Peroxisomal Biogenesis Factor 1 Protéines (PEX1)

PEX1 encodes a member of the AAA ATPase family, a large group of ATPases associated with diverse cellular activities. De plus, nous expédions PEX1 Anticorps (64) et et beaucoup plus de produits pour cette protéine.

afficher tous les protéines Gène GeneID UniProt
PEX1 5189 O43933
PEX1 71382 Q5BL07
Rat PEX1 PEX1 500006  
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Showing 4 out of 6 products:

Catalogue No. Origin Source Conjugué Images Quantité Livraison Prix Détails
Cellules d'insectes Humain His tag „Crystallography Grade“ protein due to multi-step, protein-specific purification process 1 mg 70 Days
$13,376.85
Détails
Cellules d'insectes Souris His tag „Crystallography Grade“ protein due to multi-step, protein-specific purification process 1 mg 70 Days
$13,376.85
Détails
Escherichia coli (E. coli) Humain Inconjugué SDS-PAGE analysis of Human PEX1 Protein. 100 μg 11 to 18 Days
$605.00
Détails
Escherichia coli (E. coli) Souris Inconjugué SDS-PAGE analysis of Mouse PEX1 Protein. 100 μg 11 to 18 Days
$717.56
Détails

PEX1 Protéines protéines par origine et source

Origin Exprimée danse Conjugué
Human ,
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Mouse (Murine) ,
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Plus protéines pour Peroxisomal Biogenesis Factor 1 (PEX1) partenaires d'interaction

Human Peroxisomal Biogenesis Factor 1 (PEX1) interaction partners

  1. This article reviews the abundant records of missense mutations described in Peroxisome biogenesis disorders patients with the aim to classify and rationalize them by mapping them onto a homology model of the human Pex1/Pex6 complex. [review]

  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. heterozygous mutations in the PEX1 gene in two Chinese newborns with Zellweger syndrome

  4. Major finding is linking peroxisome biogenesis factor 1 (PEX1) to obesity phenotypes, a novel mechanism of peroxisomal biogenesis and metabolism underlying the development of childhood obesity.

  5. A combination of a known missense and novel frameshift variant in PEX1 identified in a family with Heimler syndrome.

  6. 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.

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

  8. Mutations in PEX1 gene is associated with Heimler Syndrome.

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

  10. the variants in PEX genes of a family

  11. A 5' UTR polymorphism at position c.-53 and a promoter polymorphism 137 bp upstream of the PEX1 start codon are identified but strongly differing survival By genotype-phenotype analysis.

  12. Missense mutations in PEX1 cause the milder forms of the peroxisome biogenesis disorders, whereas insertions, deletions, and nonsense mutations are associated with severe clinical phenotypes.

  13. We have evaluated the impact of novel mutations, along with that of the two most common PEX1 mutations, in PBD patients by determining the levels of PEX1 mRNA, PEX1 protein, and peroxisome protein import.

  14. complete lack of PEX1 is associated with Zellweger syndrome

  15. overview of the currently known PEX1 mutations in Zellweger Syndrome [review]

  16. analysis of PEX1 coding mutations and 5' UTR regulatory polymorphisms

  17. Molecular confirmation of the clinical and biochemical diagnosis will allow the prediction of the clinical course of disease in individual PBD cases.

  18. 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.

  19. Studies provide empirical data to estimate the relative fraction of disease-causing alleles that occur in the coding and splice junction sequences of PEX1 gene.

Mouse (Murine) Peroxisomal Biogenesis Factor 1 (PEX1) interaction partners

  1. functions as a protein unfoldase in peroxisomal biogenesis, using its N-terminal putative adaptor-binding domain

  2. crystal structure of pex1

Profil protéine PEX1

Profil protéine

This gene encodes a member of the AAA ATPase family, a large group of ATPases associated with diverse cellular activities. This protein is cytoplasmic but is often anchored to a peroxisomal membrane where it forms a heteromeric complex and plays a role in the import of proteins into peroxisomes and peroxisome biogenesis. Mutations in this gene have been associated with complementation group 1 peroxisomal disorders such as neonatal adrenoleukodystrophy, infantile Refsum disease, and Zellweger syndrome.

Gene names and symbols associated with PEX1

  • peroxisomal biogenesis factor 1 (PEX1)
  • peroxisomal biogenesis factor 1 (Pex1)
  • peroxisomal biogenesis factor 1 L homeolog (pex1.L)
  • 5430414H02Rik Protéine
  • E330005K07Rik Protéine
  • PBD1A Protéine
  • PBD1B Protéine
  • pex1 Protéine
  • RGD1559939 Protéine
  • ZWS Protéine
  • ZWS1 Protéine

Protein level used designations for PEX1

Zellweger syndrome , peroxin-1 , peroxisome biogenesis disorder protein 1 , peroxisome biogenesis factor 1 , peroxisomal biogenesis factor 1 L homeolog

GENE ID SPECIES
5189 Homo sapiens
71382 Mus musculus
500006 Rattus norvegicus
475227 Canis lupus familiaris
505771 Bos taurus
443867 Xenopus laevis
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