Peroxisomal Biogenesis Factor 6 Proteine (PEX6)

PEX6 encodes a member of the AAA (ATPases associated with diverse cellular activities) family of ATPases. Zusätzlich bieten wir Ihnen PEX6 Antikörper (97) und viele weitere Produktgruppen zu diesem Protein an.

alle Proteine anzeigen Gen GeneID UniProt
PEX6 5190 Q13608
PEX6 224824 Q99LC9
Ratte PEX6 PEX6 117265 P54777
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Katalog Nr. Origin Quelle Konjugat Bilder Menge Lieferzeit Preis Details
Insektenzellen Human His tag „Crystallography Grade“ protein due to multi-step, protein-specific purification process 1 mg 70 Days
$13,741.67
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Insektenzellen Maus His tag „Crystallography Grade“ protein due to multi-step, protein-specific purification process 1 mg 70 Days
$13,741.67
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Wheat germ Human GST tag 10 μg 11 bis 12 Tage
$414.29
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HEK-293 Cells Human Myc-DYKDDDDK Tag Validation with Western Blot 20 μg 11 Days
$932.80
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PEX6 Proteine nach Spezies und Herkunft

Origin Exprimiert in Konjugat
Human , ,
, ,
Mouse (Murine)

Weitere Proteine zu Peroxisomal Biogenesis Factor 6 (PEX6) Interaktionspartnern

Human Peroxisomal Biogenesis Factor 6 (PEX6) Interaktionspartner

  1. Mutation in PAX6 gene is associated with Ophthalmic manifestations of Heimler syndrome.

  2. 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.

  3. A novel homozygous PEX6 p.Ala94Pro mutation.

  4. Overexpression models confirmed that the overrepresentation of the pathogenic PEX6 c.2578T variant compared to wild-type PEX6 c.2578C results in a peroxisome biogenesis defect and thus constitutes the cause of disease in the affected individuals.

  5. PEX6 identified as the 6p21 SCABD gene in a family with spinocerebellar ataxia with blindness and deafness.

  6. Heimler syndrome is due to four novel and two known missense variants and an 8 bp deletion in PEX6 in five families.

  7. the current study revealed novel expression quantitative trait loci (eQTLs) for SNHG5 and PEX6 genes in chromosome 6. Nucleotide substitutions of the eQTLs might be candidate factors for a variety of cancers by regulating expression of the 2 genes.

  8. As standard biochemical screening of blood for evidence of a peroxisomal disorder did not provide a diagnosis in the individuals with Heimler syndrome, patients with sensorineural hearing lossand retinal pigmentation should have mutation analysis of PEX1 and PEX6 genes.

  9. PEX6 is expressed in photoreceptor cilia and mutated in deafblindness with enamel dysplasia and microcephaly.

  10. Our structural data suggest that the tilting of a central segment of a Pex1-Pex6 pair is responsible for polypeptide movement.

  11. Mutations in PEX6 gene is associated with Heimler Syndrome.

  12. results suggested that peroxisome biogenesis requires Pex1p- and Pex6p-regulated dissociation of Pex14p from Pex26p

  13. increased incidence of Zellweger syndrome in French-Canadians of Lac-St-Jean region caused by a PEX6 founder mutation

  14. hybrid exercise increases expression of eukaryotic translation initiation factor 5A (EIFSA), peroxisomal biogenesis factor 6 (PEX6) and histone cluster 1 H4 (HIST1H4), compared with electrical stimulation alone

  15. We identified a total of 77 different mutations in Zellweger syndrome patients of which 47 mutations have not been reported previously, and 14 polymorphic variants.

  16. A PEX6-defective peroxisomal biogenesis disorder with severe phenotype in an infant, versus mild phenotype resembling Usher syndrome in the affected parents

  17. Insufficient binding to Pex1p x Pex6p complexes, or mislocalization of patient-derived Pex26p mutants is most likely responsible for the complementation group impaired peroxisome biogenesis.

  18. the relative fraction of disease-causing alleles that occur in the coding and splice junction sequences of PEX6 gene.

  19. Leigh syndrome presenting the T8993G mutation in the ATPase 6 gene with variable heteroplasmic loads (44-98%) in a single Tunisian family is a novel finding.

PEX6 Protein Überblick

Protein Überblick

This gene encodes a member of the AAA (ATPases associated with diverse cellular activities) family of ATPases. This member is a predominantly cytoplasmic protein, which plays a direct role in peroxisomal protein import and is required for PTS1 (peroxisomal targeting signal 1, a C-terminal tripeptide of the sequence ser-lys-leu) receptor activity. Mutations in this gene cause peroxisome biogenesis disorders of complementation group 4 and complementation group 6.

Genbezeichner und Symbole assoziert mit PEX6

  • peroxisomal biogenesis factor 6 (PEX6)
  • peroxin 6 (pex6)
  • peroxisomal biogenesis factor 6 (ATEG_01688)
  • peroxisomal biogenesis factor 6 (AOR_1_2608174)
  • peroxisomal biogenesis factor 6 (PTRG_08772)
  • peroxisomal biogenesis factor 6 (UREG_01405)
  • peroxisomal biogenesis factor 6 (BDBG_00087)
  • hypothetical protein (PAAG_11349)
  • peroxisomal biogenesis factor 6 (MCYG_07567)
  • peroxisomal biogenesis factor 6 (VDBG_05952)
  • peroxisomal biogenesis factor 6 (VDBG_05953)
  • peroxisomal biogenesis factor 6 (MGYG_05171)
  • peroxisomal biogenesis factor 6 (TERG_02064)
  • peroxisomal biogenesis factor 6 (Pex6)
  • AI132582 Protein
  • AO090005001500 Protein
  • D130055I09Rik Protein
  • mKIAA4177 Protein
  • NCU08373.1 Protein
  • Paf-2 Protein
  • Paf2 Protein
  • PBD4A Protein
  • PDB4B Protein
  • PXAAA1 Protein

Bezeichner auf Proteinebene für PEX6

peroxisome assembly factor 2 , peroxisomal biogenesis factor 6 , peroxisome biogenesis factor 6 , peroxin-6 , peroxisomal AAA-type ATPase 1 , peroxisomal-type ATPase 1 , PAF-2

GENE ID SPEZIES
534944 Bos taurus
3881433 Neurospora crassa OR74A
4315992 Aspergillus terreus NIH2624
5990363 Aspergillus oryzae RIB40
6347058 Pyrenophora tritici-repentis Pt-1C-BFP
8442734 Uncinocarpus reesii 1704
8508107 Ajellomyces dermatitidis SLH14081
9099673 Paracoccidioides sp. 'lutzii' Pb01
9225183 Arthroderma otae CBS 113480
9536309 Verticillium alfalfae VaMs.102
9536310 Verticillium alfalfae VaMs.102
10027852 Arthroderma gypseum CBS 118893
10378721 Trichophyton rubrum CBS 118892
100126288 Sus scrofa
5190 Homo sapiens
224824 Mus musculus
117265 Rattus norvegicus
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