Abcam, ab72617, Anti-SGSH/HSS antibody

Size: 50µg
Mouse Polyclonal SGSH/HSS antibody. Suitable for WB and reacts with Human samples. Cited in 2 publications. Immunogen corresponding to Recombinant Full Length Protein corresponding to Human SGSH.
Key facts
Host species:Mouse,
Clonality:Polyclonal,
Isotype:IgG,
Carrier free:No,
Reacts with:Human,
Applications:WBSee reactivity dataSee the reactivity data table below for information on validated species and application combinations.,
Immunogen:Recombinant Full Length Protein corresponding to Human SGSH.P51688

Properties and Storage Information:
Form-Liquid, Purification technique-Affinity purification Protein A, Storage buffer-pH: 7.4Constituents: PBS, Shipped at conditions-Blue Ice, Appropriate short-term storage conditions-+4°C, Appropriate long-term storage conditions--20°C, Aliquoting information-Upon delivery aliquot, Storage information-Avoid freeze / thaw cycle

Supplementary Information:
This supplementary information is collated from multiple sources and compiled automatically.
The SGSH protein also known as N-sulfoglucosamine sulfohydrolase or HSS is a lysosomal enzyme with a molecular weight of approximately 56 kDa. It is primarily expressed in tissues with high turnover of glycosaminoglycans especially in the liver and brain. This enzyme localizes inside the lysosomes and plays a significant role in the degradation process of heparan sulfate. SGSH expression is important for maintaining normal cell function particularly in tissues that require constant renewal of extracellular matrix components.
Biological function summary
The enzyme acts to cleave sulfate groups from N-sulfated glucosamine residues in heparan sulfate being an important component of the degradation pathway of glycosaminoglycan. SGSH works within a lysosomal degradation complex alongside other enzymes such as iduronate-2-sulfatase and heparan-alpha-glucosaminide N-acetyltransferase each responsible for different steps in the breakdown of complex carbohydrates. Together they facilitate the complete degradation of heparan sulfate into its basic monosaccharide components.
Pathways
SGSH is integral to lysosomal degradation pathways functioning alongside proteins like alpha-L-iduronidase in the catabolic process of heparan sulfate. This breakdown pathway is essential for recycling cellular components and maintaining cellular homeostasis. The efficiencies and deficiencies in these pathways influence cellular functionality and have regulatory roles in broader metabolic circuits.
Mutations in the SGSH gene lead to Sanfilippo syndrome type IIIA also known as mucopolysaccharidosis type IIIA. This genetic disorder impairs the degradation of heparan sulfate causing an accumulation of glycosaminoglycans in cells leading to neurodegenerative conditions. In Sanfilippo syndrome type IIIA the SGSH deficiency has connections with related lysosomal storage disorders involving proteins like alpha-N-acetylglucosaminidase which further illustrate the importance of coordinated lysosomal activities.