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ARSA Protein, Mouse, Recombinant (His)

ARSA Protein, Mouse, Recombinant (His)

产品编号 TMPY-00670
别名: arylsulfatase A, TISP73, AW212749, As-2, AS-A, ASA, As2

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.

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ARSA Protein, Mouse, Recombinant (His) Chemical Structure
ARSA Protein, Mouse, Recombinant (His), CAS N/A
规格 价格/CNY 货期 数量
50 μg ¥ 3,820 5日内发货

ARSA Protein, Mouse, Recombinant (His) 的其他形式现货产品:

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其他形式的 ARSA Protein, Mouse, Recombinant (His):
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产品目录号及名称: ARSA Protein, Mouse, Recombinant (His) (TMPY-00670)
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参考文献
产品描述 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.
别名 arylsulfatase A, TISP73, AW212749, As-2, AS-A, ASA, As2
分子量 53.5 kDa (predicted)

存储

Lyophilized powder: -20~-80°C for 1 year | Solution: -80°C for 6~12 month

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TargetMol Library Books参考文献

1. Laidler PM. (1991) Arylsulfatase A--physico-chemical properties and the use of enzyme radioimmunoassay in medical diagnosis Folia Med Cracov. 32(3-4): 149-68. 2. Jean S,et al.(2006) Ethanol decreases rat hepatic arylsulfatase A activity levels. Alcohol Clin Exp Res. 30(11): 1950-5.

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Keywords

ARSA Protein, Mouse, Recombinant (His) arylsulfatase A AW-212749 TISP73 AW212749 As-2 AS-A ASA TISP 73 AW 212749 As2 TISP-73 Inhibitor inhibitor inhibit

 

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