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monoubiquitination of the N-terminal cysteine of peroxisome-associated PEX5 not only functions to recycle the peroxin back to the cytosol, but also serves as a quality control mechanism to eliminate peroxisomes with a defective protein import machinery
Demyelination in cerebellum and brain stem preceded major myelin loss in corpus callosum of Pex5 deficient mice.
interaction of PEX5 with catalase and PEX14
Absence of peroxisomal metabolism in neurons and astrocytes does not provoke the neurodegenerative phenotype observed after deleting peroxisomes from oligodendrocytes. Lack of peroxisomal metabolism in astrocytes causes increased VLCFA levels in myelin
Peroxisomes were selectively reconstituted in brain or liver of Pex5 knock-out mice, a model for Zellweger syndrome, by tissue-selective overexpression of Pex5p. We found that both rescue strains exhibited a correction of the neuronal migration defect
The interactions with the docking proteins were independent of the PEX5 ubiquitination status whereas the interactions with the receptor export machinery (REM) components were increased when PEX5 is ubiquitinated
These data suggest that PEX5 may be a critical regulator of lysosomal gene expression and autophagy through the mTOR-TFEB-autophagy axis under nutrient deprivation.
This study provides evidence suggesting that monoubiquitinated PEX5 interacts directly with both PEX1 and PEX6 through its ubiquitin moiety and that the PEX5 polypeptide chain is globally unfolded during the ATP-dependent extraction event.
Data suggest that P7BP2 is localized to peroxisomes by binding to PEX5 via PEX7 in manner dependent on apparent PTS2 (type 2 peroxisomal targeting signal peptide) in N-terminal region of P7BP2; the PTS2 is subsequently cleaved off in peroxisomes. (P7BP2 = PEX7-binding protein-2; PEX5 = peroxisomal biogenesis factor-5; PEX7 = peroxisomal biogenesis factor-7)
these findings lend credit to the idea that inefficient catalase import, when coupled with the role of PEX5 as a redox-regulated import receptor, constitutes a cellular defense mechanism to combat oxidative insults of extra-peroxisomal origin.
TRIM37-mediated ubiquitylation stabilizes PEX5 and promotes peroxisomal matrix protein import, suggesting that mulibrey nanism is a new peroxisomal biogenesis disorder.
Data suggest that soluble/cytosolic PEX5 interacts with PEX14/PEX13 complex, a model for the docking/translocation module (DTM) of the peroxisomal matrix protein translocon; PEX14/PEX13 complex appears to function in peroxisomal membrane as large cavity into which cytosolic PEX5 can enter to release its cargo. (PEX = peroxisomal biogenesis factor)
data reveal subpopulations of peroxisomes showing only weak colocalization between PEX14 and PEX5 or PEX11 but at the same time a clear compartmentalized organization. This compartmentalization, which was less evident in cases of strong colocalization, indicates dynamic protein reorganization linked to changes occurring in the peroxisomes.
our data suggest that insertion of the trimeric PEX5-PEX7-PTS2 protein complex into the DTM is probably accompanied by conformational alterations in PEX5 to allow release of the PTS2 protein into the organelle matrix
Our data suggest that the functional polymorphism rs3814058C>T in 3'-UTR of PXR may be a functional biomarker to predict risk of colorectal cancer
PEX5 encodes two isoforms, PEX5L & PEX5S, & a homozygous frame shift mutation c.722dupA (p.Val242Glyfs( *)33), located in the PEX5L-specific exon 9, results in loss of PEX5L only. Loss of PEX5L results in deficient import of PTS2-tagged proteins
bulky side chain within the recognition motif, which blocks contraction of the PEX5 binding cavity
Data show that ataxia-telangiectasia mutated (ATM) phosphorylates peroxisomal biogenesis factor 5 (PEX5) at serine 141 in response to reactive oxygen species.
the molecular recognition of PTS1 cargo proteins by Pex5p
ubiquitination of peroxisome-targeting signal type 1 (PTS1) receptor Pex5p regulating PTS1 protein import
PEX5 has a role in regulating peroxisome numbers by signaling to mediate pexophagy
Cys11 in PEX5 serves as a functional redox switch regulating the peroxisomal/cytosolic localization of peroxisomal proteins.
The novel Pex14-binding site may represent the initial tethering site of Pex5 from which the cargo-loaded receptor is further processed in a sequential manner.
Translocation of proteins across the organelle membrane occurs downstream of a reversible docking step and upstream of the first cytosolic ATP-dependent step, i.e. before ubiquitination of PEX5.
The molecular mechanism of recognition by the peroxisomal receptor Pex5p, in complex with alanine-glyoxylate aminotransferase revealed by X-ray crystallography.
The product of this gene binds to the C-terminal PTS1-type tripeptide peroxisomal targeting signal (SKL-type) and plays an essential role in peroxisomal protein import. 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 of neonatal adrenoleukodystrophy (NALD), a cause of Zellweger syndrome (ZWS) as well as may be a cause of infantile Refsum disease (IRD). Alternatively spliced transcript variants encoding different isoforms have been identified.
peroxisome biogenesis factor 5
, PTS1 receptor
, peroxin 5
, peroxisomal C-terminal targeting signal import receptor
, peroxisomal targeting signal 1 receptor
, peroxisome receptor 1
, peroxisomal targeting signal 1 (SKL type) receptor
, peroxisomal targeting signal import receptor
, peroxisomal targeting signal receptor 1
, Peroxisomal C-terminal targeting signal import receptor
, Peroxisome receptor 1