Anti-SGSH antibody produced in mouse
SIGMA/SAB1406433 - purified immunoglobulin, buffered aqueous solution
Synonym: HSS; MPS3A; SFMD
Product Type: Chemical
Western Blotting Western Blot analysis of SGSH expression in transfected 293T cell line by SGSH polyclonal antibody. Lanes Lane 1: SGSH transfected lysate (55.22 kDa). Lane 2: Non-transfected lysate.
| antibody form | purified immunoglobulin |
| antibody product type | primary antibodies |
| biological source | mouse |
| clone | polyclonal |
| conjugate | unconjugated |
| form | buffered aqueous solution |
| mol wt | antigen ~56.7 kDa |
| NCBI accession no. | NM_000199.2   |
| Quality Level | 100 |
| shipped in | dry ice |
| species reactivity | human |
| storage temp. | −20°C |
| target post-translational modification | unmodified |
| technique(s) | western blot: 1 μg/mL |
| UniProt accession no. | P51688 |
| Biochem/physiol Actions: | The gene SGSH (N-sulfoglucosamine sulfohydrolase) encodes an enzyme that catalyzes the hydrolysis of N-linked sulfate groups from the GAGs (glycosaminoglycans) heparan sulfate and heparin. Deficiency of this enzyme due to mutations causes lysosomal storage of glycoaminoglycans. This leads to a disease called the mucopolysaccharidosis type IIIA or Sanfilippo A syndrome, which is a childhood-onset neurodegenerative disease characterized by facial, visceral and skeletal abnormalities. |
| Disclaimer: | Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals. |
| General description: | This gene encodes one of several enzymes involved in the lysosomal degradation of heparan sulfate. Mutations in this gene are associated with Sanfilippo syndrome A, one type of the lysosomal storage disease mucopolysaccaridosis III, which results from impaired degradation of heparan sulfate. Transcripts of varying sizes have been reported but their biological validity has not been determined. (provided by RefSeq) |
| Immunogen: | SGSH (NP_000190.1, 1 a.a. ~ 502 a.a) full-length human protein. Sequence MSCPVPACCALLLVLGLCRARPRNA |
| Physical form: | Solution in phosphate buffered saline, pH 7.4 |
| RIDADR | NONH for all modes of transport |
| WGK Germany | WGK 1 |
| Flash Point(F) | Not applicable |
| Flash Point(C) | Not applicable |
| Storage Temp. | −20°C |
| UNSPSC | 12352203 |