GNS Antibody (PACO44940)
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주문- SKU:
- PACO44940
- Product type:
- Antibody
- Reactivity:
- Human
- Host Species:
- Rabbit
- Isotype:
- IgG
- Application:
- ELISA
- Application:
- IHC
- Antibody type:
- Polyclonal
- Conjugation:
- Unconjugated
Frequently bought together:
Description
Antibody Name: | GNS Antibody (PACO44940) |
Antibody SKU: | PACO44940 |
Size: | 50ul |
Host Species: | Rabbit |
Tested Applications: | ELISA, IHC |
Recommended Dilutions: | ELISA:1:2000-1:10000, IHC:1:20-1:200 |
Species Reactivity: | Human |
Immunogen: | Recombinant Human N-acetylglucosamine-6-sulfatase protein (293-552AA) |
Form: | Liquid |
Storage Buffer: | PBS with 0.02% sodium azide, 50% glycerol, pH7.3. |
Purification Method: | Antigen Affinity Purified |
Clonality: | Polyclonal |
Isotype: | IgG |
Conjugate: | Non-conjugated |
Immunohistochemistry of paraffin-embedded human testis tissue using PACO44940 at dilution of 1:100. | |
Immunohistochemistry of paraffin-embedded human kidney tissue using PACO44940 at dilution of 1:100. |
Background: | extracellular exosome, lysosomal lumen, N-acetylglucosamine-6-sulfatase activity, sulfuric ester hydrolase activity, glycosaminoglycan catabolic process, keratan sulfate catabolic process |
Synonyms: | N-acetylglucosamine-6-sulfatase (EC 3.1.6.14) (Glucosamine-6-sulfatase) (G6S), GNS |
UniProt Protein Function: | GNS: Defects in GNS are the cause of mucopolysaccharidosis type 3D (MPS3D); also known as Sanfilippo D syndrome. MPS3D is a form of mucopolysaccharidosis type 3, an autosomal recessive lysosomal storage disease due to impaired degradation of heparan sulfate. MPS3 is characterized by severe central nervous system degeneration, but only mild somatic disease. Onset of clinical features usually occurs between 2 and 6 years; severe neurologic degeneration occurs in most patients between 6 and 10 years of age, and death occurs typically during the second or third decade of life. Belongs to the sulfatase family. |
UniProt Protein Details: | Protein type:EC 3.1.6.14; Hydrolase; Glycan Metabolism - glycosaminoglycan degradation Chromosomal Location of Human Ortholog: 12q14 Cellular Component: lysosomal lumen Molecular Function:protein binding; N-acetylglucosamine-6-sulfatase activity; sulfuric ester hydrolase activity; metal ion binding Biological Process: keratan sulfate metabolic process; glycosaminoglycan catabolic process; glycosaminoglycan metabolic process; carbohydrate metabolic process; pathogenesis; keratan sulfate catabolic process Disease: Mucopolysaccharidosis, Type Iiid |
NCBI Summary: | 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. [provided by RefSeq, Jul 2008] |
UniProt Code: | P15586 |
NCBI GenInfo Identifier: | 232126 |
NCBI Gene ID: | 2799 |
NCBI Accession: | P15586.3 |
UniProt Related Accession: | P15586 |
Molecular Weight: | |
NCBI Full Name: | N-acetylglucosamine-6-sulfatase |
NCBI Synonym Full Names: | glucosamine (N-acetyl)-6-sulfatase |
NCBI Official Symbol: | GNS |
NCBI Official Synonym Symbols: | G6S |
NCBI Protein Information: | N-acetylglucosamine-6-sulfatase |
UniProt Protein Name: | N-acetylglucosamine-6-sulfatase |
UniProt Synonym Protein Names: | Glucosamine-6-sulfatase; G6S |
Protein Family: | Protein |
UniProt Gene Name: | GNS |
UniProt Entry Name: | GNS_HUMAN |