Optimal conditions should be determined by the investigator
Restrictions
For Research Use only
Format
Lyophilized
Reconstitution
Add 100 µL of distilled water for a final peptide concentration is 1 mg/mL.
Buffer
PBS
Conseil sur la manipulation
Avoid repeated freeze/thaw cycles.
Stock
-20 °C
Stockage commentaire
Store at -20 °C long term.
Antigène
PEX19
(Peroxisomal Biogenesis Factor 19 (PEX19))
Sujet
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.