Product nameAnti-GNS antibody [EPR8329(2)] - BSA and Azide free
See all GNS primary antibodies
DescriptionRabbit monoclonal [EPR8329(2)] to GNS - BSA and Azide free
Tested applicationsSuitable for: IP, WBmore details
Unsuitable for: Flow Cyt,ICC/IF or IHC-P
Species reactivityReacts with: Human
Synthetic peptide within Human GNS. The exact sequence is proprietary.
Database link: P15586
Ab249077 is the carrier-free version of ab154177. This format is designed for use in antibody labeling, including fluorochromes, metal isotopes, oligonucleotides, enzymes.
Our carrier-free formats are supplied in a buffer free of BSA, sodium azide and glycerol for higher conjugation efficiency.
Use our conjugation kits for antibody conjugates that are ready-to-use in as little as 20 minutes with <1 minute hands-on-time and 100% antibody recovery: available for fluorescent dyes, HRP, biotin and gold.
ab249077 is compatible with the Maxpar® Antibody Labeling Kit from Fluidigm.
Our RabMAb® technology is a patented hybridoma-based technology for making rabbit monoclonal antibodies. For details on our patents, please refer to RabMab® patents.
This product is a recombinant rabbit monoclonal antibody.
Storage instructionsShipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C long term. Avoid freeze / thaw cycle.
Concentration information loading...
Our Abpromise guarantee covers the use of ab249077 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
|IP||Use at an assay dependent concentration.|
|WB||Use at an assay dependent concentration. Predicted molecular weight: 62 kDa.|
Involvement in diseaseDefects in GNS are the cause of mucopolysaccharidosis type 3D (MPS3D) [MIM:252940]; 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.
Sequence similaritiesBelongs to the sulfatase family.
modificationsThe 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.
- Information by UniProt
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ab249077 has not yet been referenced specifically in any publications.