Recombinant Human ARSB
Cat.No. : | ARSB-26104TH |
Product Overview : | Recombinant fragment of Human ARSB with N-terminal proprietary tag. Predicted MW 36.63 kDa. |
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Description : | Arylsulfatase B encoded by this gene belongs to the sulfatase family. The arylsulfatase B homodimer hydrolyzes sulfate groups of N-Acetyl-D-galactosamine, chondriotin sulfate, and dermatan sulfate. The protein is targetted to the lysozyme. Mucopolysaccharidosis type VI is an autosomal recessive lysosomal storage disorder resulting from a deficiency of arylsulfatase B. Two alternatively spliced transcript variants encoding distinct isoforms have been found for this gene. |
Protein length : | 100 amino acids |
Molecular Weight : | 36.630kDa inclusive of tags |
Source : | Wheat germ |
Form : | Liquid |
Purity : | Proprietary Purification |
Storage buffer : | pH: 8.00Constituents:0.3% Glutathione, 0.79% Tris HCl |
Storage : | Shipped on dry ice. Upon delivery aliquot and store at -80oC. Avoid freeze / thaw cycles. |
Sequences of amino acids : | FGYLLGSEDYYSHERCTLIDALNVT RCALDFRDGEEVATGYKNMYSTNIF TKRAIALITNHPPEKPLFLYLALQS VHEPLQVPEEYLKPYDFIQDKNRHH |
Sequence Similarities : | Belongs to the sulfatase family. |
Gene Name : | ARSB arylsulfatase B [ Homo sapiens ] |
Official Symbol : | ARSB |
Synonyms : | ARSB; arylsulfatase B; |
Gene ID : | 411 |
mRNA Refseq : | NM_000046 |
Protein Refseq : | NP_000037 |
MIM : | 611542 |
Uniprot ID : | P15848 |
Chromosome Location : | 5p11-q13 |
Pathway : | Chondroitin sulfate degradation, organism-specific biosystem; Chondroitin sulfate degradation, conserved biosystem; Dermatan sulfate degradation, organism-specific biosystem; Dermatan sulfate degradation, conserved biosystem; Glycosaminoglycan degradation, organism-specific biosystem; |
Function : | N-acetylgalactosamine-4-sulfatase activity; arylsulfatase activity; catalytic activity; hydrolase activity; metal ion binding; |
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For Research Use Only. Not intended for any clinical use. No products from Creative BioMart may be resold, modified for resale or used to manufacture commercial products without prior written approval from Creative BioMart.
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Q&As (6)
Ask a questionAlthough the primary function of the ARSB protein is related to the breakdown of GAGs, recent studies have suggested its involvement in other biological processes. It has been implicated in cellular signaling, inflammatory responses, and tissue remodeling. Additionally, ARSB mutations or dysregulation have been associated with certain cancers, neurodegenerative disorders, and metabolic disorders.
Yes, mutations in the ARSB gene can cause mucopolysaccharidosis type VI (MPS VI). Over 170 different mutations have been identified, including insertions, deletions, and point mutations, each leading to a deficiency in ARSB enzyme activity.
A deficiency in the ARSB protein leads to a rare inherited disorder called mucopolysaccharidosis type VI (MPS VI). This condition results in the accumulation of GAGs in various tissues and organs, causing various symptoms including skeletal abnormalities, cardiovascular issues, respiratory problems, and impaired vision and hearing.
The expression of the ARSB protein is regulated by various factors, including transcription factors and signaling pathways that control gene expression. Additionally, post-translational modifications such as phosphorylation or glycosylation can affect its activity and localization within the cell.
Yes, there are treatments available for mucopolysaccharidosis type VI. Enzyme replacement therapy (ERT) involves regular infusion of a man-made version of the ARSB enzyme to help break down the accumulated GAGs. Additionally, supportive therapies such as physical therapy, surgery, and medications are utilized to manage symptoms and complications. Gene therapy approaches are also being explored.
Mutations in the ARSB gene lead to a deficiency in the ARSB enzyme, resulting in the accumulation of GAGs within lysosomes. This accumulation is characteristic of lysosomal storage disorders, including mucopolysaccharidosis type VI. These disorders occur due to impaired lysosomal function, which leads to the build-up of undigested substances and subsequent cellular dysfunction.
Customer Reviews (3)
Write a reviewthe ARSB protein has proven to be a valuable asset in protein electron microscopy structure analysis.
By utilizing the ARSB protein, researchers can unravel the intricate three-dimensional architecture of proteins, furthering our understanding of their function and facilitating the development of novel therapeutic interventions.
The ARSB protein's superior performance in ELISA paves the way for robust and precise data analysis, providing invaluable insights into various biological processes.
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