PBS with 0.02 % sodium azide and 50 % glycerol pH 7.3 ,
储存液
Sodium azide
注意事项
This product contains Sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
储存条件
-20 °C
储存方法
-20°C for 12 months (Avoid repeated freeze / thaw cycles.)
有效期
12 months
抗原
PEX19
(Peroxisomal Biogenesis Factor 19 (PEX19))
别名
PEX19
背景
Synonyms:33 kDa housekeeping protein, D1S2223E, HK33, Peroxin 19, PEX19, PMP1, PMPI, PXF, PXMP1 Background: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.