Arylsulfatase B, also known as ARSB, ASB, MPS6 or G4S, is a 533 amino acid lysosomal protein that belongs to the sulfatase family. Existing as both a monomer and a homodimer, Arylsulfatase B uses calcium as a cofactor to hydrolyze C4-sulfate groups of N-Acetyl-D-galactosamine, dermatan sulfate and chondriotin sulfate, thereby playing a role in lysosomal degradation. Defects in the gene encoding Arylsulfatase B are the cause of mucopolysaccharidosis type 6 (MPS6) and multiple sulfatase deficiency (MSD), the first of which is a lysosomal storage disease that is characterized by short stature, stiff joints, skeletal malformations, corneal clouding, hepatosplenomegaly and cardiac abnormalities. In contrast, MSD is characterized by a decreased activity of all known sulfatases and is usually associated with mucopolysaccharidosis, chondrodysplasia punctata, hydrocephalus, ichthyosis and neurologic deterioration. Multiple isoforms of Arylsulfatase B exist due to alternative splicing events.
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen and the purity is > 101% (by SDS-PAGE).
Formulierung:
Rabbit IgG, 1mg/ml in PBS with 0.02% sodium azide, 50% glycerol, pH7.2
Application Verdünnung:
WB: 1:500~1:1000 IHC: 1:50~1:206
Anwendungsbeschreibung:
ARSB polyclonal antibody detects endogenous levels of ARSB protein.
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