ARSG Human
Greater than 85% as determined by SDS-PAGE.
THE BioTek's products are furnished for LABORATORY RESEARCH USE ONLY. The product may not be used as drugs, agricultural or pesticidal products, food additives or household chemicals.
Description
ARSG is fused to a 23 amino acid His-tag at N-terminus & purified by proprietary chromatographic techniques.
Product Specs
Product Science Overview
ARSG is synthesized as a 63-kDa single-chain precursor protein that localizes to pre-lysosomal compartments and associates with organelle membranes, most likely the endoplasmic reticulum . Proteolytic processing of ARSG results in fragments of 34-, 18-, and 10-kDa, which are found in lysosomal fractions and lose their membrane association . The processing sites and a disulfide bridge between the 18- and 10-kDa chains have been roughly mapped, with cathepsins B and L identified as the proteases involved . Interestingly, proteolytic processing is dispensable for the enzyme’s hydrolytic activity in vitro .
ARSG is differentially expressed, processed, and transported in tissues, involving a membrane-associated pre-lysosomal precursor . The lysosomal transport of ARSG in the liver is independent of mannose 6-phosphate, sortilin, and Limp2 . However, mutation of the glycosylation site N-497 abrogates the transport of ARSG to lysosomes in human fibrosarcoma cells due to impaired mannose 6-phosphate modification .
Deficiency of ARSG leads to a new type of mucopolysaccharidosis, as described in a mouse model . This condition is characterized by the accumulation of heparan sulfate due to the inability to degrade 3-O-sulfated N-sulfoglucosamine residues . Understanding the molecular characteristics and transport mechanisms of ARSG is essential for screening and diagnosing ARSG-deficient mucopolysaccharidosis patients .
