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PEX5 Rabbit anti-Human, Mouse, Rat, Polyclonal, Proteintech

Rabbit Polyclonal Antibody

Brand:  Proteintech 12545-1-AP-150UL

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Product Code. 16840304

  • 4652.90 SEK / 150µL

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Description

Description

This antibody recognizes endogenous PEX5, which migrates with an apparent molecular mass of 80 kDa.

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.
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PEX5 Rabbit anti-Human, Mouse, Rat, Polyclonal, Proteintech > 150 μL; Unconjugated

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