GCDH 抗体 (AA 201-300)
Quick Overview for GCDH 抗体 (AA 201-300) (ABIN1713809)
抗原
See all GCDH 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 201-300
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预测反应
- Human,Mouse,Rat
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纯化方法
- Purified by Protein A.
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免疫原
- KLH conjugated synthetic peptide derived from human GCDH
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亚型
- IgG
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应用备注
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WB 1:300-5000
ELISA 1:500-1000
IHC-P 1:200-400
IHC-F 1:100-500
IF(IHC-P) 1:50-200
IF(IHC-F) 1:50-200
IF(ICC) 1:50-200
ICC 1:100-500 -
限制
- 仅限研究用
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状态
- Liquid
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浓度
- 1 μg/μL
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缓冲液
- 0.01M TBS( pH 7.4) with 1 % BSA, 0.02 % Proclin300 and 50 % Glycerol.
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储存液
- ProClin
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注意事项
- This product contains ProClin: a POISONOUS AND HAZARDOUS SUBSTANCE, which should be handled by trained staff only.
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储存条件
- 4 °C,-20 °C
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储存方法
- Shipped at 4°C. Store at -20°C for one year. Avoid repeated freeze/thaw cycles.
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有效期
- 12 months
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- GCDH (Glutaryl-CoA Dehydrogenase (GCDH))
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别名
- GCDH
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背景
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Synonyms: ACAD5, EC 1.3.99.7, GCD, Gcdh, GCDH_HUMAN, Glutaryl CoA dehydrogenase, mitochondrial, Glutaryl Coenzyme A dehydrogenase, Glutaryl-CoA dehydrogenase, mitochondrial, MS781.
Background: GCDH is a 438 amino acid protein that localizes to the mitochondrial matrix and belongs to the acyl-CoA dehydrogenase family. Existing as a homotetramer, GCDH uses FAD as a cofactor to catalyze the oxidative decarboxylation of glutaryl-CoA to crotonyl-CoA and CO(2) in the degradative pathway of L-lysine, L-hydroxylysine and L-tryptophan metabolism. While GCDH exists as both a long and short isoform, only the long isoform is a functionally active protein. Defects in the gene encoding GCDH are the cause of glutaric acidemia type I (GA-I), an autosomal recessive disorder that is characterized by the accumulation of glutaconic acid and is associated with such symptoms as progressive dystonia and athetosis due to gliosis and neuronal loss in the basal ganglia.
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基因ID
- 2639
抗原
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