sgsh
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  • Official Full Name
  • N-sulfoglucosamine sulfohydrolase
  • Background
  • 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
Cat.#:SGSH-695HTag:His|GST
Source (Host):E. coliSpecies:Human
Product nameRecombinant Human SGSH Protein, His/GST-tagged
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Cat.#:SGSH-195HTag:His
Source (Host):HEK293Species:Human
Product nameRecombinant Human SGSH protein, His-tagged
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Cat.#:SGSH-22HTag:His
Source (Host):Insect cellsSpecies:Human
Product nameRecombinant Human SGSH protein, His-tagged
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Cat.#:SGSH-2634HTag:His
Source (Host):E. coliSpecies:Human
Product nameRecombinant Human SGSH, His-tagged
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Cat.#:SGSH-279HTag:Myc|DDK
Source (Host):HEK293Species:Human
Product nameRecombinant Human SGSH, MYC/DDK-tagged
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Cat.#:SGSH-280HTag:GST
Source (Host):Wheat germSpecies:Human
Product nameRecombinant Human SGSH, GST-tagged
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Cat.#:SGSH-30971THTag:N/A
Source (Host):Wheat germSpecies:Human
Product nameRecombinant Human SGSH
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Cat.#:Sgsh-713MTag:His
Source (Host):Baculovirus Sf21 Insect CellsSpecies:Mouse
Product nameRecombinant Mouse Sgsh Protein, His-tagged
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Cat.#:SGSH-6660ZTag:His
Source (Host):Mammalian CellsSpecies:Zebrafish
Product nameRecombinant Zebrafish SGSH
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Cat.#:SGSH-1884HCLTag:N/A
Source (Host):Species:Human
Product nameRecombinant Human SGSH 293 Cell Lysate
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Involved Pathway

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 degradation
HYAL2; GNS; IDS; SPAM1; ARSB; HGSNAT; NAGLU; HPSE; GALNS; HYAL1
Metabolic pathways
DGKE; FDPS; AMY2A2; CYP19A1; NDUFS8; PTDSS1; NDUFB8; PLCG1; FUT9A; Aldh1a7
Lysosome
ACP5B; LIPA; NAGLU; TPP1; ARSG; ATP6V0A1B; CTSBB; GUSB; LAMP1; GGA3

Protein Function

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
Function N-sulfoglucosamine sulfohydrolase activity
Related Protein SGSH
Function catalytic activity
Related Protein GNSA; PBLD1; PHKA1; AGLA; ALDH1L1; PPM1DB; DPYDB; ISOC1; CDYL; PPM1E
Function metal ion binding
Related Protein ZFP628; PITPNM1; HINFP; TGM4; ZNF544; PPM1LA; ACY3; SUMF2; FYNB; ZNF726
Function sulfuric ester hydrolase activity
Related Protein GNSA; GNSB; SULF2A; SULF2B; GALNS; STS; ARSA; GNS; SGSH

Interacting Protein

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.
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SGSH Related Articles

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).