BackgroundThis 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.
DescriptionPEX19 Monoclonal Antibody. Unconjugated. Raised in: Rabbit.
FormulationBuffer: PBS containing 50% glycerol and 0.05% BSA, preserved with proclin300 or sodium azide, pH 7.3.
SpecificityHuman
IsotypeIgG
Uniprot IDP40855
PurificationAffinity Purified
ImmunogenSynthetic peptide. Please contact EpigenTek for more information.
StorageStore at -20°C. Avoid freeze / thaw cycles.
Alternative NamesPXF; HK33; PMP1; PMPI; PXMP1; PBD12A; D1S2223E; PEX19
ApplicationWB, ELISA; Recommended dilution: WB, 1:500 - 1:1000; ELISA, Recommended starting concentration is 1 µg/mL. Please optimize the concentration based on your specific assay requirements.