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SGSH

  • Official Full Name

    N-sulfoglucosamine sulfohydrolase

  • Overview

    This gene encodes one of several enzymes involved in the lysosomal degradation of heparan sulfate. Mutations in this gene are associated with Sanfilippo syndrome A, one type of the lysosomal storage disease mucopolysaccaridosis III, which results from impaired degradation of heparan sulfate. Transcripts of varying sizes have been reported but their biological validity has not been determined.
  • Synonyms

    SGSH; N-sulfoglucosamine sulfohydrolase; N-sulphoglucosamine sulphohydrolase; HSS; MPS3A; mucopolysaccharidosis type IIIA; SFMD; sulfamidase; Heparan sulfate sulfatase; Heparan sulphate sulphatase; MPS 3A; MPS3 A; N sulfoglucosamine sulfohydrolase (sulfamidase); SPHM_HUMAN; Sulfoglucosamine sulfamidase; Sulphamidase; Sulphoglucosamine sulphamidase;

  • Recombinant Proteins
  • Cell & Tissue Lysates
  • Protein Pre-coupled Magnetic Beads
  • Human
  • Mouse
  • Zebrafish
  • E.coli
  • HEK293
  • HEK293T
  • In Vitro Cell Free System
  • Insect Cell
  • Mammalian Cell
  • Mammalian cells
  • Sf21 Insect Cell
  • Wheat Germ
  • C
  • His
  • Flag
  • GST
  • His (Fc)
  • Avi
  • His|GST
  • Myc
  • DDK
  • Myc|DDK
  • N/A
  • N
Species Cat.# Product name Source (Host) Tag Protein Length Price
Human SGSH-22H Active Recombinant Human SGSH protein, His-tagged Insect Cell His
Human SGSH-279H Recombinant Human SGSH, MYC/DDK-tagged HEK293 Myc/DDK
Human SGSH-30971TH Recombinant Human SGSH Wheat Germ N/A 502 amino acids
Human SGSH-195H Recombinant Human SGSH protein, His-tagged HEK293 His
Human SGSH-2634H Recombinant Human SGSH, His-tagged E.coli His 152-502aa
Human SGSH-280H Recombinant Human SGSH, GST-tagged Wheat Germ GST
Human SGSH-1884HCL Recombinant Human SGSH 293 Cell Lysate HEK293 N/A
Human SGSH-473HF Recombinant Full Length Human SGSH Protein In Vitro Cell Free System 502 amino acids
Human SGSH-301590H Recombinant Human SGSH protein, GST-tagged E.coli GST Ile152-Leu502
Human SGSH-695H Recombinant Human SGSH Protein, His/GST-tagged E.coli His/GST Arg21~Asn389
Human SGSH-1998H-B Recombinant Human SGSH Protein Pre-coupled Magnetic Beads HEK293
Human SGSH-965HFL Recombinant Full Length Human SGSH Protein, C-Flag-tagged Mammalian cells Flag
Human SGSH-474HF Recombinant Full Length Human SGSH Protein, GST-tagged In Vitro Cell Free System GST 502 amino acids
Human SGSH-6275H Recombinant Human SGSH Protein (Arg21-Leu502), C-His tagged Mammalian cells C-His Arg21-Leu502
Human SGSH-6276H Recombinant Human SGSH Protein (Arg21-Asn389), N-GST tagged E.coli N-GST Arg21-Asn389
Human SGSH-1998H Recombinant Human SGSH Protein, His (Fc)-Avi-tagged HEK293 His (Fc)-Avi
Mouse Sgsh-713M Active Recombinant Mouse Sgsh Protein, His-tagged Sf21 Insect Cell His Arg23-Leu502
Mouse Sgsh-5829M Recombinant Mouse Sgsh Protein, Myc/DDK-tagged HEK293T Myc/DDK
Zebrafish SGSH-6660Z Recombinant Zebrafish SGSH Mammalian Cell His
  • Background
  • Quality Guarantee
  • Case Study
  • Involved Pathway
  • Protein Function
  • Interacting Protein
  • SGSH Related Articles
SGSH-7.jpg

Fig1. Mapping of SGSH primary and secondary structures.

