N-sulfoglucosamine sulfohydrolase (sulfamidase)

believed to be defect in mucopolysaccharidosis IIIA (Sanfilippo syndrome A)

Also Known As:

sulfamidase; sulphoglucosamine sulphamidase; 2-deoxy-D-glucoside-2-sulfamate sulfohydrolase; MPS3A protein, human; N-sulfoglucosamine sulfohydrolase (sulfamidase) protein, mouse; N-sulfoglucosamine sulfohydrolase protein, human; SFMD protein, human; SGSH protein, human; Sgsh protein, mouse; heparin N-sulfatase; heparin sulfamatase; heparin sulfamidase; sulfamate sulfatase; sulfamate sulfohydrolase; sulfoglucosamine sulfamidase; sulphamate sulphamidase; sulphamidase

Networked: 125 relevant articles (8 outcomes, 9 trials/studies)

Relationship Network

Bio-Agent Context: Research Results

Enzymes and Coenzymes: 1
- Enzymes: 152586
  - Hydrolases: 2166
    - N-sulfoglucosamine sulfohydrolase: 125

Experts

1.	Hopwood, John J: 32 articles (01/2018 - 02/2002)
2.	Hemsley, Kim M: 24 articles (06/2020 - 02/2004)
3.	Hassiotis, Sofia: 15 articles (01/2017 - 03/2009)
4.	Beard, Helen: 12 articles (06/2020 - 03/2009)
5.	King, Barbara: 10 articles (01/2017 - 03/2007)
6.	Lau, Adeline A: 9 articles (06/2020 - 02/2004)
7.	Snel, Marten F: 9 articles (06/2020 - 01/2015)
8.	Trim, Paul J: 9 articles (06/2020 - 01/2015)
9.	Hopwood, J J: 9 articles (12/2019 - 06/2000)
10.	Byers, Sharon: 8 articles (12/2021 - 01/2007)

Related Diseases

1.	Mucopolysaccharidosis III (Sanfilippo Syndrome) 01/01/2016 - "Continual Low-Dose Infusion of Sulfamidase Is Superior to Intermittent High-Dose Delivery in Ameliorating Neuropathology in the MPS IIIA Mouse Brain." 05/01/2016 - "Repeated replacement of sulphamidase via cerebrospinal fluid injection is an effective treatment for pathological changes in the brain in mice and dogs with the lysosomal storage disorder, mucopolysaccharidosis type IIIA (MPS IIIA). " 10/16/2012 - "A recent report on PerT-treated sulfamidase in murine MPS IIIA confirmed enhanced delivery to other tissues but failed to observe clearance of storage in neurons. " 12/01/2018 - "The aim of this study was to analyze the clinical and imaging features of a pediatric patient with mucopolysaccharidosis type IIIA (MPS IIIA) and a novel mutation of the N-sulfoglucosamine sulfohydrolase (SGSH) in 1 pedigree. " 01/01/2010 - "The aim of this study was to investigate mouse embryonic stem (ES) cell-derived glial precursor cells as a vehicle for sulfamidase delivery in the MPS-IIIA mouse brain. "
2.	Mucopolysaccharidoses 02/01/2012 - "Liver production of sulfamidase reverses peripheral and ameliorates CNS pathology in mucopolysaccharidosis IIIA mice." 12/01/2022 - "Intracerebroventricular dosing of N-sulfoglucosamine sulfohydrolase in mucopolysaccharidosis IIIA mice reduces markers of brain lysosomal dysfunction." 01/01/2022 - "Chemically modified recombinant human sulfamidase (SOBI003) in mucopolysaccharidosis IIIA patients: Results from an open, non-controlled, multicenter study." 12/13/2019 - "Enhancing the Therapeutic Potential of Sulfamidase for the Treatment of Mucopolysaccharidosis IIIA." 01/01/2018 - "Overcoming Limitations Inherent in Sulfamidase to Improve Mucopolysaccharidosis IIIA Gene Therapy."
3.	Gliosis 04/01/2016 - "Sulphamidase infusion mediated a statistically significant reduction in primary (heparan sulphate) and secondary (gangliosides GM2, GM3) substrate accumulation in the brain, with small reductions in micro- but not astro-gliosis. "
4.	Lysosomal Storage Diseases (Lysosomal Storage Disease) 01/01/2015 - "Mucopolysaccharidosis type IIIA (MPS-IIIA, Sanfilippo syndrome) is a Lysosomal Storage Disease caused by cellular deficiency of N-sulfoglucosamine sulfohydrolase (SGSH). " 04/01/2015 - "Mucopolysaccharidosis (MPS) IIIA is a neuropathic lysosomal storage disease caused by deficiency in N-sulfoglucosamine sulfohydrolase (SGSH). " 01/01/2018 - "Sulfamidase (SGSH) deficiency causes mucopolysaccharidosis type IIIA (MPS IIIA), a lysosomal storage disease (LSD) that affects the CNS. " 01/01/2019 - "Patients with the lysosomal storage disease mucopolysaccharidosis IIIA (MPSIIIA) lack the lysosomal enzyme N-sulfoglucosamine sulfohydrolase (SGSH), one of the many enzymes involved in degradation of heparan sulfate. " 02/01/2012 - "Mucopolysaccharidosis type IIIA (MPSIIIA) is an inherited lysosomal storage disease caused by deficiency of sulfamidase, resulting in accumulation of the glycosaminoglycan (GAG) heparan sulfate. "
5.	Neurodegenerative Diseases (Neurodegenerative Disease) 12/01/2019 - "Sanfilippo syndrome (mucopolysaccharidosis type IIIA; MPS IIIA) is an inherited paediatric-onset neurodegenerative disorder caused by the lysosomal deficiency of sulphamidase with subsequent accumulation of heparan sulphate. " 11/01/2017 - "Mucopolysaccharidosis IIIA or Sanfilippo disease type A is a progressive neurodegenerative disorder presenting in early childhood, caused by an inherited deficiency of the lysosomal hydrolase sulfamidase. " 01/01/2016 - "Mucopolysaccharidosis IIIA is a heritable neurodegenerative disorder resulting from the dysfunction of the lysosomal hydrolase sulphamidase. " 05/01/1997 - "Sanfilippo A syndrome (mucopolysaccharidosis type IIIA, MPS-IIIA) is an autosomal recessive neurodegenerative disorder due to an enzymatic defect of the lysosomal enzyme sulphamidase (EC 3.10.1.1) required for the degradation of heparan sulphate. " 08/01/2012 - "Mucopolysaccharidosis IIIA (MPS IIIA or Sanfilippo disease) is a neurodegenerative disorder caused by a deficiency in the lysosomal enzyme sulfamidase (SGSH), catabolizing heparan sulfate (HS). "

Related Drugs and Biologics

1.	Enzymes
2.	Heparitin Sulfate (Heparan Sulfate)
3.	Gangliosides
4.	Polysaccharides (Glycans)
5.	N-sulfoglucosamine sulfohydrolase (sulfamidase)
6.	Glycosaminoglycans
7.	Lysergic Acid Diethylamide (LSD)
8.	Sulfatases
9.	Hydrolases
10.	alpha-N-acetyl-D-glucosaminidase (alpha-N-acetylglucosaminidase)

Related Therapies and Procedures

1.	Injections
2.	Spinal Injections
3.	Cannula
4.	Spinal Infusions
5.	Therapeutics