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Anti-PEX5PolyclonalAntibody-一抗-抗體-蛋白與免疫

2023-10-29

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Anti-PEX5 Polyclonal Antibody品牌:solarbio | 貨號:K004208P

英文名稱Anti-PEX5 Polyclonal Antibody
宿主Rabbit
別名PBD2A;PBD2B;PTS1-BP;PTS1R;PXR1;RCDP5
應用WB IHC IF
稀釋比例WB 1:500-2000. IHC 1:50-200. IF 1:50-200.
交叉反應 Human Mouse Rat
蛋白分子量71kDa
Gene ID5830
保存Store at -20°C. Avoid freeze / thaw cycles.
儲存液Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
純化方法Affinity purification
亞型IgG
免疫原Recombinant protein of human PEX5
性狀液體
Public Immunogen RangeRecombinant protein of human PEX5
Subcellular LocationsCytoplasm Peripheral membrane protein Peroxisome membrane
Swiss ProtP50542
克隆類型 Polyclonal Antibody
背景資料The product of this gene binds to the C-terminal PTS1-type tripeptide peroxisomal targeting signal (SKL-type) and plays an essential role in peroxisomal protein import. 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 of neonatal adrenoleukodystrophy (NALD), a cause of Zellweger syndrome (ZWS) as well as may be a cause of infantile Refsum disease (IRD). Alternatively spliced transcript variants encoding different isoforms have been identified.

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