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15  structures 635  species 2  interactions 1043  sequences 18  architectures

Family: Pex14_N (PF04695)

Summary: Peroxisomal membrane anchor protein (Pex14p) conserved region

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This is the Wikipedia entry entitled "PEX14". More...

PEX14 Edit Wikipedia article

Protein PEX14 PDB 2W84.png
Available structures
PDB Ortholog search: PDBe RCSB
Aliases PEX14, NAPP2, PBD13A, Pex14p, dJ734G22.2, peroxisomal biogenesis factor 14
External IDs MGI: 1927868 HomoloGene: 37936 GeneCards: PEX14
Genetically Related Diseases
breast cancer[1]
RNA expression pattern
PBB GE PEX14 203503 s at tn.png

PBB GE PEX14 33760 at tn.png
More reference expression data
Species Human Mouse
RefSeq (mRNA)



RefSeq (protein)



Location (UCSC) Chr 1: 10.47 – 10.63 Mb Chr 4: 148.96 – 149.1 Mb
PubMed search [2] [3]
View/Edit Human View/Edit Mouse
Peroxisomal membrane anchor protein (Pex14p) conserved region
Symbol Pex14_N
Pfam PF04695
InterPro IPR006785

Peroxisomal membrane protein PEX14 is a protein that in humans is encoded by the PEX14 gene.[4][5]


This gene encodes an essential component of the peroxisomal import machinery. The protein is integrated into peroxisome membranes with its C-terminus exposed to the cytosol, and interacts with the cytosolic receptor for proteins containing a PTS1 peroxisomal targeting signal. The protein also functions as a transcriptional corepressor and interacts with a histone deacetylase. A mutation in this gene results in one form of Zellweger syndrome.[5]


PEX14 has been shown to interact with


  1. ^ "Diseases that are genetically associated with PEX14 view/edit references on wikidata". 
  2. ^ "Human PubMed Reference:". 
  3. ^ "Mouse PubMed Reference:". 
  4. ^ Fransen M, Terlecky SR, Subramani S (Jul 1998). "Identification of a human PTS1 receptor docking protein directly required for peroxisomal protein import". Proceedings of the National Academy of Sciences of the United States of America. 95 (14): 8087–92. doi:10.1073/pnas.95.14.8087. PMC 20933Freely accessible. PMID 9653144. 
  5. ^ a b "Entrez Gene: PEX14 peroxisomal biogenesis factor 14". 
  6. ^ a b Otera H, Setoguchi K, Hamasaki M, Kumashiro T, Shimizu N, Fujiki Y (Mar 2002). "Peroxisomal targeting signal receptor Pex5p interacts with cargoes and import machinery components in a spatiotemporally differentiated manner: conserved Pex5p WXXXF/Y motifs are critical for matrix protein import". Molecular and Cellular Biology. 22 (6): 1639–55. doi:10.1128/MCB.22.6.1639-1655.2002. PMC 135613Freely accessible. PMID 11865044. 
  7. ^ Okumoto K, Abe I, Fujiki Y (Aug 2000). "Molecular anatomy of the peroxin Pex12p: ring finger domain is essential for Pex12p function and interacts with the peroxisome-targeting signal type 1-receptor Pex5p and a ring peroxin, Pex10p". The Journal of Biological Chemistry. 275 (33): 25700–10. doi:10.1074/jbc.M003303200. PMID 10837480. 
  8. ^ Shimizu N, Itoh R, Hirono Y, Otera H, Ghaedi K, Tateishi K, Tamura S, Okumoto K, Harano T, Mukai S, Fujiki Y (Apr 1999). "The peroxin Pex14p. cDNA cloning by functional complementation on a Chinese hamster ovary cell mutant, characterization, and functional analysis". The Journal of Biological Chemistry. 274 (18): 12593–604. doi:10.1074/jbc.274.18.12593. PMID 10212238. 
  9. ^ a b Managadze D, Würtz C, Sichting M, Niehaus G, Veenhuis M, Rottensteiner H (Jun 2007). "The peroxin PEX14 of Neurospora crassa is essential for the biogenesis of both glyoxysomes and Woronin bodies". Traffic. 8 (6): 687–701. doi:10.1111/j.1600-0854.2007.00560.x. PMID 17461798. 

Further reading

External links

This page is based on a Wikipedia article. The text is available under the Creative Commons Attribution/Share-Alike License.

This tab holds the annotation information that is stored in the Pfam database. As we move to using Wikipedia as our main source of annotation, the contents of this tab will be gradually replaced by the Wikipedia tab.

Peroxisomal membrane anchor protein (Pex14p) conserved region Provide feedback

Family of peroxisomal membrane anchor proteins which bind the PTS1 (peroxisomal targeting signal) receptor and are required for the import of PTS1-containing proteins into peroxisomes. Loss of functional Pex14p results in defects in both the PTS1 and PTS2-dependent import pathways. Deletion analysis of this conserved region implicates it in selective peroxisome degradation. In the majority of members this region is situated at the N-terminus of the protein [1,2].

Literature references

  1. Albertini M, Rehling P, Erdmann R, Girzalsky W, Kiel JA, Veenhuis M, Kunau WH; , Cell 1997;89:83-92.: Pex14p, a peroxisomal membrane protein binding both receptors of the two PTS-dependent import pathways. PUBMED:9094717 EPMC:9094717

  2. Bellu AR, Komori M, van der Klei IJ, Kiel JA, Veenhuis M; , J Biol Chem 2001;276:44570-44574.: Peroxisome biogenesis and selective degradation converge at Pex14p. PUBMED:11564741 EPMC:11564741

External database links

This tab holds annotation information from the InterPro database.

InterPro entry IPR006785

This conserved region defines a group of peroxisomal membrane anchor proteins which bind the PTS1 (peroxisomal targeting signal) receptor and are required for the import of PTS1-containing proteins into peroxisomes. Loss of functional Pex14p results in defects in both the PTS1 and PTS2-dependent import pathways. Deletion analysis of this conserved region implicates it in selective peroxisome degradation. In the majority of members this region is situated at the N terminus of the protein [PUBMED:9094717, PUBMED:11564741].

Domain organisation

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Seed source: Pfam-B_4121 (release 7.5)
Previous IDs: none
Type: Family
Author: Waterfield DI, Finn RD
Number in seed: 144
Number in full: 1043
Average length of the domain: 120.30 aa
Average identity of full alignment: 27 %
Average coverage of the sequence by the domain: 30.13 %

HMM information View help on HMM parameters

HMM build commands:
build method: hmmbuild -o /dev/null HMM SEED
search method: hmmsearch -Z 26740544 -E 1000 --cpu 4 HMM pfamseq
Model details:
Parameter Sequence Domain
Gathering cut-off 35.0 35.0
Trusted cut-off 35.4 35.3
Noise cut-off 34.4 34.9
Model length: 157
Family (HMM) version: 12
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Species distribution

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There are 2 interactions for this family. More...

Pex19 Pex14_N


For those sequences which have a structure in the Protein DataBank, we use the mapping between UniProt, PDB and Pfam coordinate systems from the PDBe group, to allow us to map Pfam domains onto UniProt sequences and three-dimensional protein structures. The table below shows the structures on which the Pex14_N domain has been found. There are 15 instances of this domain found in the PDB. Note that there may be multiple copies of the domain in a single PDB structure, since many structures contain multiple copies of the same protein seqence.

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