Entity Details

Primary name PEX26_HUMAN
Entity type UniProt
Source Source Link

Details

AccessionQ7Z412
EntryNamePEX26_HUMAN
FullNamePeroxisome assembly protein 26
TaxID9606
Evidenceevidence at protein level
Length305
SequenceStatuscomplete
DateCreated2004-05-24
DateModified2021-06-02

Ontological Relatives

GenesPEX26

GO terms

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GOName
GO:0005777 peroxisome
GO:0005778 peroxisomal membrane
GO:0005779 integral component of peroxisomal membrane
GO:0005829 cytosol
GO:0008022 protein C-terminus binding
GO:0008104 protein localization
GO:0016558 protein import into peroxisome matrix
GO:0044877 protein-containing complex binding
GO:0045046 protein import into peroxisome membrane
GO:0051117 ATPase binding

Subcellular Location

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Subcellular Location
Peroxisome membrane

Domains

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DomainNameCategoryType
IPR010797 Peroxisome assembly protein 26FamilyFamily

Diseases

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Disease IDSourceNameDescription
614873 OMIMPeroxisome biogenesis disorder 7B (PBD7B)A peroxisome biogenesis disorder that includes neonatal adrenoleukodystrophy (NALD) and infantile Refsum disease (IRD), two milder manifestations of the Zellweger disease spectrum. The clinical course of patients with the NALD and IRD presentation is variable and may include developmental delay, hypotonia, liver dysfunction, sensorineural hearing loss, retinal dystrophy and vision impairment. Children with the NALD presentation may reach their teens, while patients with the IRD presentation may reach adulthood. The clinical conditions are often slowly progressive in particular with respect to loss of hearing and vision. The biochemical abnormalities include accumulation of phytanic acid, very long chain fatty acids (VLCFA), di- and trihydroxycholestanoic acid and pipecolic acid. The disease is caused by variants affecting the gene represented in this entry.
614872 OMIMPeroxisome biogenesis disorder complementation group 8 (PBD-CG8)A peroxisomal disorder arising from a failure of protein import into the peroxisomal membrane or matrix. The peroxisome biogenesis disorders (PBD group) are genetically heterogeneous with at least 14 distinct genetic groups as concluded from complementation studies. Include disorders are: Zellweger syndrome (ZWS), neonatal adrenoleukodystrophy (NALD), infantile Refsum disease (IRD), and classical rhizomelic chondrodysplasia punctata (RCDP). ZWS, NALD and IRD are distinct from RCDP and constitute a clinical continuum of overlapping phenotypes known as the Zellweger spectrum (PBD-ZSS). The disease is caused by variants affecting the gene represented in this entry.
614872 OMIMPeroxisome biogenesis disorder complementation group 8 (PBD-CG8)A peroxisomal disorder arising from a failure of protein import into the peroxisomal membrane or matrix. The peroxisome biogenesis disorders (PBD group) are genetically heterogeneous with at least 14 distinct genetic groups as concluded from complementation studies. Include disorders are: Zellweger syndrome (ZWS), neonatal adrenoleukodystrophy (NALD), infantile Refsum disease (IRD), and classical rhizomelic chondrodysplasia punctata (RCDP). ZWS, NALD and IRD are distinct from RCDP and constitute a clinical continuum of overlapping phenotypes known as the Zellweger spectrum (PBD-ZSS). The disease is caused by variants affecting the gene represented in this entry.

Interactions

38 interactions

InteractorPartnerSourcesPublicationsLink
PEX26_HUMANSUFU_HUMANBioGRID, HPRD, IntAct16189514 details
PEX26_HUMANPEX1_HUMANBioGRID, IntAct12717447 16257970 details
PEX26_HUMANPEX6_HUMANBioGRID, HPRD, IntAct12717447 16257970 30366024 details
PEX26_HUMANPEX19_HUMANBioGRID, IntAct, MINT20531392 30366024 32296183 details
PEX26_HUMANAP2B1_HUMANIntAct32814053 details
PEX26_HUMANWDR61_HUMANIntAct32814053 details
PEX26_HUMANE2F8_HUMANIntAct32814053 details
PEX26_HUMANBBLN_HUMANIntAct32814053 details
PEX26_HUMANKCD15_HUMANIntAct32814053 details
PEX26_HUMANLHX5_HUMANIntAct32814053 details
PEX26_HUMANAB17C_HUMANIntAct32814053 details
PEX26_HUMANMSRB2_HUMANIntAct32814053 details
PEX26_HUMANNEBL_HUMANIntAct32814053 details
PEX26_HUMANBAIP2_HUMANIntAct32814053 details
PEX26_HUMANIQEC1_HUMANIntAct32814053 details
PEX26_HUMANCSN3_HUMANIntAct32814053 details
PEX26_HUMANRN112_HUMANIntAct32814053 details
PEX26_HUMANMKRN3_HUMANIntAct32814053 details
PEX26_HUMANP53_HUMANIntAct32814053 details
PEX26_HUMANPFD5_HUMANIntAct32814053 details
PEX26_HUMANMEOX1_HUMANIntAct32814053 details
PEX26_HUMANDAXX_HUMANIntAct32814053 details
PEX26_HUMANBIRC5_HUMANIntAct32814053 details
PEX26_HUMANHDA10_HUMANIntAct32814053 details
PEX26_HUMANBMF_HUMANIntAct32814053 details
PEX26_HUMANCBWD3_HUMANIntAct32814053 details
PEX26_HUMANARL16_HUMANIntAct32814053 details
PEX26_HUMANKR191_HUMANIntAct32814053 details
PEX26_HUMANKRA81_HUMANIntAct32814053 details
PEX26_HUMANFA78A_HUMANIntAct32814053 details
PEX26_HUMANFOXR1_HUMANIntAct32814053 details
PEX26_HUMANSAS6_HUMANIntAct32814053 details
PEX26_HUMANZMAT2_HUMANIntAct32814053 details
PEX26_HUMANKLC3_HUMANIntAct32814053 details
PEX26_HUMANASCL4_HUMANIntAct32814053 details
PEX26_HUMANPEX5_HUMANBioGRID30375424 details
PEX26_HUMANPEX14_HUMANBioGRID30366024 30375424 details
PEX26_HUMANPEX26_HUMANBioGRID30366024 details