Catalogue Number: BS-12624R-CY3-BSS
| Manufacturer: | Bioss Inc |
| Type: | Polyclonal Primary Antibody - Conjugated |
| Shipping Condition: | Blue Ice |
| Unit(s): | 100 ul |
| Host name: | Rabbit |
| Clone: | |
| Isotype: | IgG |
| Immunogen: | KLH conjugated synthetic peptide derived from human PEX5 |
| Application: | ICC, IF, IHC-P, IHC-Fr |
Description: 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]
PEX5
P50542
5830
Protein A purified
1 ug/ul
Polyclonal
Unmodified
FLJ50634; FLJ50721; FLJ51948; Peroxin 5; Peroxin-5; Peroxisomal biogenesis factor 5; Peroxisomal C terminal targeting signal import receptor; Peroxisomal C-terminal targeting signal import receptor; Peroxisomal targeting signal 1 receptor; Peroxisome receptor 1; pex5; PEX5_HUMAN; PTS1 BP; PTS1 receptor; PTS1-BP; PTS1R; PXR1.
Store at -20°C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.
512,550nm/570,615nm