This gene is necessary for early peroxisomal biogenesis.
It acts both as a cytosolic chaperone and as an import receptor for peroxisomal membrane proteins (PMPs).
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.
These disorders have at least 14 complementation groups, with more than one phenotype being observed for some 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 Zellweger syndrome (ZWS), as well as peroxisome biogenesis disorder complementation group 14 (PBD-CG14), which is also known as PBD-CGJ.
Alternative splicing results in multiple transcript variants.
Applications:Suitable for use in Western Blot, ELISA
DO NOT FREEZE! Stable at 4°C for 12 months after receipt as an undiluted liquid.
Dilute required amount only prior to immediate use.
Further dilutions can be made in assay buffer.
For maximum recovery of product, centrifuge the original vial prior to removing the cap.
Note: Applications are based on unconjugated antibody.
仕様
Size：200ul
Host：rabbit
Source Antibody：human
Grade：Affinity Purified
Purity：Purified by Protein A affinity chromatography.
Form：Supplied as a liquid in PBS, pH 7.2. No preservative added. Labeled with Alkaline Phophatase (AP).
Specificity：Human
Isotype：IgG
Calc Applications Abbrev：E WB
Calc Crossreactivity：Hu
Immunogen：PEX19 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 32-61 amino acids from the N-terminal region of human PEX19.
