SAB5702103

Anti-PEX19 Antibody, clone 9O7Q9, Rabbit Monoclonal

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

Propiedades

• origen biológico: rabbit
• Nivel de calidad: 100
• Materiales: colorless
• clon: 9O7Q9, monoclonal
• formulario: liquid
• mol peso: 35 kDa
• reactividad de especies: rat, human
• concentración: 0.4 mg/mL
• técnicas: western blot: 1:500 - 1:2000
• color: colorless
• isotipo: IgG
• secuencia del inmunógeno: MAAAEEGCSVGAEADRELEELLESALDDFDKAKPSPAPPSTTTAPDASGPQKRSPGDTAKDALFASQEKFFQELFDSELASQATAEFEKAMKELAEEEPH
• Nº de acceso UniProt: P40855
• Condiciones de envío: wet ice
• temp. de almacenamiento: −20°C
• modificación del objetivo postraduccional: unmodified
• Información sobre el gen: human ... PEX19(5824)

Descripción

Descripción general

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.

Inmunógeno

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.

Aplicación

WB

Información de seguridad

• Código de clase de almacenamiento: 10 - Combustible liquids
• Clase de riesgo para el agua (WGK): WGK 1