SAB5702103
Anti-PEX19 Antibody, clone 9O7Q9, Rabbit Monoclonal
Synonym(s):
PEX19, D1S2223E, HK33, PBD12A, PMP1, PMPI, PXF, PXMP1, peroxisomal biogenesis factor 19
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About This Item
UNSPSC Code:
12352203
NACRES:
NA.46
biological source
rabbit
Quality Level
conjugate
unconjugated
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.
shipped in
wet ice
storage temp.
−20°C
target post-translational modification
unmodified
Gene Information
human ... PEX19(5824)
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
Storage Class Code
10 - Combustible liquids
WGK
WGK 1
Regulatory Information
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