What is SGSH protein?

SGSH (N-sulfoglucosamine sulfohydrolase) gene is a protein coding gene which situated on the long arm of chromosome 17 at locus 17q25. Also known as N-sulfoglucosamine sulfohydrolase and heparan N-sulfatase, Sulfamidase/SGSH is an important member of the sulfatase family involved in the degradation of heparan sulfate (HS). Different from the HS specific endosulfatases that remove sulfate from internal GlcNAc residues, SGSH removes sulfate group from the non-reducing end glucosamine residues on HS. The SGSH protein is consisted of 502 amino acids and its molecular mass is approximately 56.7 kDa.

What is the function of SGSH protein?

Sulfated polysaccharides are a kind of important biomacromolecules, which exist widely in extracellular matrix, connective tissue and cartilage. Its degradation and synthesis are regulated by many enzymes. SGSH, as one of the key enzymes in the degradation of sulfated polysaccharides, plays an important role in maintaining the homeostasis of extracellular matrix. SGSH is an inflammation-related enzyme, and changes in its activity may affect the metabolism of extracellular matrix, thus participating in the occurrence and development of inflammatory response.

SGSH Related Signaling Pathway

SGSH has important physiological functions in cells, especially in maintaining intracellular glutathione (GSH) levels. Although it is not clear which signaling pathway SGSH is directly involved in, it may indirectly affect other signaling pathways by regulating GSH levels. Including: GSH/ REDOX signaling pathway, NF-κB signaling pathway, apoptosis signaling pathway, iron death signaling pathway and so on.

SGSH Related Diseases

The SGSH deficiency results in mucopolysaccharidosis type IIIA (MPS IIIA, Sanfilippo A syndrome), an autosomal recessive lysosomal storage disease characterized by neurological dysfunction but relatively mild somatic manifestations. Due to the absence or abnormal function of SGSH in adrenal cortical cells, NSG cannot be metabolized properly, resulting in congenital adrenal hyperplasia (CAH). A deficiency or abnormal function of SGSH, which results in the deposition of gangliosides in the nervous system, is ganglioside deposition disorder (GM1 ganglioside deposition disorder). Abnormal SGSH function is also associated with cirrhosis.

Bioapplications of SGSH

SGSH is widely used in drug development, especially in the development of antibiotics and antiviral drugs. SGSH can catalyze the metabolism of antibiotics and antiviral drugs, thus affecting their efficacy and toxicity. SGSH plays an important role in the treatment of mucoglycans storage disease. SGSH enzyme replacement therapy can degrade accumulated GAGs by supplementing exogenous SGSH enzymes to patients, alleviating symptoms and improving quality of life.

Case study 1: Julie Weidner, 2019

COPD is associated with gene polymorphisms in SUMF1, a master regulator of sulfatases. Sulfatases are involved in extracellular matrix remodeling and activated by SUMF1, but their role in the lung is poorly described. This article aimed to examine how sulfatases are affected in the airways of patients with COPD compared to ever smokers and never smokers. Examinations of sulfatases, including GALNS, IDS, and SGSH by mRNA, protein expression, sulfatase activity levels, and localization of have been completed. Additionally, immunohistochemistry on lung biopsies was used to test the expression of sulfatases in COPD patients. The findings showed IDS, ARSB, GNS and SGSH in fibroblasts were localized to sites other than their reported destination and the mRNA expression of them increased. This could contribute to the understanding of the disease mechanism in COPD and in the long run, to lead to more individualized therapies.

SGSH-3.jpg

Fig1. In some, but not all, COPD patients we also observed an increase of SGSH activity. The activity of SGSH was examined in cultured lung fibroblasts from COPD patients.

SGSH-4.jpg

Fig2. SGSH appeared to partially co-localize to the lysosomes in ever smoker and COPD fibroblasts.

