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PEX13 Pex13p

Pex13p, PEX13
This gene encodes a peroxisomal membrane protein that binds the type 1 peroxisomal targeting signal receptor via a SH3 domain located in the cytoplasm. Mutations and deficiencies in peroxisomal protein importing and peroxisome assembly lead to peroxisomal biogenesis disorders, an example of which is Zellweger syndrome. [provided by RefSeq, Oct 2008] (from NCBI)
Top mentioned proteins: PEX7, PEX12, CAN, V1a, PEX10
Papers on Pex13p
A de-novo interstitial microduplication involving 2p16.1-p15 and mirroring 2p16.1-p15 microdeletion syndrome: Clinical and molecular analysis.
Basel-Vanagaite et al., Israel. In Eur J Paediatr Neurol, Nov 2015
CONCLUSION: The involvement of genes in this location including BCL11A, USP34 and PEX13, affecting fundamental developmental processes both within and outside the nervous system may explain the clinical features of the individual described in this report.
Compromised peroxisomes in idiopathic pulmonary fibrosis, a vicious cycle inducing a higher fibrotic response via TGF-β signaling.
Baumgart-Vogt et al., Gießen, Germany. In Proc Natl Acad Sci U S A, May 2015
By comparing peroxisome-related protein and gene expression in lung tissue and isolated lung fibroblasts between human control and IPF patients, we found that IPF lungs exhibited a significant down-regulation of peroxisomal biogenesis and metabolism (e.g., PEX13p and acyl-CoA oxidase 1).
Hsa-miR-195 targets PCMT1 in hepatocellular carcinoma that increases tumor life span.
Tawab et al., Cairo, Egypt. In Tumour Biol, 2014
FGF7, GHR, PCMT1, CITED2, PEX5, PEX13, NOVA1, AXIN2, and TSPYL2 were detected with high significant (P < 0.005).
A viable Arabidopsis pex13 missense allele confers severe peroxisomal defects and decreases PEX5 association with peroxisomes.
Bartel et al., Houston, United States. In Plant Mol Biol, 2014
Using a forward-genetic screen designed to recover severe peroxisome-defective mutants, we isolated a viable allele of the peroxisome biogenesis gene PEX13 with striking peroxisomal defects.
Central serotonergic neuron deficiency in a mouse model of Zellweger syndrome.
Crane et al., Griffith, Australia. In Neuroscience, 2014
Here we present findings using a mouse model of ZS neuropathology with conditional brain inactivation of the PEX13 gene.
Arabidopsis DAYU/ABERRANT PEROXISOME MORPHOLOGY9 is a key regulator of peroxisome biogenesis and plays critical roles during pollen maturation and germination in planta.
Yang et al., Beijing, China. In Plant Cell, 2014
DAU interacts with peroxisomal membrane proteins PEROXIN13 (PEX13) and PEX16 in planta.
Peroxisome biogenesis in mammalian cells.
Tamura et al., Fukuoka, Japan. In Front Physiol, 2013
In regard to peroxisome-cytoplasmic shuttling of Pex5p, Pex5p initially targets to an 800-kDa docking complex consisting of Pex14p and Pex13p and then translocates to a 500-kDa RING translocation complex.
Functional analysis of PEX13 mutation in a Zellweger syndrome spectrum patient reveals novel homooligomerization of PEX13 and its role in human peroxisome biogenesis.
Gärtner et al., In Hum Mol Genet, 2013
Previously, we identified the W313G mutation located within the SH3 domain of the peroxisomal protein, PEX13.
Pex5p stabilizes Pex14p: a study using a newly isolated pex5 CHO cell mutant, ZPEG101.
Fujiki et al., Fukuoka, Japan. In Biochem J, 2013
Furthermore, we found that Pex5p-Pex13p interaction is essential for the import of PTS1 proteins as well as catalase, but not for that of PTS2 proteins.
