The product of PEX3 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 PEX3 are a cause Zellweger syndrome (ZWS).
Applications:Suitable for use in Western Blot and ELISA.
Other applications not tested.
Recommended Dilution:Western Blot: 1:500-1:2000ELISA: 1:10,000Optimal dilutions to be determined by the researcher.
Storage and Stability:May be stored at 4°C for short-term only.
Aliquot to avoid repeated freezing and thawing.
Store at -20°C.
Aliquots are stable for 12 months after receipt.
For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
仕様
Size：30ul
Host：rabbit
Source Antibody：human
Grade：Affinity Purified
Purity：Purified by immunoaffinity chromatography.
Form：Supplied as a liquid in PBS, 0.5% BSA, 0.02% sodium azide, 50% glycerol.
Specificity：Recognizes endogenous levels of human Peroxin 3. Species Crossreactivity: mouse and rat
Isotype：IgG
Calc Applications Abbrev：E WB
Calc Crossreactivity：Hu Mo Rt
Immunogen：Synthetic peptide corresponding to aa30-110 of human Peroxin 3 at N-terminal.
