γ-5羧酸合成酶抗体

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γ-5羧酸合成酶抗体

本产品不向个人销售，仅用作科学研究，不用于任何人体实验及非科研性质的动物实验。

产品名称

Anti-ALDH18A1
Anti-aldehyde dehydrogenase 18 family member A1 Antibody

产品介绍

靶标：

ALDH18A1

产品别名：

ADCL3； ARCL3A； GSAS； P5CS； PYCS； SPG9； SPG9A； SPG9B； ALDH18A1； aldehyde dehydrogenase 18 family member A1； aldehyde dehydrogenase 18 family member A1； delta-1-pyrroline-5-carboxylate synthase； Spastic paraplegia-9 (spastic paraparesis with amyotrophy, cataracts and gastroesophageal reflux)； aldehyde dehydrogenase family 18 member A1； delta-1-pyrroline-5-carboxylate synthetase； delta1-pyrroline-5-carboxlate synthetase； pyrroline-5-carboxylate synthetase (glutamate gamma-semialdehyde synthetase)； spastic paraplegia 9 (autosomal dominant)； γ-5羧酸合成酶；

背景信息：

Delta-1-pyrroline-5-carboxylate synthetase (P5CS) is an enzyme that in humans is encoded by the ALDH18A1 gene. This gene is mapped to 10q24.1. This gene is a member of the aldehyde dehydrogenase family and encodes a bifunctional ATP- and NADPH-dependent mitochondrial enzyme with both gamma-glutamyl kinase and gamma-glutamyl phosphate reductase activities. The encoded protein catalyzes the reduction of glutamate to delta1-pyrroline-5-carboxylate,a critical step in the de novo biosynthesis of proline,ornithine and arginine. Mutations in this gene lead to hyperammonemia,hypoornithinemia,hypocitrullinemia,hypoargininemia and hypoprolinemia and may be associated with neurodegeneration,cataracts and connective tissue diseases. Alternatively spliced transcript variants,encoding different isoforms,have been described for this gene.

宿主：Rbt
类型：Pab
同种型:IgG
应用：WB
纯化方式：亲和纯化
偶联物：Unconjugated
性状：液体
存储溶液：参阅说明书
浓度：Batch dependent (Please refer to the vial label for the specific concentration.)
稀释比例: Optimal dilutions/concentrations should be determined by the end user
储存：经常使用则4°C保存。-20°C保存不超过两年。避免反复冻融。
注意事项：仅供实验室使用。不适用于人类或动物的任何临床，治疗或诊断用途。不适合动物或人类食用。