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AB96029

Anti-SGSH/HSS抗体

Anti-SGSH/HSS antibody

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(2 Publications)

Rabbit Polyclonal SGSH/HSS antibody. Suitable for WB, IHC-P and reacts with Human samples. Cited in 2 publications. Immunogen corresponding to Recombinant Fragment Protein within Human SGSH aa 300-500.

查看别名

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

2 Images
Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-SGSH/HSS antibody (AB96029)
  • IHC-P

Unknown

Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-SGSH/HSS antibody (AB96029)

Immunohistochemical analysis of formalin fixed paraffin embedded OVCAR3 xenograft, using ab96029 antibody at 1/100 dilution.

Western blot - Anti-SGSH/HSS antibody (AB96029)
  • WB

Unknown

Western blot - Anti-SGSH/HSS antibody (AB96029)

7.5% SDS Page

All lanes:

Western blot - Anti-SGSH/HSS antibody (ab96029) at 1/1000 dilution

All lanes:

Molt-4 whole cell lysate at 30 µg

Predicted band size: 57 kDa

false

关键信息

宿主种属

Rabbit

克隆

Polyclonal

亚型

IgG

不含载体蛋白

No

反应种属

Human

应用

IHC-P, WB

applications

免疫原

Recombinant Fragment Protein within Human SGSH aa 300-500. The exact immunogen used to generate this antibody is proprietary information.

P51688

反应性数据

{ "title": "Reactivity Data", "filters": { "stats": ["", "Species", "Dilution Info", "Notes"], "tabs": { "all-applications": {"fullname" : "All Applications", "shortname": "All Applications"}, "WB" : {"fullname" : "Western blot", "shortname":"WB"}, "IHCP" : {"fullname" : "Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections)", "shortname":"IHC-P"} }, "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>", "IHCP-species-checked": "testedAndGuaranteed", "IHCP-species-dilution-info": "1/100 - 1/500", "IHCP-species-notes": "<p></p>" } } }

性能和储存信息

形式
Liquid
纯化工艺
Affinity purification Immunogen
存储溶液
pH: 7 Preservative: 0.01% Thimerosal (merthiolate) Constituents: 10% Glycerol (glycerin, glycerine), 1.21% Tris, 0.75% Glycine
运输条件
Blue Ice
推荐的短期储存条件
+4°C
推荐的长期储存条件
-20°C
分装信息
Upon delivery aliquot
储存信息
Avoid freeze / thaw cycle

补充信息

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.

产品实验方案

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

靶点信息

Catalyzes a step in lysosomal heparan sulfate degradation.
See full target information SGSH

文献 (2)

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

ICC/IF

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

Molecular pharmaceutics 11:2928-34 PubMed24949884

2014

Insulin receptor antibody-sulfamidase fusion protein penetrates the primate blood-brain barrier and reduces glycosoaminoglycans in Sanfilippo type A cells.

Applications

WB

Species

Unspecified reactive species

Ruben J Boado,Jeff Zhiqiang Lu,Eric Ka-Wai Hui,William M Pardridge
View all publications

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