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Peroxin 19 Polyclonal Antibody

说明书

BYab-04070

  • 20UL ¥460 50UL ¥945 100UL ¥1350
  • 货期: 3天左右

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宿主
反应性
应用
WB;IHC;IF;ELISA
分子量(DA)
33kD
免疫原
The antiserum was produced against synthesized peptide derived from human PEX19. AA range:219-268
特异性
Peroxin 19 Polyclonal Antibody detects endogenous levels of Peroxin 19 protein.
来源
Polyclonal, Rabbit,IgG
组成(Formulation)
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
稀释比例
WB: 1/500 - 1/2000. IHC: 1/100 - 1/300. ELISA: 1/5000.. IF 1:50-200
纯化工艺(Immunogen)
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
浓度
1 mg/ml
背景(Background)
peroxisomal biogenesis factor 19(PEX19) Homo sapiens 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
功能
alternative products:Experimental confirmation may be lacking for some isoforms,disease:Defects in PEX19 are a cause of Zellweger syndrome (ZWS) [MIM:214100]. ZWS is a fatal peroxisome biogenesis disorder characterized by dysmorphic facial features, hepatomegaly, ocular abnormalities, renal cysts, hearing impairment, profound psychomotor retardation, severe hypotonia and neonatal seizures. Death occurs within the first year of life.,disease:Defects in PEX19 are the cause of peroxisome biogenesis disorder complementation group 14 (PBD-CG14) [MIM:600279]; also known as PBD-CGJ. PBD refers to a group of peroxisomal disorders arising from a failure of protein import into the peroxisomal membrane or matrix. The PBD group is comprised of four disorders: Zellweger syndrome (ZWS), neonatal adrenoleukodystrophy (NALD), infantile Refsum disease (IRD), and classical rhizomelic chondrodysplasia punc
基因名称(Gene Name)
PEX19
蛋白名称
Peroxisomal biogenesis factor 19
简称
Peroxin 19
其他名称
PEX19; HK33; PXF; OK/SW-cl.22; Peroxisomal biogenesis factor 19; 33 kDa housekeeping protein; Peroxin-19; Peroxisomal farnesylated protein
Fields
>>Peroxisome
人基因ID
5824
人蛋白质序列数据库
P40855
小鼠基因ID
小鼠蛋白质序列数据库
Q8VCI5
大鼠基因ID
大鼠蛋白质序列数据库
细胞定位
Cytoplasm . Peroxisome membrane ; Lipid-anchor ; Cytoplasmic side . Mainly cytoplasmic. Some fraction membrane-associated to the outer surface of peroxisomes. .
组织表达
Ubiquitously expressed. Isoform 1 is strongly predominant in all tissues except in utero where isoform 2 is the main form.
储存(Storage)
-20°C/1 year

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Peroxin 19 Polyclonal Antibody

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