免疫原
The antiserum was produced against synthesized peptide derived from human ARSA. AA range:251-300
特异性
Arylsulfatase A Polyclonal Antibody detects endogenous levels of Arylsulfatase A protein.
来源
Polyclonal, Rabbit,IgG
组成（Formulation）
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
稀释比例
WB: 1/500 - 1/2000. IHC: 1/100 - 1/300. ELISA: 1/20000.. IF 1:50-200
纯化工艺（Immunogen）
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
背景（Background）
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],
功能
catalytic activity:A cerebroside 3-sulfate + H(2)O = a cerebroside + sulfate.,cofactor:Binds 1 magnesium ion per subunit.,disease:Arylsulfatase A activity is defective in multiple sulfatase deficiency (MSD) [MIM:272200]. MSD is a disorder characterized by decreased activity of all known sulfatases. MSD is due to defects in SUMF1 resulting in the lack of post-translational modification of a highly conserved cysteine into 3-oxoalanine. It combines features of individual sulfatase deficiencies such as metachromatic leukodystrophy, mucopolysaccharidosis, chondrodysplasia punctata, hydrocephalus, ichthyosis, neurologic deterioration and developmental delay.,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 tis
其他名称
ARSA; Arylsulfatase A; ASA; Cerebroside-sulfatase
Fields
>>Sphingolipid metabolism;>>Metabolic pathways;>>Lysosome
细胞定位
Endoplasmic reticulum . Lysosome .
组织表达
B-cell,Liver,Small intestine,Testis,
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