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.

Clonality:

polyclonal

Classification:

Primary antibody

Alternative names:

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

Specificity:

Human, Mouse, Rat

Host:

Rabbit

Isotype:

IgG

Immunogen:

peroxisomal biogenesis factor 5

Purification:

Immunogen affinity purified

Purity:

≥95% as determined by SDS-PAGE

Calculated molecular weight:

70 kDa

Form:

liquid

Applications:

ELISA, WB, IHC, IF

Recommended dilution:

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

Storage:

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

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