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Recombinant Human SGSH, MYC/DDK-tagged

Cat.No. : SGSH-279H
Product Overview : Recombinant Human SGSH fused with C-terminal MYC/DDK, was expressed in HEK293 Cells.
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Cat. No. : SGSH-279H
Description : 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.
Source : HEK293 Cells
Molecular Mass : 54.6 kDa
Purity : >80% as determined by SDS-PAGE and Coomassie blue staining
Storagebuffer : 25 mM Tris.HCl, pH 7.3, 100 mM glycine, 10% glycerol.
Concentration : >50 ug/mL as determined by microplate BCA method
Preparation : Recombinant protein was captured through anti-DDK affinity column followed by conventional chromatography steps.
Storage : Store at -800C. Avoid repeated freeze-thaw cycles. Stable for at least 3 months from receipt of products under proper storage and handling conditions.
OfficialSymbol : SGSH
Gene Name : SGSH N-sulfoglucosamine sulfohydrolase [ Homo sapiens ]
Synonyms : SGSH; N-sulfoglucosamine sulfohydrolase; HSS; MPS3A; mucopolysaccharidosis type IIIA; SFMD; sulfamidase; heparan sulfate sulfatase; sulfoglucosamine sulfamidase; N-sulphoglucosamine sulphohydrolase; EC 3.10.1.1; Sulfoglucosamine sulfamidase; Sulphamidase
Gene ID : 6448
mRNA Refseq : NM_000199
Protein Refseq : NP_000190
MIM : 605270
UniProt ID : P51688
Chromosome Location : 17q25.3
Pathway : Glycosaminoglycan degradation; Heparan sulfate degradation; Lysosome; MPS I - Hurler syndrome
Function : N-sulfoglucosamine sulfohydrolase activity; catalytic activity; metal ion binding; sulfuric ester hydrolase activity

For Research Use Only. Not intended for any clinical use. No products from Creative BioMart may be resold, modified for resale or used to manufacture commercial products without prior written approval from Creative BioMart.

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Q&As (7)

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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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