Anti-SGSH/HSS antibody (ab96030)
Key features and details
- Rabbit polyclonal to SGSH/HSS
- Suitable for: WB
- Reacts with: Human
- Isotype: IgG
Overview
-
Product name
Anti-SGSH/HSS antibody
See all SGSH/HSS primary antibodies -
Description
Rabbit polyclonal to SGSH/HSS -
Host species
Rabbit -
Tested applications
Suitable for: WBmore details -
Species reactivity
Reacts with: Human
Predicted to work with: Mouse, Dog
-
Immunogen
Recombinant fragment corresponding to Human SGSH/HSS aa 44-200.
-
Positive control
- 293T whole cell lysate
-
General notes
This product was previously labelled as SGSH
The Life Science industry has been in the grips of a reproducibility crisis for a number of years. Abcam is leading the way in addressing this with our range of recombinant monoclonal antibodies and knockout edited cell lines for gold-standard validation. Please check that this product meets your needs before purchasing.
If you have any questions, special requirements or concerns, please send us an inquiry and/or contact our Support team ahead of purchase. Recommended alternatives for this product can be found below, along with publications, customer reviews and Q&As
Properties
-
Form
Liquid -
Storage instructions
Shipped at 4°C. Upon delivery aliquot and store at -20°C. Avoid freeze / thaw cycles. -
Storage buffer
pH: 7.00
Preservative: 0.01% Thimerosal (merthiolate)
Constituents: 59.99% PBS, 40% Glycerol (glycerin, glycerine) -
Concentration information loading... -
Purity
Protein A purified -
Clonality
Polyclonal -
Isotype
IgG -
Research areas
Associated products
-
Compatible Secondaries
-
Isotype control
-
Positive Controls
-
Recombinant Protein
Applications
The Abpromise guarantee
Our Abpromise guarantee covers the use of ab96030 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
| Application | Abreviews | Notes |
|---|---|---|
| WB |
1/500 - 1/3000. Predicted molecular weight: 57 kDa.
|
| Notes |
|---|
|
WB
1/500 - 1/3000. Predicted molecular weight: 57 kDa. |
Target
-
Involvement in disease
Defects in SGSH are the cause of mucopolysaccharidosis type 3A (MPS3A) [MIM:252900]; also known as Sanfilippo syndrome A. MPS3A is a severe 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. MPS3A is characterized by earlier onset, rapid progression of symptoms and shorter survival. -
Sequence similarities
Belongs to the sulfatase family. -
Post-translational
modificationsThe 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. -
Cellular localization
Lysosome. - Information by UniProt
-
Database links
- Entrez Gene: 6448 Human
- Entrez Gene: 27029 Mouse
- Omim: 605270 Human
- SwissProt: P51688 Human
- Unigene: 31074 Human
- Unigene: 103788 Mouse
-
Alternative names
- Heparan sulfate sulfatase antibody
- Heparan sulphate sulphatase antibody
- HSS antibody
see all
Images
-
Western blot - Anti-SGSH/HSS antibody (ab96030)Anti-SGSH/HSS antibody (ab96030) at 1/1000 dilution + 293T whole cell lysate at 30 µg
Predicted band size: 57 kDa
7.5% SDS Page
Datasheets and documents
-
SDS download
-
Datasheet download
References (0)
ab96030 has not yet been referenced specifically in any publications.