Entry
Name
Peroxisome - Homo sapiens (human)
Description
Peroxisomes are essential organelles that play a key role in redox signalling 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.
Class
Cellular Processes; Transport and catabolism
BRITE hierarchy
Pathway map
Ortholog table
Drug
D00224 Allopurinol (JP18/USP/INN)
D01206 Febuxostat (JAN/USAN/INN)
D11044 Enasidenib mesylate (USAN)
D11081 Febuxostat hydrate (JAN)
D11811 Tofersen (JAN/USAN/INN)
D11835 Vorasidenib citrate (USAN)
D12150 Elivaldogene autotemcel (USAN/INN)
Other DBs
Organism
Homo sapiens (human) [GN:
hsa ]
Gene
9409 PEX16; peroxisomal biogenesis factor 16 [KO:K13335 ]
8504 PEX3; peroxisomal biogenesis factor 3 [KO:K13336 ]
5824 PEX19; peroxisomal biogenesis factor 19 [KO:K13337 ]
5825 ABCD3; ATP binding cassette subfamily D member 3 [KO:K05677 ]
5189 PEX1; peroxisomal biogenesis factor 1 [KO:K13338 ]
5190 PEX6; peroxisomal biogenesis factor 6 [KO:K13339 ]
55670 PEX26; peroxisomal biogenesis factor 26 [KO:K13340 ]
5191 PEX7; peroxisomal biogenesis factor 7 [KO:K13341 ]
5830 PEX5; peroxisomal biogenesis factor 5 [KO:K13342 ]
51555 PEX5L; peroxisomal biogenesis factor 5 like [KO:K13342 ]
5195 PEX14; peroxisomal biogenesis factor 14 [KO:K13343 ]
5194 PEX13; peroxisomal biogenesis factor 13 [KO:K13344 ]
5193 PEX12; peroxisomal biogenesis factor 12 [KO:K13345 ]
5192 PEX10; peroxisomal biogenesis factor 10 [KO:K13346 ]
5828 PEX2; peroxisomal biogenesis factor 2 [KO:K06664 ]
5827 PXMP2; peroxisomal membrane protein 2 [KO:K13347 ]
4358 MPV17; mitochondrial inner membrane protein MPV17 [KO:K13348 ]
84769 MPV17L2; MPV17 mitochondrial inner membrane protein like 2 [KO:K13348 ]
255027 MPV17L; MPV17 mitochondrial inner membrane protein like [KO:K13349 ]
11264 PXMP4; peroxisomal membrane protein 4 [KO:K13350 ]
8800 PEX11A; peroxisomal biogenesis factor 11 alpha [KO:K13351 ]
8799 PEX11B; peroxisomal biogenesis factor 11 beta [KO:K13352 ]
92960 PEX11G; peroxisomal biogenesis factor 11 gamma [KO:K13353 ]
10478 SLC25A17; solute carrier family 25 member 17 [KO:K13354 ]
215 ABCD1; ATP binding cassette subfamily D member 1 [KO:K05675 ]
225 ABCD2; ATP binding cassette subfamily D member 2 [KO:K05676 ]
5826 ABCD4; ATP binding cassette subfamily D member 4 [KO:K05678 ]
10901 DHRS4; dehydrogenase/reductase 4 [KO:K11147 ] [EC:1.1.-.-]
Reference
Authors
Wanders RJ, Waterham HR
Title
Peroxisomal disorders: the single peroxisomal enzyme deficiencies.
Journal
Reference
Authors
Steinberg SJ, Dodt G, Raymond GV, Braverman NE, Moser AB, Moser HW
Title
Peroxisome biogenesis disorders.
Journal
Reference
Authors
Rottensteiner H, Theodoulou FL
Title
The ins and outs of peroxisomes: co-ordination of membrane transport and peroxisomal metabolism.
Journal
Reference
Authors
Iida R, Yasuda T, Tsubota E, Takatsuka H, Matsuki T, Kishi K
Title
Human Mpv17-like protein is localized in peroxisomes and regulates expression of antioxidant enzymes.
Journal
Related pathway
hsa00900 Terpenoid backbone biosynthesis
KO pathway