Gene Ontology annotation through association of InterPro records with GO terms
Annotation inferences using phylogenetic trees
Gene Ontology annotation based on UniProtKB/Swiss-Prot keyword mapping
Gene Ontology annotation based on UniProtKB/Swiss-Prot Subcellular Location vocabulary mapping, accompanied by conservative changes to GO terms applied by UniProt
Gene Ontology annotation based on curation of immunofluorescence data
Automatic transfer of experimentally verified manual GO annotation data to orthologs using Ensembl Compara
Electronic Gene Ontology annotations created by ARBA machine learning models
Combined Automated Annotation using Multiple IEA Methods
Phenotype-genotype relationships in peroxisome biogenesis disorders of PEX1-defective complementation group 1 are defined by Pex1p-Pex6p interaction.
Mutations in the peroxin Pex26p responsible for peroxisome biogenesis disorders of complementation group 8 impair its stability, peroxisomal localization, and interaction with the Pex1p x Pex6p complex.
Shuttling mechanism of peroxisome targeting signal type 1 receptor Pex5: ATP-independent import and ATP-dependent export.
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PEX5 import into peroxisomes is ATP-independent
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PEX5 export requires ATP and is dependent on PEX1, PEX6, and PEX26
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PEX5 recycling occurs through multiple rounds
Dynamic and functional assembly of the AAA peroxins, Pex1p and Pex6p, and their membrane receptor Pex26p.
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ATP binding in both D1 and D2 is required for PEX1-PEX6 interaction
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Walker A and B mutagenesis defines functional requirements
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PEX6 and PEX26 are predominantly peroxisomal
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PEX1 exists as homo-oligomer in cytosol and hetero-oligomer on peroxisomes
Properties of the ubiquitin-pex5p thiol ester conjugate.
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Monoubiquitination of PEX5 at Cys-11 is required for ATP-dependent export
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C11K mutant PEX5 is fully functional for import and export
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Soluble Ub-PEX5 retains cargo binding capacity
Recruiting mechanism of the AAA peroxins, Pex1p and Pex6p, to Pex26p on the peroxisomal membrane.
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PEX26 residues 33-40 required for PEX1-PEX6 recruitment to peroxisomes
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PEX6 targeting requires ATP but not ATP hydrolysis
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PEX1 targeting requires ATP hydrolysis
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ATP binding induces conformational changes in PEX1 and PEX6
PEX6 is Expressed in Photoreceptor Cilia and Mutated in Deafblindness with Enamel Dysplasia and Microcephaly.
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PEX6 localizes to cilia of retinal photoreceptor cells
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PEX6 localizes to ameloblasts and odontoblasts in mice
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PEX6 G413V mutation causes deafblindness with enamel dysplasia
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Links peroxisome biogenesis disorders to ciliopathies
Peroxisomal monoubiquitinated PEX5 interacts with the AAA ATPases PEX1 and PEX6 and is unfolded during its dislocation into the cytosol.
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DTM-embedded Ub-PEX5 interacts directly with both PEX1 and PEX6 via ubiquitin moiety
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PEX5 polypeptide chain is globally unfolded during ATP-dependent extraction
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Fusion of stable DHFR domain to PEX5 arrests extraction
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Ub-PEX5 is a bona fide substrate of the PEX1-PEX6 complex
Insights into the Structure and Function of the Pex1/Pex6 AAA-ATPase in Peroxisome Homeostasis.
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PEX1-PEX6 forms heterohexameric AAA-ATPase
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Functions in receptor recycling and prevention of pexophagy
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Unfolds substrates by processive threading through central pore
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PEX1/PEX6 mutations are most common cause of PBDs
The peroxisome biogenesis disorder group 4 gene, PXAAA1, encodes a cytoplasmic ATPase required for stability of the PTS1 receptor.
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PEX6 (PXAAA1) is the CG4 PBD gene
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PEX6 is predominantly cytoplasmic
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Walker A lysine mutation abolishes biological activity
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PEX6 required for PEX5 (PTS1 receptor) stability
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Expression restores peroxisomal protein import in CG4 cells
Human peroxisome assembly factor-2 (PAF-2): a gene responsible for group C peroxisome biogenesis disorder in humans.
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Human PAF-2 (PEX6) cDNA restores peroxisomes in group C Zellweger cells
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Two pathogenic mutations identified
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Gene located on chromosome 6p21.1
A cytoplasmic AAA family peroxin, Pex1p, interacts with Pex6p.
PEX1:PEX6:PEX26:ZFAND6 dissociates Ub:PEX5L and PEX7 from PEX14:PEX13:PEX2:PEX10:PEX12 and translocates PEX5L and PEX7 from the peroxisomal membrane to the cytosol
PEX2:PEX10:PEX12:Ub:PEX5L:PEX7:PEX13:PEX14 binds PEX1:PEX6:PEX26 and ZFAND6
PEX2:PEX10:PEX12:Ub:PEX5S,L:PEX13:PEX14 binds PEX1:PEX6:PEX26 and ZFAND6