Key features and details
- Mouse polyclonal to GNS
- Suitable for: WB
- Reacts with: Human
- Isotype: IgG
Storage instructionsShipped at 4°C. Upon delivery aliquot and store at -20°C or -80°C. Avoid repeated freeze / thaw cycles.
Storage bufferpH: 7.40
Constituent: 2.68% PBS
Concentration information loading...
PurityProtein G purified
Our Abpromise guarantee covers the use of ab69971 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
|WB||1/500 - 1/1000. Detects a band of approximately 75 kDa (predicted molecular weight: 63 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
- 2610016K11Rik antibody
- AU042285 antibody
- C87209 antibody
All lanes : Anti-GNS antibody (ab69971) at 1/500 dilution
Lane 1 : GNS transfected 293T lysate
Lane 2 : Non transfected 293T lysate
Lysates/proteins at 25 µg per lane.
All lanes : Goat Anti-Mouse IgG (H&L)-HRP at 1/2500 dilution
Predicted band size: 63 kDa
Observed band size: 75 kDa why is the actual band size different from the predicted?
ab69971 has not yet been referenced specifically in any publications.