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PEX3 Pex3p

PEX3, Pex3p, PAS3
The product of this gene is involved in peroxisome biosynthesis and integrity. It assembles membrane vesicles before the matrix proteins are translocated. Peroxins (PEXs) are proteins that are essential for the assembly of functional peroxisomes. The peroxisome biogenesis disorders (PBDs) are a group of genetically heterogeneous autosomal recessive, lethal diseases characterized by multiple defects in peroxisome function. The peroxisomal biogenesis disorders are a heterogeneous group with at least 14 complementation groups and with more than 1 phenotype being observed in cases falling into particular complementation groups. Although the clinical features of PBD patients vary, cells from all PBD patients exhibit a defect in the import of one or more classes of peroxisomal matrix proteins into the organelle. Defects in this gene are a cause Zellweger syndrome (ZWS). [provided by RefSeq, Oct 2008] (from NCBI)
Top mentioned proteins: ACID, CAN, PEX10, Pex13p, PEX2
Papers on PEX3
Human peroxin PEX3 is co-translationally integrated into the ER and exits the ER in budding vesicles.
Johnson et al., College Station, United States. In Traffic, Dec 2015
We show that the human PMP PEX3 inserts co-translationally into the mammalian ER via the Sec61 translocon.
The birth of yeast peroxisomes.
van der Klei et al., Groningen, Netherlands. In Biochim Biophys Acta, Oct 2015
This is especially evident for pex3 and pex19 mutants, where the localization of receptor docking proteins (Pex13, Pex14) resulted in the identification of peroxisomal membrane remnants, which do not contain other peroxisomal membrane proteins, such as the ring proteins Pex2, Pex10 and Pex12.
Multiple Domains in PEX16 Mediate Its Trafficking and Recruitment of Peroxisomal Proteins to the ER.
Kim et al., Guelph, Canada. In Traffic, Aug 2015
Recently, we showed that human PEX16 plays a critical role in the ER-dependent targeting of PMPs by mediating the recruitment of two other PMPs, PEX3 and PMP34, to the ER.
PEX16 contributes to peroxisome maintenance by constantly trafficking PEX3 via the ER.
Kim et al., Toronto, Canada. In J Cell Sci, 2014
To explore ER involvement in the maintenance of peroxisomes, we redirect a peroxisomal membrane protein (PMP), PEX3, to directly target to the ER using the N-terminal ER signal sequence from preprolactin.
The membrane peroxin PEX3 induces peroxisome-ubiquitination-linked pexophagy.
Fujiki et al., Fukuoka, Japan. In Autophagy, 2014
PEX3 is a peroxisomal membrane protein (PMP) that functions in the import of PMPs into the peroxisomal membrane and has been shown to interact with pexophagic receptor proteins during pexophagy in yeast.
Peroxisomes are required for lipid metabolism and muscle function in Drosophila melanogaster.
McNew et al., Houston, United States. In Plos One, 2013
We have generated Drosophila mutants with impaired peroxisome biogenesis by disrupting the early peroxin gene pex3, which participates in budding of pre-peroxisomes from the ER and peroxisomal membrane protein localization.
A critical reflection on the principles of peroxisome formation in yeast.
van der Klei et al., Groningen, Netherlands. In Front Physiol, 2013
In cells that lack peroxisomes, for instance in pex3 and pex19 mutants or in mutants that display inheritance defects, peroxisomes may form de novo.
Hydrophobic handoff for direct delivery of peroxisome tail-anchored proteins.
Jedd et al., Singapore, Singapore. In Nat Commun, 2013
The PEX19 protein binds peroxisome TA proteins in the cytoplasm and delivers them to the membrane through the PEX3 receptor protein.
Association between the intrinsically disordered protein PEX19 and PEX3.
Ora et al., Helsinki, Finland. In Plos One, 2013
For further insight into the interaction of PEX3 and PEX19, we used hydrogen exchange mass spectrometry to monitor conformational changes during complex formation between PEX3 and PEX19 in vitro.
