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首页>>免疫学>>一抗>>芳基硫酸酯酶A抗体
芳基硫酸酯酶A抗体
  • 产品货号：
    BN40989R
  • 中文名称：
    芳基硫酸酯酶A抗体
  • 英文名称：
    Rabbit anti-ARSA Polyclonal antibody
  • 品牌：
    Biorigin

  • 货号

    产品规格

    售价

    备注

  • BN40989R-100ul

    100ul

    ¥2360.00

    交叉反应：Mouse,Rat(predicted:Human,Dog,Pig,Cow,Horse,Rabbit) 推荐应用：WB,IHC-P,IHC-F,IF,ELISA

  • BN40989R-200ul

    200ul

    ¥3490.00

    交叉反应：Mouse,Rat(predicted:Human,Dog,Pig,Cow,Horse,Rabbit) 推荐应用：WB,IHC-P,IHC-F,IF,ELISA

产品描述

英文名称ARSA
中文名称芳基硫酸酯酶A抗体
别    名As 2; As2; ASA; metachromatic leucodystrophy; TISP73; arylsulfatase A; AS A; MLD.  
研究领域免疫学  神经生物学  
抗体来源Rabbit
克隆类型Polyclonal
交叉反应Mouse, Rat,  (predicted: Human, Dog, Pig, Cow, Horse, Rabbit, )
产品应用WB=1:500-2000 ELISA=1:5000-10000 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.
分 子 量45/54kDa
细胞定位细胞浆 
性    状Liquid
浓    度1mg/ml
免 疫 原KLH conjugated synthetic peptide derived from human ARSA :368-412/507 
亚    型IgG
纯化方法affinity purified by Protein A
储 存 液0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存条件Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
PubMedPubMed
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 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

Entrez Gene: 315222 Rat

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细胞膜上的一种亚砷酸根阴离子泵的水溶性部分。在亚砷酸根存在的情况下。ArsA具有ATP酶活力。它水解ATP,发生构象变化.芳基硫酯酶A（ArylsulfataseA， ARSA）的缺陷，使溶酶体内脑硫酯水解受阻，沉积于中枢神经系统的白质、周围神经系统及其它内脏组织，导致异染性脑白质营养不良（Metachromatic Leukodystrophy，MLD），他是一种较常见的脑白质营养不良，也是一种最常见的溶酶体病，为常染色体隐性遗传。























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