Case study 2: Ruben J Boado, 2018

Mucopolysaccharidosis Type IIIA (MPSIIIA), also known as Sanfilippo A syndrome, is an inherited neurodegenerative disease caused by mutations in the lysosomal enzyme, N-sulfoglucosamine sulfohydrolase (SGSH). Treatment of MPSIIIA with intravenous recombinant SGSH is not possible because this large molecule does not cross the blood–brain barrier (BBB). BBB penetration by SGSH was enabled in the present study by re-engineering this enzyme as an IgG-SGSH fusion protein, where the IgG domain is a chimeric monoclonal antibody (mAb) against the mouse transferrin receptor (TfR), designated the cTfRMAb. In conclusion, substantial reductions in brain pathologic GAGs in a murine model of MPSIIIA are produced by chronic systemic administration of an IgG-SGSH fusion protein engineered to penetrate the BBB via receptor-mediated transport.

SGSH-5.jpg

Fig3. Western blot with a primary antibody against either mouse IgG (left panel) or human SGSH (right panel).

SGSH-6.jpg

Fig4. ELISA shows the binding of the cTfRMAb-SGSH fusion protein, or the mouse IgG1k control antibody, to the mouse TfR extracellular domain.

SGSH involved in several pathways and played different roles in them. We selected most pathways SGSH participated on our site, such as Glycosaminoglycan degradation, Metabolic pathways, Lysosome, which may be useful for your reference. Also, other proteins which involved in the same pathway with SGSH were listed below. Creative BioMart supplied nearly all the proteins listed, you can search them on our site.

Pathway Name Pathway Related Protein
Glycosaminoglycan degradationIDS;GALNS;HYAL2;HPSE2;HPSE;IDUA;ARSB;GUSB;HYAL1
Metabolic pathwaysLASS5;GAD1;PGM1;SDHA;B3GNTL2;ACOT2;AOX2P;GNPDA1;G6PC
LysosomeSGSH;FUCA1;CTSG;CTSLA;ENTPD4;ASAH1B;HYAL1;IDS;CTSM

SGSH has several biochemical functions, for example, N-sulfoglucosamine sulfohydrolase activity, catalytic activity, metal ion binding. Some of the functions are cooperated with other proteins, some of the functions could acted by SGSH itself. We selected most functions SGSH had, and list some proteins which have the same functions with SGSH. You can find most of the proteins on our site.

Function Related Protein
N-sulfoglucosamine sulfohydrolase activity
catalytic activityPNP5A;ACLYA;GLA;GLULB;ACCSL;PCYT1AB;HSD3B6;DIP2BA;MOXD1L
metal ion bindingCYP2AD3;MMP1A;ZFP574;EXO1;BTK;DPF2L;GNAI3;ASPA;ZNF436
sulfuric ester hydrolase activitySULF2B;ARSA;SGSH;GNSA;GALNS;GNSB;STS;GNS;SULF2A

SGSH has direct interactions with proteins and molecules. Those interactions were detected by several methods such as yeast two hybrid, co-IP, pull-down and so on. We selected proteins and molecules interacted with SGSH here. Most of them are supplied by our site. Hope this information will be useful for your research of SGSH.

cona_canen

Boado, RJ; Lu, JZ; et al. Insulin Receptor Antibody-Sulfamidase Fusion Protein Penetrates the Primate Blood-Brain Barrier and Reduces Glycosoaminoglycans in Sanfilippo Type A Cells. MOLECULAR PHARMACEUTICS 11:2928-2934(2014).
Hemsley, KM; Norman, EJ; et al. Effect of cisternal sulfamidase delivery in MPS IIIA Huntaway dogs-A proof of principle study. MOLECULAR GENETICS AND METABOLISM 98:383-392(2009).
Hemsley, KM; Beard, H; et al. Effect of high dose, repeated intra-cerebrospinal fluid injection of sulphamidase on neuropathology in mucopolysaccharidosis type IIIA mice. GENES BRAIN AND BEHAVIOR 7:740-753(2008).
  • Q&As
  • Reviews

Q&As (7)

Ask a question
What are the challenges and limitations in developing effective treatments for lysosomal storage disorders caused by SGSH deficiency? 08/19/2022

Challenges include the need for efficient delivery of therapies to affected tissues and overcoming the blood-brain barrier to treat neurological manifestations. The heterogeneous nature of lysosomal storage disorders and individual variations in disease progression pose additional challenges. Limited understanding of disease mechanisms and lack of validated biomarkers also hinder the development of targeted treatments.

