anti- PEX5 antibody

Host

Rabbit

Uniprot ID

P50542

Form

liquid

Purification

Immunogen affinity purified

Purity

>= 95%

Clonality

polyclonal

Clone ID

Isotype

IgG

Storage

PBS with 0.02% sodium azide and 50% glycerol pH 7.3 , -20℃ for 12 months (Avoid repeated freeze / thaw cycles.)

Background

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.

Immunogen

peroxisomal biogenesis factor 5

Synonyms

FLJ50634, FLJ50721, FLJ51948, Peroxin 5, Peroxisome receptor 1, PEX5, PTS1 BP, PTS1 receptor, PTS1R, PXR1

Observed MW

70 kDa

Reactivity

Human, Mouse, Rat

Tested Application

ELISA, WB, IHC, IF

Recommended dilution

WB: 1:500 - 1:2000; IHC: 1:50 - 1:200; IF: 1:50 - 1:200

Gene ID

5830

Research Area

Metabolism