AB96030

Anti-SGSH/HSS antibody

Name: Anti-SGSH/HSS antibody (ab96030) | Abcam
Brand: Abcam Limited
SKU: AB96030
Price: 520 USD
Availability: InStock

Be the first to review this product! Submit a review

(1 Publication)

Rabbit Polyclonal SGSH/HSS antibody. Suitable for WB and reacts with Human samples. Cited in 1 publication. Immunogen corresponding to Recombinant Fragment Protein within Human SGSH aa 1-200.

View Alternative Names

HSS, SGSH, N-sulphoglucosamine sulphohydrolase, Sulfoglucosamine sulfamidase, Sulphamidase

1 Images

Unknown

Western blot - Anti-SGSH/HSS antibody (AB96030)

7.5% SDS Page

All lanes:

Western blot - Anti-SGSH/HSS antibody (ab96030) at 1/1000 dilution

All lanes:

293T whole cell lysate at 30 µg

Predicted band size: 57 kDa

false

Key facts

Host species

Rabbit

Clonality

Polyclonal

Isotype

IgG

Carrier free

Reacts with

Human

Applications

applications

Immunogen

Recombinant Fragment Protein within Human SGSH aa 1-200. The exact immunogen used to generate this antibody is proprietary information.

P51688

style

left-column

Reactivity data

{ "title": "Reactivity Data", "filters": { "stats": ["", "Species", "Dilution Info", "Notes"], "tabs": { "all-applications": {"fullname" : "All Applications", "shortname": "All Applications"}, "WB" : {"fullname" : "Western blot", "shortname":"WB"} }, "product-promise": { "all": "all", "testedAndGuaranteed": "tested", "guaranteed": "expected", "predicted": "predicted", "notRecommended": "not-recommended" } }, "values": { "Human": { "WB-species-checked": "testedAndGuaranteed", "WB-species-dilution-info": "1/500 - 1/3000", "WB-species-notes": "<p></p>" }, "Mouse": { "WB-species-checked": "predicted", "WB-species-dilution-info": "", "WB-species-notes": "" }, "Dog": { "WB-species-checked": "predicted", "WB-species-dilution-info": "", "WB-species-notes": "" } } }

style

left-column

style

left-column

Properties and storage information

Form

Liquid

Purification technique

Affinity purification Protein A

Storage buffer

pH: 7 Preservative: 0.01% Thimerosal (merthiolate) Constituents: PBS, 40% Glycerol (glycerin, glycerine)

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

style

left-column

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.

style

left-column

style

left-column

Product protocols

For this product, it's our understanding that no specific protocols are required. You can visit:

Visit the General protocols
Visit the Troubleshooting

style

left-column

Target data

Catalyzes a step in lysosomal heparan sulfate degradation.

See full target information SGSH

style

left-column

Publications (1)

Recent publications for all applications. Explore the full list and refine your search

Molecular therapy : the journal of the American So 23:638-47 PubMed25592334

2015

Broad functional correction of molecular impairments by systemic delivery of scAAVrh74-hSGSH gene delivery in MPS IIIA mice.

Applications

Unspecified application

Species

Unspecified reactive species

F Jason Duncan,Bartholomew J Naughton,Kimberly Zaraspe,Darren A Murrey,Aaron S Meadows,Kelly Reed Clark,David E Newsom,Peter White,Haiyan Fu,Douglas M McCarty

View all publications

style

left-column

style

left-column

style

right-column