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BN40989R-100ul
100ul
￥2360.00
交叉反應(yīng)：Mouse,Rat(predicted:Human,Dog,Pig,Cow,Horse,Rabbit) 推薦應(yīng)用：WB,IHC-P,IHC-F,IF,ELISA
BN40989R-200ul
200ul
￥3490.00
交叉反應(yīng)：Mouse,Rat(predicted:Human,Dog,Pig,Cow,Horse,Rabbit) 推薦應(yīng)用：WB,IHC-P,IHC-F,IF,ELISA
產(chǎn)品描述
| 英文名稱 | ARSA |
| 中文名稱 | 芳基硫酸酯酶A抗體 |
| 別 名 | As 2; As2; ASA; metachromatic leucodystrophy; TISP73; arylsulfatase A; AS A; MLD. |
| 研究領(lǐng)域 | 免疫學(xué) 神經(jīng)生物學(xué) |
| 抗體來(lái)源 | Rabbit |
| 克隆類型 | Polyclonal |
| 交叉反應(yīng) | Mouse, Rat, (predicted: Human, Dog, Pig, Cow, Horse, Rabbit, ) |
| 產(chǎn)品應(yīng)用 | WB=1:500-2000 ELISA=1:5000-10000 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:100-500 （石蠟切片需做抗原修復(fù)） not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 分 子 量 | 45/54kDa |
| 細(xì)胞定位 | 細(xì)胞漿 |
| 性 狀 | Liquid |
| 濃 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human ARSA :368-412/507 |
| 亞 型 | IgG |
| 純化方法 | affinity purified by Protein A |
| 儲(chǔ) 存 液 | 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. |
| 保存條件 | Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. |
| PubMed | PubMed |
| 產(chǎn)品介紹 | 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 ultimately death. Alternatively spliced transcript variants have been described for this gene. [provided by RefSeq, Dec 2010]. Function: Hydrolyzes cerebroside sulfate. Subunit: Homodimer at neutral pH and homooctamer at acidic pH. Exists both as a single chain of 58 kDa (component A) or as a chain of 50 kDa (component B) linked by disulfide bond(s) to a 7 kDa chain (component C). Interacts with SUMF1. 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. SWISS: P15289 Gene ID: 410 Database links: Entrez Gene: 410 Human Entrez Gene: 11883 Mouse Omim: 607574 Human SwissProt: P15289 Human SwissProt: P50428 Mouse Unigene: 88251 Human Unigene: 620 Mouse Unigene: 23323 Rat Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. ArsA蛋白是存在于微生物細(xì)胞膜上的一種亞砷酸根陰離子泵的水溶性部分。在亞砷酸根存在的情況下。ArsA具有ATP酶活力。它水解ATP,發(fā)生構(gòu)象變化.芳基硫酯酶A（ArylsulfataseA， ARSA）的缺陷，使溶酶體內(nèi)腦硫酯水解受阻，沉積于中樞神經(jīng)系統(tǒng)的白質(zhì)、周圍神經(jīng)系統(tǒng)及其它內(nèi)臟組織，導(dǎo)致異染性腦白質(zhì)營(yíng)養(yǎng)不良（Metachromatic Leukodystrophy，MLD），他是一種較常見的腦白質(zhì)營(yíng)養(yǎng)不良，也是一種最常見的溶酶體病，為常染色體隱性遺傳。 |
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