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Anti-ARSA Antibody (9A5)

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产品编号 TMAY-00845

Anti-ARSA Antibody (9A5) 是一种 Rabbit 抗体,靶向 ARSA。Anti-ARSA Antibody (9A5) 可用于 ELISA。

Anti-ARSA Antibody (9A5)

Anti-ARSA Antibody (9A5)

Rating icon 还可以
产品编号 TMAY-00845

Anti-ARSA Antibody (9A5) 是一种 Rabbit 抗体,靶向 ARSA。Anti-ARSA Antibody (9A5) 可用于 ELISA。

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产品介绍

生物活性
产品描述
Anti-ARSA Antibody (9A5) is a Rabbit antibody targeting ARSA. Anti-ARSA Antibody (9A5) can be used in ELISA.
Ig Type
Monoclonal Rabbit IgG
克隆号
9A5
交叉反应
Human
应用
ELISA
推荐剂量
ELISA: 1:1000-1:2000
抗体种类
Monoclonal
宿主来源Rabbit
构建方式This antibody was obtained from a rabbit immunized with purified, recombinant Human Arylsulfatase A / ARSA (rh Arylsulfatase A / ARSA; TMPY-00669; P15289-1; Met 1-Ala 507).
纯化方式Protein A
性状Liquid
缓冲液0.2 μm filtered solution in PBS
研究背景Arylsulfatase A (ARSA) is synthesized as a 52KDa lysosomal enzyme. It is a member of the sulfatase family that is required for the lysosomal degradation of cerebroside-3-sulfate, a sphingolipid sulfate ester and a major constituent of the myelin sheet. Arylsulfatase A is activated by a required co- or posttranslational modification with the oxidation of cysteine to formylglycine. Metachromatic leukodystrophy (MLD) is a lysosomal storage disease in the central and peripheral nervous systems with severe and progressive neurological symptoms caused by the deficiency of Arylsulfatase A. Deficiency of this enzyme is also found in apparently healthy individuals, a condition for which the term pseudodeficiency is introduced. ARSA forms dimers after receiving three N-linked oligosaccharides in the endoplasmic reticulum, and then the dimers are transported to the Golgi where they receive mannose 6-phosphate recognition markers. And thus, ARSA is transported and delivered to dense lysosomes in a mannose 6-phosphate receptor-dependent manner. It has been shown that within the lysosomes, the ARSA dimers can oligomerize to an octamer in a pH-dependent manner. The ARSA deficiency leads to metachromatic leucodystrophy (MLD), a lysosomal storage disorder associated with severe and progressive demyelination in he central and peripheral nervous system. Additionally, the serum level of arylsulfatase A might be helpful in diagnosis of lung and central nervous system cancer.
偶联与修饰
偶联
Unconjugated
抗原信息
免疫原
Recombinant Human Arylsulfatase A / ARSA protein (TMPY-00669)
抗原种属
Human
存储&运输
储存方式Store at 2°C-8°C for 1 month. Store at -20°C or -80°C for 12 months. Avoid repeated freeze-thaw cycles. Preservative-Free.
运输方式Shipping with blue ice.

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