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PEX5 Produits

(Peroxisomal Biogenesis Factor 5 (PEX5))

Catégories

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. [provided by RefSeq, Oct 2008].

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Catégories PEX5 en vedette

PEX5 Anticorps

High quality antibodies with extensive validation data.

PEX5 Protéines

Proteins for various applications incl. WB, ELISA, IF etc.

PEX5 recommandé Anticorps

Produit
Reactivity
Application
Validations
N° du produit
Quantité
Fiche technique
Reactivity Human
Application WB, IHC, IF
Validations
  • (5)
N° du produit ABIN6145469
Quantité 100 μL
Fiche technique Fiche technique
Reactivity Human, Mouse, Rat
Application IHC, IF
Validations
  • (4)
N° du produit ABIN7261734
Quantité 200 μL
Fiche technique Fiche technique
Reactivity Dog, Human, Monkey, Rat
Application WB, IF, FACS
Validations
  • (4)
N° du produit ABIN1500156
Quantité 100 μL
Fiche technique Fiche technique

PEX5 recommandé Kits ELISA

Produit
Reactivity
Analytical Method
Validations
N° du produit
Quantité
Fiche technique
Reactivity Human
Analytical Method Quantitative Sandwich ELISA
Validations
N° du produit ABIN6233831
Quantité 96 tests
Fiche technique Fiche technique

PEX5 recommandé Protéines

Produit
Reactivity
Source
Validations
N° du produit
Quantité
Fiche technique
Reactivity Human
Source Escherichia coli (E. coli)
Validations
  • (1)
N° du produit ABIN7124372
Quantité 50 μg
Fiche technique Fiche technique
Reactivity Human
Source Wheat germ
Validations
  • (1)
N° du produit ABIN1314707
Quantité 25 μg
Fiche technique Fiche technique
Reactivity Mouse
Source Tobacco (Nicotiana tabacum)
Validations
N° du produit ABIN3131369
Quantité 1 mg
Fiche technique Fiche technique

Dernières publications sur nos produits PEX5

Styles, Shonsey, Falany, Guidry, Barnes, Falany: "Carboxy-terminal mutations of bile acid CoA:N-acyltransferase alter activity and substrate specificity." dans: Journal of lipid research, Vol. 57, Issue 7, pp. 1133-43, (2016) (PubMed).

Zhang, Kim, Alexander, Cai, Tripathi, Dere, Tee, Tait-Mulder, Di Nardo, Han, Kwiatkowski, Dunlop, Dodd, Folkerth, Faust, Kastan, Sahin, Walker: "A tuberous sclerosis complex signalling node at the peroxisome regulates mTORC1 and autophagy in response to ROS." dans: Nature cell biology, Vol. 15, Issue 10, pp. 1186-96, (2013) (PubMed).

Alencastre, Rodrigues, Grou, Fransen, Sá-Miranda, Azevedo: "Mapping the cargo protein membrane translocation step into the PEX5 cycling pathway." dans: The Journal of biological chemistry, Vol. 284, Issue 40, pp. 27243-51, (2009) (PubMed).

Baumgart, Vanhorebeek, Grabenbauer, Borgers, Declercq, Fahimi, Baes: "Mitochondrial alterations caused by defective peroxisomal biogenesis in a mouse model for Zellweger syndrome (PEX5 knockout mouse)." dans: The American journal of pathology, Vol. 159, Issue 4, pp. 1477-94, (2001) (PubMed).

Chang, Warren, Sacksteder, Gould: "PEX12 interacts with PEX5 and PEX10 and acts downstream of receptor docking in peroxisomal matrix protein import." dans: The Journal of cell biology, Vol. 147, Issue 4, pp. 761-74, (1999) (PubMed).

Schliebs, Saidowsky, Agianian, Dodt, Herberg, Kunau: "Recombinant human peroxisomal targeting signal receptor PEX5. Structural basis for interaction of PEX5 with PEX14." dans: The Journal of biological chemistry, Vol. 274, Issue 9, pp. 5666-73, (1999) (PubMed).

Baes, Gressens, Baumgart, Carmeliet, Casteels, Fransen, Evrard, Fahimi, Declercq, Collen, van Veldhoven, Mannaerts: "A mouse model for Zellweger syndrome." dans: Nature genetics, Vol. 17, Issue 1, pp. 49-57, (1997) (PubMed).

Synonymes et noms alternatifs relatifs à PEX5

peroxisomal biogenesis factor 5 (pex5), peroxisomal biogenesis factor 5 (Pex5), peroxisomal biogenesis factor 5 (PEX5), AW212715, ESTM1, PBD2A, PBD2B, Peroxin-5, PTS1-BP, PTS1R, Pxr1, PXR1, X83306

Protein level used designations for PEX5

  • peroxisome biogenesis factor 5
  • PTS1 receptor
  • PTS1-BP
  • PXR1P
  • peroxin 5
  • peroxisomal C-terminal targeting signal import receptor
  • peroxisomal targeting signal 1 receptor
  • peroxisome receptor 1
  • peroxin-5
  • peroxisomal targeting signal 1 (SKL type) receptor
  • peroxisomal targeting signal import receptor
  • peroxisomal targeting signal receptor 1
  • PTS1R
  • Peroxisomal C-terminal targeting signal import receptor
  • Peroxisome receptor 1
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