What future research directions hold promise for advancing our understanding and treatment of lysosomal storage disorders connected to SGSH deficiency? 06/08/2022

Future research could focus on elucidating the molecular mechanisms underlying SGSH deficiency and lysosomal dysfunction, exploring novel therapeutic targets, and improving delivery methods for effective treatment. Advances in gene editing technologies, such as CRISPR/Cas9, may also offer potential avenues for correcting genetic mutations leading to SGSH deficiency, thus providing a curative approach to lysosomal storage disorders.

What are the key cellular and physiological consequences of SGSH deficiency in lysosomal storage disorders? 09/05/2020

SGSH deficiency impacts numerous cellular pathways and physiological systems. The accumulation of undegraded heparan sulfate disrupts intracellular trafficking, impairs lysosomal function, and triggers inflammation. These cascading effects lead to cognitive decline, neurological manifestations, skeletal abnormalities, connective tissue damage, and organ dysfunction observed in lysosomal storage disorders associated with SGSH deficiency.

What are the current therapeutic approaches targeting SGSH deficiency in lysosomal storage disorders? 12/10/2019

Therapeutic strategies aim to address SGSH deficiency by introducing functional SGSH proteins or enhancing their activity. This includes enzyme replacement therapy, which delivers exogenous SGSH to degrade accumulated heparan sulfate. Additionally, gene therapy techniques and small molecule-based chaperone therapy are being explored to restore or stabilize SGSH expression and function.

What is the specific tertiary structure of the SGSH protein and how does it contribute to its function in lysosomal storage disorders? 05/20/2018

The SGSH protein adopts a compact globular fold with distinctive domains responsible for substrate binding and catalytic activity. This structural arrangement allows SGSH to efficiently interact with heparan sulfate molecules within lysosomes, facilitating their degradation. The tight folding of SGSH enables precise coordination of enzymatic residues, ensuring optimal hydrolysis of sulphated glucosamine residues in heparan sulfate.

How does the absence or malfunction of SGSH lead to the development of lysosomal storage disorders, specifically mucopolysaccharidosis type III? 02/06/2018

Without functional SGSH, heparan sulfate cannot be properly catabolized. This results in the accumulation of undegraded heparan sulfate within lysosomes, leading to cellular dysfunction and tissue damage characteristic of mucopolysaccharidosis type III. The lack of SGSH-mediated hydrolysis prevents the timely clearance of sulphated glucosamine residues, disrupting cellular processes and compromising tissue homeostasis.

How is SGSH-related disorder diagnosed and managed 06/11/2016

SGSH-related disorders are diagnosed through clinical evaluations, enzyme activity assays, genetic testing, and analysis of urinary glycosaminoglycans. Once diagnosed, management of SGSH deficiency aims to alleviate symptoms and provide supportive care. This can include enzyme replacement therapy, which involves regular administration of the missing or malfunctioning SGSH enzyme. Symptom-specific interventions, such as developmental interventions, behavioral therapies, and medications, may also be utilized. Close monitoring and multidisciplinary care are essential for optimizing the quality of life for individuals with SGSH-related disorders.

Customer Reviews (3)

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Reviews
02/06/2021

    The shelf life of this reagent is long, ensuring the feasibility of long-term experiments.

    02/27/2020

      As an outstanding product, it enables me to effectively showcase my research findings.

      02/11/2020

        Playing a crucial role in providing technical support, this experimental reagent enables me to focus on experimental design and data analysis.

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