DATASHEET
Host:
Rabbit
Target Protein:
PEX5
Immunogen Range:
51-150/639
Clonality:
Polyclonal
Isotype:
IgG
Entrez Gene:
5830
Swiss Prot:
P50542
Source:
KLH conjugated synthetic peptide derived from human PEX5
Purification:
Purified by Protein A.
Storage Buffer:
Aqueous buffered solution containing 0.01M TBS (pH 7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
Storage:
Store at -20°C. Aliquot into multiple vials to 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. [provided by RefSeq, Oct 2008]
Conjugation:
Cy3
Excitation/ Emission:
512,550nm/570,615nm
Size:
100ul
Concentration:
1ug/ul
Applications:
IF(IHC-P)(1:50-200)
IF(IHC-F)(1:50-200)
IF(ICC)(1:50-200)
71
Mouse
Predicted Cross Reactive Species:
Human
Rat
Dog
Cow
Sheep
Pig
Horse
For research use only. Not intended for diagnostic or therapeutic use.