PEX1 抗体 (AA 25-1284)
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Quick Overview for PEX1 抗体 (AA 25-1284) (ABIN7872829)
抗原
See all PEX1 抗体适用
宿主
克隆类型
标记
应用范围
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抗原表位
- AA 25-1284
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原理
- Peroxin 1 Antibody / PEX1
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纯化方法
- Immunogen affinity purified
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免疫原
- E.coli-derived mouse PEX1 recombinant protein (Position: R25-A1284) was used as the immunogen for the Peroxin 1 antibody.
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亚型
- IgG
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应用备注
- Optimal dilution of the Peroxin 1 antibody should be determined by the researcher.
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限制
- 仅限研究用
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状态
- Lyophilized
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溶解方式
- Adding 0.2 mL of distilled water will yield a concentration of 500 μg/mL
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缓冲液
- Each vial contains 4 mg Trehalose, 0.9 mg NaCl, 0.2 mg Na2HPO4.
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储存条件
- 4 °C,-20 °C
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储存方法
- After reconstitution, the Peroxin 1 antibody can be stored for up to one month at 4oC. For long-term, aliquot and store at -20oC. Avoid repeated freezing and thawing.
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- PEX1 (Peroxisomal Biogenesis Factor 1 (PEX1))
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别名
- Peroxin 1
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背景
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Peroxin 1 antibody detects Peroxisomal biogenesis factor 1, encoded by the PEX1 gene on chromosome 7q21.2. Peroxin 1 antibody is commonly used in studies of peroxisome biology, metabolism, and genetic disorders. Peroxin 1 is a large AAA ATPase that is essential for peroxisome biogenesis and maintenance. It functions in the recycling of the peroxisomal import receptor PEX5, thereby supporting import of matrix proteins into peroxisomes. This process ensures that enzymes required for beta-oxidation of fatty acids, plasmalogen synthesis, and detoxification of reactive oxygen species are correctly localized.
Structurally, Peroxin 1 is a ~147 kDa protein containing two AAA ATPase domains that hydrolyze ATP to drive conformational changes. These domains form a hexameric ring structure that provides mechanical force for receptor recycling. PEX1 localizes to the peroxisomal membrane, where it cooperates with PEX6 to dislocate PEX5 after cargo delivery. Mutations in PEX1 disrupt this process, leading to defective peroxisome assembly.
Functionally, Peroxin 1 supports peroxisomal protein import, organelle maintenance, and lipid metabolism. It plays a vital role in processing very long-chain fatty acids, bile acid intermediates, and ether phospholipids. Researchers use Peroxin 1 antibody to study peroxisome biogenesis, metabolic regulation, and genetic disease mechanisms.
Clinically, mutations in PEX1 are the most common cause of Zellweger spectrum disorders, a group of peroxisome biogenesis disorders characterized by developmental delay, liver dysfunction, and neurodegeneration. Severity ranges from lethal neonatal disease to milder phenotypes. Understanding PEX1 function is critical for developing therapies for peroxisomal disorders. NSJ Bioreagents supplies Peroxin 1 antibody for research in peroxisome biology and metabolic diseases.
Experimentally, Peroxin 1 antibody is used in western blotting to detect the ~147 kDa protein, in immunofluorescence microscopy to visualize peroxisomal localization, and in immunohistochemistry to study tissue-specific expression. Co-immunoprecipitation with Peroxin 1 antibody helps identify interaction partners, including PEX6 and PEX5. -
UniProt
- Q5BL07
抗原
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