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Mouse Anti-GNS Recombinant Antibody (CBLG1-1465) (CBMAB-G4247-LY)

This product is antibody recognizes GNS. The antibody CBLG1-1465 immunoassay techniques such as: ELISA, WB.
See all GNS antibodies

Summary

Host Animal
Mouse
Specificity
Human
Clone
CBLG1-1465
Antibody Isotype
IgG2b
Application
ELISA, WB

Basic Information

Immunogen
Human cell-derived rhGNS
Specificity
Human
Antibody Isotype
IgG2b
Clonality
Monoclonal
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Formulations & Storage [For reference only, actual COA shall prevail!]

Format
Liquid
Purity
> 95% Purity determined by SDS-PAGE.
Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freezethaw cycles.

Target

Full Name
glucosamine (N-acetyl)-6-sulfatase
Introduction
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]
Entrez Gene ID
2799
UniProt ID
P15586
Alternative Names
Glucosamine (N-Acetyl)-6-Sulfatase; N-Acetylglucosamine-6-Sulfatase; EC 3.1.6.14; G6S; Glucosamine -6-Sulfatase; Sanfilippo Disease IIID; Glucosamine-6-Sulfatase; EC 3.1.6;
Biological Process
Glycosaminoglycan catabolic process Source: ProtInc
Keratan sulfate catabolic process Source: Reactome
Cellular Location
Lysosome
Involvement in disease
Mucopolysaccharidosis 3D (MPS3D):
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.
PTM
The form A (78 kDa) is processed by internal peptidase cleavage to a 32 kDa N-terminal species (form B) and a 48 kDa C-terminal species.
The conversion to 3-oxoalanine (also known as C-formylglycine, FGly), of a serine or cysteine residue in prokaryotes and of a cysteine residue in eukaryotes, is critical for catalytic activity.
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For research use only. Not intended for any clinical use.

Custom Antibody Labeling

We also offer labeled antibodies developed using our catalog antibody products and nonfluorescent conjugates (HRP, AP, Biotin, etc.) or fluorescent conjugates (Alexa Fluor, FITC, TRITC, Rhodamine, Texas Red, R-PE, APC, Qdot Probes, Pacific Dyes, etc.).

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