GNS
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Official Full Name
glucosamine (N-acetyl)-6-sulfatase
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Overview
The product of this gene is a lysosomal enzyme found in all cells. It is involved in the catabolism of heparin, heparan;sulphate, and keratan sulphate. Deficiency of this enzyme results in the accumulation of undegraded substrate and the;lysosomal storage disorder mucopolysaccharidosis type IIID (Sanfilippo D syndrome). Mucopolysaccharidosis type IIID is;the least common of the four subtypes of Sanfilippo syndrome. -
Synonyms
GNS; glucosamine (N-acetyl)-6-sulfatase; N-acetylglucosamine-6-sulfatase; N acetylglucosamine 6 sulfatase; Sanfilippo disease IIID; N28088; 2610016K11Rik; AU042285; C87209; G6S; Glucosamine (N-acetyl) 6 sulfatase; Glucosamine 6 sulfatase; Glucosamine-6-sulfatase; GNS_HUMAN; MGC21274; N acetylglucosamine 6 sulfatase [Precursor];
- Recombinant Proteins
- Cell & Tissue Lysates
- Protein Pre-coupled Magnetic Beads
- Chicken
- Human
- Mouse
- CHO
- HEK293
- HEK293T
- Human Cell
- In Vitro Cell Free System
- Mammalian Cell
- Mammalian cells
- Wheat Germ
- C
- His
- GST
- His (Fc)
- Avi
- Myc
- DDK
- N/A
- Involved Pathway
- Protein Function
- Interacting Protein
- GNS Related Articles
- GNS Related Research Area
GNS involved in several pathways and played different roles in them. We selected most pathways GNS 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 GNS 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 | ARSB;SGSH;HYAL6;HEXA;GLB1;IDS;HGSNAT;HPSE2;HYAL3 |
| Metabolic pathways | B3GALT6;SDHB;DAO.1;SMPD2A;ITGB1BP3;B3GNT3;DGAT1;GGT7;CEPT1B |
| Lysosome | ASAH1A;LIPA;IGF2R;ACP5B;Ctsl;CTSBA;AP3S2;GM2A;GNS |
GNS has several biochemical functions, for example, N-acetylglucosamine-6-sulfatase activity, metal ion binding, protein binding. Some of the functions are cooperated with other proteins, some of the functions could acted by GNS itself. We selected most functions GNS had, and list some proteins which have the same functions with GNS. You can find most of the proteins on our site.
| Function | Related Protein |
|---|---|
| N-acetylglucosamine-6-sulfatase activity | GNS;SULF1;GNSB;SULF2;GNSA |
| metal ion binding | NR5A5;CYP3C1L2;MCM10;NUBPL;ZFPM1;PRKCHA;ZNF143;ITGB2;TRIM26 |
| protein binding | SYNPO2L;GPS2;CREBZF;DZIP3;ARHGAP9;PVRL3;FRMPD2;CDH5;ANKRD49 |
| sulfuric ester hydrolase activity | GNS;SGSH;SULF2B;ARSA;GNSB;STS;SULF2A;GNSA;GALNS |
GNS 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 GNS here. Most of them are supplied by our site. Hope this information will be useful for your research of GNS.
ABL1; FYN; SRC; NCK1; GRB2; q8clp4_yerpe; Cep152; KIF2A; FGFR1OP; cona_canen; Msn; Prkaa1; PARD6B
- Q&As
- Reviews
Q&As (5)
Ask a questionGNS exhibits substrate specificity for N-acetylglucosamine-6-sulfate in GAGs. Its recognition of specific sulfated glycosaminoglycans is determined by the structure of the substrate.
GNS utilizes a catalytic mechanism involving conserved residues. The active site accommodates the substrate, and specific residues play a role in sulfate removal through hydrolysis.
GNS undergoes intracellular trafficking and post-translational modifications essential for its enzymatic activity. Proper folding and trafficking to lysosomes are crucial for functional GNS.
GNS is a sulfatase enzyme responsible for catalyzing the removal of sulfate groups from N-acetylglucosamine-6-sulfate in glycosaminoglycans, contributing to their degradation.
GNS is crucial for maintaining extracellular matrix integrity by participating in GAG degradation. Dysfunction of GNS leads to MPS IIID, a lysosomal storage disorder characterized by GAG accumulation.
Customer Reviews (3)
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