PEX5

Last updated
PEX5
Protein PEX5 PDB 1fch.png
Available structures
PDB Ortholog search: PDBe RCSB
Identifiers
Aliases PEX5 , peroxisomal biogenesis factor 5, RCDP5, PTS1R, PBD2B, PTS1-BP, PXR1, PBD2A
External IDs OMIM: 600414 MGI: 1098808 HomoloGene: 270 GeneCards: PEX5
Orthologs
SpeciesHumanMouse
Entrez
Ensembl
UniProt
RefSeq (mRNA)

NM_001277330
NM_001277805
NM_008995
NM_175933
NM_001360570

RefSeq (protein)

NP_001264259
NP_001264734
NP_033021
NP_787947
NP_001347499

Location (UCSC) Chr 12: 7.19 – 7.22 Mb Chr 6: 124.37 – 124.39 Mb
PubMed search [3] [4]
Wikidata
View/Edit Human View/Edit Mouse

Peroxisomal targeting signal 1 receptor (PTS1R) is a protein that in humans is encoded by the PEX5 gene. [5]

PTS1R is a peroxisomal targeting sequence involved in the specific transport of molecules for oxidation inside the peroxisome. SKL binds to PTS1R in the cytosol followed by binding to the Pex14p receptor allowing importation of the peroxisomal protein through the pexsubunit transporter.

Diseases associated with dysfunctional PTS1R receptors include X-linked adrenoleukodystrophy and Zellweger syndrome.

Interactions

PEX5 has been shown to interact with PEX12, [6] [7] PEX13 [8] [9] and PEX14. [7] [9] [10]

Related Research Articles

<span class="mw-page-title-main">Peroxisome</span> Type of organelle

A peroxisome (IPA:[pɛɜˈɹɒksɪˌsoʊm]) is a membrane-bound organelle, a type of microbody, found in the cytoplasm of virtually all eukaryotic cells. Peroxisomes are oxidative organelles. Frequently, molecular oxygen serves as a co-substrate, from which hydrogen peroxide (H2O2) is then formed. Peroxisomes owe their name to hydrogen peroxide generating and scavenging activities. They perform key roles in lipid metabolism and the reduction of reactive oxygen species.

Protein targeting or protein sorting is the biological mechanism by which proteins are transported to their appropriate destinations within or outside the cell. Proteins can be targeted to the inner space of an organelle, different intracellular membranes, the plasma membrane, or to the exterior of the cell via secretion. Information contained in the protein itself directs this delivery process. Correct sorting is crucial for the cell; errors or dysfunction in sorting have been linked to multiple diseases.

In biochemical protein targeting, a peroxisomal targeting signal (PTS) is a region of the peroxisomal protein that receptors recognize and bind to. It is responsible for specifying that proteins containing this motif are localised to the peroxisome.

<span class="mw-page-title-main">PEX1</span> Protein-coding gene in the species Homo sapiens

Peroxisome biogenesis factor 1, also known as PEX1, is a protein which in humans is encoded by the PEX1 gene.

<span class="mw-page-title-main">PEX19</span> Protein-coding gene in the species Homo sapiens

Peroxisomal biogenesis factor 19 is a protein that in humans is encoded by the PEX19 gene.

<span class="mw-page-title-main">PEX14</span> Protein-coding gene in the species Homo sapiens

Peroxisomal membrane protein PEX14 is a protein that in humans is encoded by the PEX14 gene.

<span class="mw-page-title-main">ABCD3</span> Protein-coding gene in the species Homo sapiens

ATP-binding cassette sub-family D member 3 is a protein that in humans is encoded by the ABCD3 gene.

<span class="mw-page-title-main">Peroxisomal biogenesis factor 2</span> Protein found in humans

Peroxisomal biogenesis factor 2 is a protein that in humans is encoded by the PEX2 gene.

<span class="mw-page-title-main">PEX12</span> Protein-coding gene in humans

Peroxisome assembly protein 12 is a protein that in humans is encoded by the PEX12 gene.

<span class="mw-page-title-main">PEX13</span> Protein-coding gene in the species Homo sapiens

Peroxisomal membrane protein PEX13 is a protein that in humans is encoded by the PEX13 gene. It located on chromosome 2 next to KIAA1841

<span class="mw-page-title-main">PEX3</span> Protein-coding gene in the species Homo sapiens

Peroxisomal biogenesis factor 3 is a protein that in humans is encoded by the PEX3 gene.

