SAB5702103

Anti-PEX19 Antibody, clone 9O7Q9, Rabbit Monoclonal

• Sinónimos: PEX19, D1S2223E, HK33, PBD12A, PMP1, PMPI, PXF, PXMP1, peroxisomal biogenesis factor 19
• UNSPSC Code: 12352203
• NACRES: NA.46

Propiedades

• biological source: rabbit
• Quality Level: 100
• material: colorless
• clone: 9O7Q9, monoclonal
• form: liquid
• mol wt: 35 kDa
• species reactivity: rat, human
• concentration: 0.4 mg/mL
• technique(s): western blot: 1:500 - 1:2000
• color: colorless
• isotype: IgG
• immunogen sequence: MAAAEEGCSVGAEADRELEELLESALDDFDKAKPSPAPPSTTTAPDASGPQKRSPGDTAKDALFASQEKFFQELFDSELASQATAEFEKAMKELAEEEPH
• UniProt accession no.: P40855
• shipped in: wet ice
• storage temp.: −20°C
• target post-translational modification: unmodified
• Gene Information: human ... PEX19(5824)

Descripción

General description

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.

Immunogen

A synthetic peptide corresponding to a sequence within amino acids 1-100 of human PEX19 (P40855).

PBS with 0.02% sodium azide,0.05% BSA,50% glycerol,pH7.3.

Application

WB

Información de seguridad

• Storage Class: 10 - Combustible liquids
• wgk_germany: WGK 1