BD-PT0347

Arylsulfatase A Rabbit Polyclonal Antibody

ARSA; Arylsulfatase A; ASA; Cerebroside-sulfatase

常规抗体

ARSA

Arylsulfatase A

WB

Human,Mouse,Rat

1 mg/ml

Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% New type preservative N.

410

http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=410

P15289

http://www.uniprot.org/uniprotkb/P15289/entry

11883

http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=11883

P50428

http://www.uniprot.org/uniprot/P50428

The antiserum was produced against synthesized peptide derived from human ARSA. AA range:251-300

Arylsulfatase A Polyclonal Antibody detects endogenous levels of Arylsulfatase A protein.

WB 1:500 - 1:2000. IHC 1:100 - 1:300. ELISA: 1:20000.. IF 1:50-200

54kD

-20°C/1 year

Polyclonal, Rabbit,IgG

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],

B-cell,Liver,Small intestine,Testis,

Endoplasmic reticulum . Lysosome .

Sphingolipid metabolism;Lysosome;

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 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.,function:Hydrolyzes cerebroside sulfate.,online information:Arylsulfatase A entry,PTM: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).,similarity:Belongs to the sulfatase family.,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).,

现货

The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.