Peroxisome biogenesis disorders: molecular basis for impaired peroxisomal membrane assembly: in metabolic functions and biogenesis of peroxisomes in health and disease.
Matsuzaki et al., Fukuoka, Japan. In Biochim Biophys Acta, 2012
Gene defects of peroxins required for both membrane assembly and matrix protein import are identified: ten mammalian pathogenic peroxins for ten complementation groups of PBDs, are required for matrix protein import; three, Pex3p, Pex16p and Pex19p, are shown to be essential for peroxisome membrane assembly and responsible for the most severe ZS in PBDs of three complementation groups 12, 9, and 14, respectively.
Pex3-anchored Atg36 tags peroxisomes for degradation in Saccharomyces cerevisiae.
Hettema et al., Sheffield, United Kingdom. In Embo J, 2012
It was concluded that Pex3 recruits the pexophagy receptor Atg36. This reinforces the pivotal role played by Pex3 in coordinating the size of the peroxisome pool, and establishes its role in pexophagy in S. cerevisiae.
Structural basis for docking of peroxisomal membrane protein carrier Pex19p onto its receptor Pex3p.
Kato et al., Kyoto, Japan. In Embo J, 2011
The Pex19p peptide contains a characteristic motif, consisting of the leucine triad (Leu18, Leu21, Leu22), and Phe29, which are critical for the Pex3p binding and peroxisome biogenesis.
De novo synthesis of peroxisomes upon mitochondrial targeting of Pex3p.
Erdmann et al., Bochum, Germany. In Eur J Cell Biol, 2010
Data conclude that natural or artificial targeting of Pex3p to any endomembrane may initiate peroxisome formation and that also Pex3p-containing mitochondria can serve as source for the de novo synthesis of peroxisomes.
Insights into peroxisome function from the structure of PEX3 in complex with a soluble fragment of PEX19.
Dodt et al., Tübingen, Germany. In J Biol Chem, 2010
The crystal structure of the cytosolic domain of PEX3 in complex with a PEX19-derived peptide. PEX3 adopts a novel fold that is best described as a large helical bundle.
Zellweger syndrome with unusual findings: non-immune hydrops fetalis, dermal erythropoiesis and hypoplastic toe nails.
Waterham et al., Ankara, Turkey. In J Inherit Metab Dis, 2009
Subsequent molecular genetic analysis revealed a homozygous c.856C>T mutation (R268X) in the PEX3 gene, which made this patient the third to have a defect in this gene.
A dual function for Pex3p in peroxisome formation and inheritance.
Hettema et al., Sheffield, United Kingdom. In J Cell Biol, 2009
Pex3p functions as an anchor for inheritance protein Inp1p at the peroxisomal membrane, independently of its role at the endoplasmic reticulum in peroxisome biogenesis.
A genomic integration method to visualize localization of endogenous mRNAs in living yeast.
Gerst et al., Israel. In Nat Methods, 2007
Upon coexpression of MS2-CP fused with GFP, we demonstrate the localization of endogenous mRNAs (ASH1, SRO7, PEX3 and OXA1) in living yeast (Saccharomyces cerevisiae).
Contribution of the endoplasmic reticulum to peroxisome formation.
Tabak et al., Utrecht, Netherlands. In Cell, 2005
The PEX3 protein could first be observed in the endoplasmic reticulum (ER), where it concentrates in foci that then bud off in a Pex19-dependent manner and mature into fully functional peroxisomes.
Peroxisome Biogenesis Disorders, Zellweger Syndrome Spectrum
Moser et al., Seattle, United States. In Unknown Journal, 2004
Mutations in twelve different PEX genes (PEX1, PXMP3 [PEX2], PEX3, PEX5, PEX6, PEX10, PEX12, PEX13, PEX14, PEX16, PEX19, and PEX26) — those that encode peroxins, the proteins required for normal peroxisome assembly — have been identified in PBD, ZSS.
The Pas2 protein essential for peroxisome biogenesis is related to ubiquitin-conjugating enzymes.
Kunau et al., Bochum, Germany. In Nature, 1992
3) and PAS3 (ref.
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