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.
The peroxisomal biogenesis disorders are a heterogeneous group with at least 14 complementation groups and with more than 1 phenotype being observed in cases falling into particular 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 Zellweger syndrome (ZWS).
[provided by RefSeq
Applications:Suitable for use in Western Blot.
Other applications not tested.
Recommended Dilution:Optimal dilutions to be determined by the researcher.
AA Sequence:MMouse polyclonal antibody raised against a full-length human PEX19 protein.EEGCSVGAEADRELEELLESALDDFDKAKPSPAPPSTTTAPDASGPQKRSPGDTAKDALFASQEKFFQELFDSELASQATAEFEKAMKELAEEEPHLVEQFQKLSEAAGRVGSDMTSQQEFTSCLKETLSGLAKNATDLQNSSMSEEELTKAMEGLGMDEGDGEGNILPIMQGIMQNLLSKDVLYPSLKEITEKYPEWLQSHRESLPPEQFEKYQEQHSVMCKICEQFEAETPTDSETTQKARFEMVLDLMQQLQDLGHPPKELAGEMPPGLNFDLDAPNLSGPPGASGEQCLIM
Storage and Stability:May be stored at 4℃ for short-term only.
Aliquot to avoid repeated freezing and thawing.
Store at -20℃.
Aliquots are stable for 12 months.
For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
仕様
Size：50ug
抗体種類：Pab
宿主：mouse
Source Antibody：human
Grade：Purified
Purity：Purified
Form：Supplied as a liquid in PBS, pH 7.4.
Specificity：Recognizes human PEX19.
Isotype：IgG
Calc Applications Abbrev：WB
Calc Crossreactivity：Hu
Immunogen：PEX19 (AAH00496.1, 1aa-299aa) full-length human protein.
