芳香基硫酸酯酶1抗体

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我们提供的抗体用于实验研究，而不用于诊临床断或治疗程序。
芳香基硫酸酯酶1抗体Maintain refrigerated at 2-8°C for up to 6 months. For long term storage store at -20°C in small aliquots to prevent freeze-thaw cycles.
英文名称 Anti-ARSA
中文名称 芳香基硫酸酯酶1抗体
别 名 As 2; As2; ASA; metachromatic leucodystrophy; TISP73; arsA; ARSA_HUMAN; arylsulfatase A; Arylsulfatase A component C; AS A; Cerebroside-sulfatase; MGC125207; MLD.
浓 度 1mg/1ml
规 格 0.2ml/200μg
抗体来源 Rabbit
克隆类型 polyclonal
交叉反应 Human, Mouse, Rat
产品类型 一抗
研究领域 肿瘤 细胞生物 神经生物学 细胞类型标志物 细胞自噬
蛋白分子量 predicted molecular weight: 47/54kDa
性 状 Lyophilized or Liquid
免 疫 原 KLH conjugated synthetic peptide derived from human ARSA
亚 型 IgG
纯化方法 affinity purified by Protein A
储 存 液 Preservative: 15mM Sodium Azide, Constituents: 1% BSA, 0.01M PBS, pH 7.4
产品应用 WB=1:100-500 ELISA=1:500-1000 IP=1:20-100 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:100-500
（石蜡切片需做抗原修复）
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
保存条件 Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
Important Note This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
产品介绍 The protein encoded by this gene hydrolyzes cerebroside sulfate to cerebroside and sulfate. Defects in this gene lead to metachromatic leucodystrophy （MLD）, a progressive demyelination disease which results in a variety of neurological symptoms and ultimay death. Alternatively spliced transcript variants have been described for this gene. [provided by RefSeq, Dec 2010].
Function : Hydrolyzes cerebroside sulfate.
Subcellular Location : Lysosome.
Post-translational modifications : The conversion to 3-oxoalanine （also known as C-formylglycine, FGly）, of a serine or cysteine residue in prokaryotes and of a cysteine residue in eukaryotes, is critical for catalytic activity. This post-translational modification is severely defective in multiple sulfatase deficiency （MSD）.
DISEASE : Defects in ARSA are a cause of leukodystrophy metachromatic （MLD） [MIM:250100]. MLD is a disease due to a lysosomal storage defect. It is characterized by intralysosomal storage of cerebroside-3-sulfate in neural and non-neural tissues, with a diffuse loss of myelin in the central nervous system. Progressive demyelination causes a variety of neurological symptoms, including gait disturbances, ataxias, optical atrophy, dementia, seizures, and spastic tetraparesis. Three forms of the disease can be distinguished according to the age at onset: late-infantile, juvenile and adult.
Arylsulfatase A activity is defective in multiple sulfatase deficiency （MSD） [MIM:272200]. A clinically and biochemically heterogeneous disorder caused by the simultaneous impairment of all sulfatases, due to defective post-translational modification and activation. It combines features of individual sulfatase deficiencies such as metachromatic leukodystrophy, mucopolysaccharidosis, chondrodysplasia punctata, hydrocephalus, ichthyosis, neurologic deterioration and developmental delay. Note=Arylsulfatase A activity is impaired in multiple sulfatase deficiency due to mutations in SUMF1. SUMF1 mutations result in defective post-translational modification of ARSA at residue Cys-69 that is not converted to 3-oxoalanine.
Similarity : Belongs to the sulfatase family.
Database links : UniProtKB/Swiss-Prot: P15289.3
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