Peroxisome Biogenesis Pathway

Preferred Label : Peroxisome Biogenesis Pathway;

NCIt related terms : Peroxisome;

Alternative definition : KEGG: Peroxisomes are essential organelles that play a key role in redox signaling and lipid homeostasis. They contribute to many crucial metabolic processes such as fatty acid oxidation, biosynthesis of ether lipids and free radical detoxification. The biogenesis of peroxisomes starts with the early peroxins PEX3, PEX16 and PEX19 and proceeds via several steps. The import of membrane proteins into peroxisomes needs PEX19 for recognition, targeting and insertion via docking at PEX3. Matrix proteins in the cytosol are recognized by peroxisomal targeting signals (PTS) and transported to the docking complex at the peroxisomal membrane. Peroxisomes' deficiencies lead to severe and often fatal inherited peroxisomal disorders (PD). PDs are usually classified in two groups. The first group is disorders of peroxisome biogenesis which include Zellweger syndrome, and the second group is single peroxisomal enzyme deficiencies.;

KEGG ID : hsa04146;

Details

Origin ID

C91498;

UMLS CUI

C2984308;

Automatic exact mappings (from CISMeF team)
- Microbody (cell structure) [SNOMED CT concept]
- Peroxisome [FMA entity]
- hydroxysteroid 17-beta dehydrogenase 4 [HGNC gene]
- microbodies [MeSH Descriptor]
- peroxisome [IUPAC Term]
- peroxisome [GO term]
- peroxisomes [MeSH concept]
- peroxisomes [MeSH Descriptor]
Semantic type(s)
- Functional Concept [UMLS semantic type]
has_gene_product_element
- 2-Hydroxyacyl-CoA Lyase 1 [NCIt concept]
- ATP-Binding Cassette Sub-Family D Member 1 [NCIt concept]
- Alpha-Methylacyl-CoA Racemase [NCIt concept]
- Catalase [NCIt concept]
- D-Amino-Acid Oxidase [NCIt concept]
- Enoyl-CoA Delta Isomerase 2, Mitochondrial [NCIt concept]
- Long-Chain-Fatty-Acid-CoA Ligase 1 [NCIt concept]
- Long-Chain-Fatty-Acid-CoA Ligase 3 [NCIt concept]
- Long-Chain-Fatty-Acid-CoA Ligase 6 [NCIt concept]
- Lysosomal Cobalamin Transporter ABCD4 [NCIt concept]
- Mevalonate Kinase [NCIt concept]
- Nitric Oxide Synthase, Inducible [NCIt concept]
- Peroxisomal Acyl-Coenzyme A Oxidase 1 [NCIt concept]
- Peroxisomal Acyl-Coenzyme A Oxidase 2 [NCIt concept]
- Peroxisomal Multifunctional Enzyme Type 2 [NCIt concept]
- Superoxide Dismutase [Cu-Zn] [NCIt concept]
- Superoxide Dismutase [Mn], Mitochondrial [NCIt concept]
- Xanthine Dehydrogenase/Oxidase [NCIt concept]
pathway_has_gene_element
- ABCD1 wt Allele [NCIt concept]
- ABCD4 wt Allele [NCIt concept]
- ACOX1 wt Allele [NCIt concept]
- ACOX2 wt Allele [NCIt concept]
- ACSL1 wt Allele [NCIt concept]
- ACSL3 wt Allele [NCIt concept]
- ACSL6 wt Allele [NCIt concept]
- AMACR wt Allele [NCIt concept]
- CAT wt Allele [NCIt concept]
- DAO wt Allele [NCIt concept]
- ECI2 wt Allele [NCIt concept]
- HACL1 wt Allele [NCIt concept]
- HSD17B4 wt Allele [NCIt concept]
- MVK wt Allele [NCIt concept]
- NOS2 wt Allele [NCIt concept]
- SOD1 wt Allele [NCIt concept]
- SOD2 wt Allele [NCIt concept]
- XDH wt Allele [NCIt concept]

Keyword's position in hierarchy(ies) :

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