ID:PEX14_HUMAN DESCRIPTION: RecName: Full=Peroxisomal membrane protein PEX14; AltName: Full=PTS1 receptor-docking protein; AltName: Full=Peroxin-14; AltName: Full=Peroxisomal membrane anchor protein PEX14; FUNCTION: Component of the peroxisomal translocation machinery with PEX13 and PEX17. Interacts with both the PTS1 and PTS2 receptors. Binds directly to PEX17. SUBUNIT: Interacts with PEX5 and PEX19. INTERACTION: P40855:PEX19; NbExp=4; IntAct=EBI-594898, EBI-594747; SUBCELLULAR LOCATION: Peroxisome membrane; Peripheral membrane protein; Cytoplasmic side. DISEASE: Defects in PEX14 are the cause of peroxisome biogenesis disorder complementation group K (PBD-CGK) [MIM:601791]. PBD-CGK is 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). DISEASE: Defects in PEX14 are a cause of Zellweger syndrome (ZWS) [MIM:214100]. ZWS is a fatal peroxisome biogenesis disorder characterized by dysmorphic facial features, hepatomegaly, ocular abnormalities, renal cysts, hearing impairment, profound psychomotor retardation, severe hypotonia and neonatal seizures. Death occurs within the first year of life. SIMILARITY: Belongs to the peroxin-14 family. WEB RESOURCE: Name=GeneReviews; URL="http://www.ncbi.nlm.nih.gov/sites/GeneTests/lab/gene/PEX14";
The RNAfold program from the Vienna RNA Package is used to perform the secondary structure predictions and folding calculations. The estimated folding energy is in kcal/mol. The more negative the energy, the more secondary structure the RNA is likely to have.
ModBase Predicted Comparative 3D Structure on O75381
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Orthologous Genes in Other Species
Orthologies between human, mouse, and rat are computed by taking the best BLASTP hit, and filtering out non-syntenic hits. For more distant species reciprocal-best BLASTP hits are used. Note that the absence of an ortholog in the table below may reflect incomplete annotations in the other species rather than a true absence of the orthologous gene.
Biological Process: GO:0006625 protein targeting to peroxisome GO:0007031 peroxisome organization GO:0015031 protein transport GO:0016558 protein import into peroxisome matrix GO:0016561 protein import into peroxisome matrix, translocation GO:0016567 protein ubiquitination GO:0032091 negative regulation of protein binding GO:0034453 microtubule anchoring GO:0036250 peroxisome transport along microtubule GO:0043433 negative regulation of sequence-specific DNA binding transcription factor activity GO:0044721 protein import into peroxisome matrix, substrate release GO:0045892 negative regulation of transcription, DNA-templated GO:0051260 protein homooligomerization GO:0065003 macromolecular complex assembly GO:1901094 negative regulation of protein homotetramerization
AK293684 - Homo sapiens cDNA FLJ50455 complete cds, highly similar to Peroxisomal membrane protein PEX14. AK307416 - Homo sapiens cDNA, FLJ97364. AK298136 - Homo sapiens cDNA FLJ51918 complete cds, highly similar to Peroxisomal membrane protein PEX14. AK002194 - Homo sapiens cDNA FLJ11332 fis, clone PLACE1010599, highly similar to Peroxisomal membrane protein PEX14. BC006327 - Homo sapiens peroxisomal biogenesis factor 14, mRNA (cDNA clone MGC:12767 IMAGE:4138822), complete cds. BC017848 - Homo sapiens peroxisomal biogenesis factor 14, mRNA (cDNA clone IMAGE:4696946), partial cds. AK310726 - Homo sapiens cDNA, FLJ17768. AK313046 - Homo sapiens cDNA, FLJ93521, Homo sapiens peroxisomal biogenesis factor 14 (PEX14), mRNA. AF045186 - Homo sapiens peroxisomal membrane anchor protein HsPex14p (PEX14) mRNA, complete cds. AB017546 - Homo sapiens Pex14 mRNA for peroxisomal membrane anchor protein, complete cds. CU675355 - Synthetic construct Homo sapiens gateway clone IMAGE:100019144 5' read PEX14 mRNA. AB527589 - Synthetic construct DNA, clone: pF1KB6583, Homo sapiens PEX14 gene for peroxisomal biogenesis factor 14, without stop codon, in Flexi system. CR450321 - Homo sapiens full open reading frame cDNA clone RZPDo834G031D for gene PEX14, peroxisomal biogenesis factor 14; complete cds; without stopcodon. DQ895905 - Synthetic construct Homo sapiens clone IMAGE:100010365; FLH189614.01L; RZPDo839H0264D peroxisomal biogenesis factor 14 (PEX14) gene, encodes complete protein. CR542083 - Homo sapiens full open reading frame cDNA clone RZPDo834B0137D for gene PEX14, peroxisomal biogenesis factor 14; complete cds, incl. stopcodon. DQ892775 - Synthetic construct clone IMAGE:100005405; FLH189618.01X; RZPDo839H0274D peroxisomal biogenesis factor 14 (PEX14) gene, encodes complete protein. AK307462 - Homo sapiens cDNA, FLJ97410. BC054017 - Homo sapiens peroxisomal biogenesis factor 14, mRNA (cDNA clone IMAGE:6739162), partial cds. JD241985 - Sequence 223009 from Patent EP1572962. JD423149 - Sequence 404173 from Patent EP1572962. JD230951 - Sequence 211975 from Patent EP1572962. JD227000 - Sequence 208024 from Patent EP1572962. JD414612 - Sequence 395636 from Patent EP1572962. JD185625 - Sequence 166649 from Patent EP1572962. JD477550 - Sequence 458574 from Patent EP1572962. JD322830 - Sequence 303854 from Patent EP1572962. JD044749 - Sequence 25773 from Patent EP1572962. JD335886 - Sequence 316910 from Patent EP1572962. JD525528 - Sequence 506552 from Patent EP1572962. JD518862 - Sequence 499886 from Patent EP1572962. JD444488 - Sequence 425512 from Patent EP1572962. JD409470 - Sequence 390494 from Patent EP1572962. JD519542 - Sequence 500566 from Patent EP1572962. JD260851 - Sequence 241875 from Patent EP1572962. JD475828 - Sequence 456852 from Patent EP1572962. JD116800 - Sequence 97824 from Patent EP1572962. JD250418 - Sequence 231442 from Patent EP1572962. JD106717 - Sequence 87741 from Patent EP1572962.
Biochemical and Signaling Pathways
Reactome (by CSHL, EBI, and GO)
Protein O75381 (Reactome details) participates in the following event(s):
R-HSA-8953917 PEX2:PEX10:PEX12 binds PEX5S,L (in PEX5S:PEX13:PEX14) and Ub:UBE2D1,2,3 R-HSA-9033516 Ub:PEX5L (in PEX2:PEX10:PEX12:Ub:PEX5L:PEX7:PEX13:PEX14) binds PEX1:PEX6:PEX26 and ZFAND6 R-HSA-9033485 PEX2:PEX10:PEX12 monoubiquitinates PEX5L at cysteine-11 R-HSA-9033527 PEX2:PEX10:PEX12 binds PEX5L (in PEX5L:PEX7:PEX13:PEX14:PEX2:PEX10:PEX12) and Ub:UBE2D1,2,3 R-HSA-9033533 Ub:PEX5S,L (in PEX2:PEX10:PEX12:Ub:PEX5S:PEX13:PEX14) binds PEX1:PEX6:PEX26 and ZFAND6 R-HSA-8953946 PEX2:PEX10:PEX12 monoubiquitinates PEX5S,L at cysteine-11 R-HSA-9033236 PEX5S,L:Cargo binds PEX13:PEX14 of PEX13:PEX14:PEX2:PEX10:PEX12 (Docking and Translocation Complex) R-HSA-9033238 PEX5L:PEX7:Cargo binds PEX13:PEX14 of PEX13:PEX14:PEX2:PEX10:PEX12 (Docking and Translocation Complex) R-HSA-8866654 E3 ubiquitin ligases ubiquitinate target proteins R-HSA-9033241 Peroxisomal protein import R-HSA-8852135 Protein ubiquitination R-HSA-392499 Metabolism of proteins R-HSA-597592 Post-translational protein modification
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Sequence and Links to Tools and Databases 
Common Gene Haplotype Alleles 