<span class="mw-page-title-main">PEX6</span> Protein-coding gene in the species Homo sapiens

Peroxisome assembly factor 2 is a protein that in humans is encoded by the PEX6 gene. PEX6 is an AAA ATPase that localizes to the peroxisome. PEX6 forms a hexamer with PEX1 and is recruited to the membrane by PEX26.

<span class="mw-page-title-main">PEX10</span> Protein-coding gene in the species Homo sapiens

Peroxisome biogenesis factor 10 is a protein that in humans is encoded by the PEX10 gene. Alternative splicing results in two transcript variants encoding different isoforms.

<span class="mw-page-title-main">Peroxisomal membrane protein PMP34</span> Protein found in humans

Peroxisomal membrane protein PMP34 is a protein that in humans is encoded by the SLC25A17 gene.

<span class="mw-page-title-main">PEX16</span> Protein-coding gene in the species Homo sapiens

Peroxisomal membrane protein PEX16 is a protein that in humans is encoded by the PEX16 gene.

<span class="mw-page-title-main">PEX26</span> Protein-coding gene in the species Homo sapiens

Peroxisome assembly protein 26 is a protein that in humans is encoded by the PEX26 gene.

<span class="mw-page-title-main">PEX11B</span> Protein-coding gene in the species Homo sapiens

Peroxisomal membrane protein 11B is a protein that in humans is encoded by the PEX11B gene. It is involved in the regulation of peroxisome abundance.

<span class="mw-page-title-main">PEX11A</span> Protein-coding gene in the species Homo sapiens

Peroxisomal membrane protein 11A is a protein that in humans is encoded by the PEX11A gene.

<span class="mw-page-title-main">PXMP4</span> Protein-coding gene in the species Homo sapiens

Peroxisomal membrane protein 4 is a protein that in humans is encoded by the PXMP4 gene.

<span class="mw-page-title-main">Tetratricopeptide repeat</span> Protein tandem repeat

The tetratricopeptide repeat (TPR) is a structural motif. It consists of a degenerate 34 amino acid tandem repeat identified in a wide variety of proteins. It is found in tandem arrays of 3–16 motifs, which form scaffolds to mediate protein–protein interactions and often the assembly of multiprotein complexes. These alpha-helix pair repeats usually fold together to produce a single, linear solenoid domain called a TPR domain. Proteins with such domains include the anaphase-promoting complex (APC) subunits cdc16, cdc23 and cdc27, the NADPH oxidase subunit p67-phox, hsp90-binding immunophilins, transcription factors, the protein kinase R (PKR), the major receptor for peroxisomal matrix protein import PEX5, protein arginine methyltransferase 9 (PRMT9), and mitochondrial import proteins.

References

  1. 1 2 3 ENSG00000288217 GRCh38: Ensembl release 89: ENSG00000139197, ENSG00000288217 - Ensembl, May 2017
  2. 1 2 3 GRCm38: Ensembl release 89: ENSMUSG00000005069 - Ensembl, May 2017
  3. "Human PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
  4. "Mouse PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
  5. "Entrez Gene: PEX5 Peroxisomal biogenesis factor 5".
  6. Chang CC, Warren DS, Sacksteder KA, Gould SJ (Nov 1999). "PEX12 interacts with PEX5 and PEX10 and acts downstream of receptor docking in peroxisomal matrix protein import". The Journal of Cell Biology. 147 (4): 761–74. doi:10.1083/jcb.147.4.761. PMC   2156163 . PMID   10562279.
  7. 1 2 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. Gould SJ, Kalish JE, Morrell JC, Bjorkman J, Urquhart AJ, Crane DI (Oct 1996). "Pex13p is an SH3 protein of the peroxisome membrane and a docking factor for the predominantly cytoplasmic PTs1 receptor". The Journal of Cell Biology. 135 (1): 85–95. doi:10.1083/jcb.135.1.85. PMC   2121023 . PMID   8858165.
  9. 1 2 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   135613 . PMID   11865044.
  10. 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.

Further reading