectrum

  • Peroxisome Biogenesis Disorder

  • Peroxisome Biogenesis Disorder Spectrum

  • Peroxisome Biogenesis Disorders

  • Zellweger Spectrum Disorder

  • Hyperpipecolic Acidaemia

Diseases Alias
Waldenstroem'S Macroglobulinemia

Waldenstroem'S Macroglobulinemia

Waldenstroem'S Macroglobulinemia

Macroglobulinemia Of Waldenstrom

Lymphoplasmacytic Lymphoma With Igm Gammopathy

Lymphoplasmacytic Lymphoma

Waldenstroem'S Macroglobulinemia

Waldenstroem'S Macroglobulinemia

Macroglobulinemia Of Waldenstrom

Lymphoplasmacytic Lymphoma With Igm Gammopathy

Lymphoplasmacytic Lymphoma

Orthologs Information

Species Symbol Source ID
Rattus norvegicus PIPOX RGD RGD:1311347
Bos taurus PIPOX VGNC VGNC:32914
Mus musculus PIPOX MGD MGI:1197006
Macaca mulatta PIPOX VGNC VGNC:75822
Canis familiaris PIPOX VGNC VGNC:44578
Felis catus PIPOX VGNC VGNC:64185