Cre-mediated stress affects sirtuin expression levels, peroxisome biogenesis and metabolism, antioxidant and proinflammatory signaling pathways.
Baumgart-Vogt et al., Gießen, Germany. In Plos One, 2011
Our results indicate that Cre-expression itself in Sertoli cells already has led to oxidative stress and lipid peroxidation (4-HNE lysine adducts), inducing PPARα/γ, peroxisome proliferation and alterations of peroxisome biogenesis (PEX5, PEX13 and PEX14) as well as metabolic proteins (ABCD1, ABCD3, MFP1, thiolase B, catalase).
PEX13 deficiency in mouse brain as a model of Zellweger syndrome: abnormal cerebellum formation, reactive gliosis and oxidative stress.
Crane et al., Brisbane, Australia. In Dis Model Mech, 2011
PEX13 deficiency leads to mitochondria-mediated oxidative stress, neuronal cell death and impairment of cerebellar development.
Reducing PEX13 expression ameliorates physiological defects of late-acting peroxin mutants.
Bartel et al., Houston, United States. In Traffic, 2011
pex13-1 allele had deleterious effects when combined with pex5-1 and pex14-2, which are defective in early-acting peroxins, as shown by reduced matrix protein import and enhanced physiological defects
Targeting of Pex8p to the peroxisomal importomer.
Erdmann et al., Bochum, Germany. In Eur J Cell Biol, 2010
Data demonstrate that Pex8p is capable to interact with the PTS2-receptor Pex7p and the docking complex component Pex13p with its C-terminal SH3-domain providing the binding site.
Zellweger syndrome caused by PEX13 deficiency: report of two novel mutations.
Alkuraya et al., Riyadh, Saudi Arabia. In Am J Med Genet A, 2009
Zellweger syndrome caused by PEX13 deficiency: report of two novel mutations.
The peroxin loss-of-function mutation abstinence by mutual consent disrupts male-female gametophyte recognition.
Schroeder et al., San Diego, United States. In Curr Biol, 2008
AMC is strongly and transiently expressed in both male and female gametophytes during fertilization and functions in gametophytes as a peroxin essential for protein import into peroxisomes.
Dynamic architecture of the peroxisomal import receptor Pex5p.
Wilmanns et al., Hamburg, Germany. In Biochim Biophys Acta, 2006
Despite evidence for alpha-helical binding motifs for some of these components (Pex13p, Pex14p) its overall appearance is that of a molten globule and folding/unfolding transitions may play a critical role in its function.
Pex13p: docking or cargo handling protein?
Distel et al., Amsterdam, Netherlands. In Biochim Biophys Acta, 2006
The Src homology 3 (SH3) domain-containing peroxisomal membrane protein Pex13p is an essential component of the import machinery for matrix proteins and forms a binding site for the peroxisomal targeting type I (PTS1) receptor Pex5p.
Peroxisome Biogenesis Disorders, Zellweger Syndrome Spectrum
Moser et al., Seattle, United States. In Unknown Journal, 2004
CLINICAL CHARACTERISTICS: Peroxisome biogenesis disorders, Zellweger syndrome spectrum (PBD, ZSS) is a continuum of three phenotypes — Zellweger syndrome (ZS), the most severe; neonatal adrenoleukodystrophy (NALD); and infantile Refsum disease (IRD), the least severe — that were originally described before the biochemical and molecular bases of these disorders had been fully determined.
Clinical, biochemical and genetic aspects and neuronal migration in peroxisome biogenesis disorders.
Kondo et al., Gifu, Japan. In J Inherit Metab Dis, 2001
PEX13 protein has an SH3 docking site that binds to the PTS-1 receptor.
Pex14p, a peroxisomal membrane protein binding both receptors of the two PTS-dependent import pathways.
Kunau et al., Bochum, Germany. In Cell, 1997
Pex14p also interacts with two other membrane-bound peroxins including Pex13p, another binding protein for the PTS1 receptor.
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