Anticorps Monoclonal Anti - PEX3,(c - terminal) produit en souris, clone 3C2, immunoglobulin purifié, solution aqueuse tamponnée

Non disponible en dehors du Royaume-Uni et de l'Irlande

General description

The product of this gene is involved in peroxisome biosynthesis and integrity. It assembles membrane vesicles before the matrix proteins are translocated. 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 Zellweger syndrome (ZWS). (provided by RefSeq)

Immunogen

PEX3 (NP_003621, 271 a.a. ~ 373 a.a) partial recombinant protein with GST tag. MW of the GST tag alone is 26 KDa.SequenceLESPDFSTVLNTCLNRGFSRLLDNMAEFFRPTEQDLQHGNSMNSLSSVSLPLAKIIPIVNGQIHSVCSETPSHFVQDLLTMEQVKDFAANVYEAFSTPQQLEK

Physical form

Solution in phosphate buffered saline, pH 7.4