The SGSH Polyclonal Antibody (CAB8148) is a valuable tool for researchers studying SGSH, a crucial enzyme involved in lysosomal storage disorders such as mucopolysaccharidosis III. This antibody, generated in rabbits, exhibits high reactivity with human samples and has been validated for use in various applications, including Western blotting.SGSH, also known as N-sulfoglucosamine sulfohydrolase, plays a key role in breaking down glycosaminoglycans in the lysosome. Mutations in the SGSH gene can lead to the accumulation of glycosaminoglycans and the development of MPS III, a devastating genetic disorder that affects the central nervous system.
The SGSH Polyclonal Antibody enables researchers to detect and analyze SGSH levels in cell samples, providing valuable insights into lysosomal storage disorders and potential therapeutic strategies.By studying SGSH and its role in lysosomal function, researchers can advance our understanding of MPS III and other related diseases, ultimately contributing to the development of new treatments and diagnostic tools. The SGSH Polyclonal Antibody is a powerful resource for investigators seeking to unravel the complexities of lysosomal storage disorders and improve patient outcomes.
Product Name:
SGSH Rabbit Polyclonal Antibody
SKU:
CAB8148
Size:
20uL, 100uL
Isotype:
IgG
Host Species:
Rabbit
Reactivity:
Human,Mouse,Rat
Immunogen:
Recombinant fusion protein containing a sequence corresponding to amino acids 273-502 of human SGSH (NP_000190.1).
This gene encodes the enzyme sulfamidase; one of several enzymes involved in the lysosomal degradation of heparan sulfate. Mutations in this gene are associated with the lysosomal storage disease mucopolysaccaridosis IIIA, also known as Sanfilippo syndrome A, which results from impaired degradation of heparan sulfate. Transcripts of varying sizes have been reported but their biological validity has not been determined.
Purification Method:
Affinity purification
Gene ID:
6448
Storage Buffer:
Store at -20℃. Avoid freeze / thaw cycles.Buffer: PBS with 0.02% sodium azide,50% glycerol,pH7.3.
Western blot analysis of Mouse lung, using SGSH antibody (CAB8148) at 1:500 dilution.Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (CABS014) at 1:10000 dilution.Lysates/proteins: 25μg per lane.Blocking buffer: 3% nonfat dry milk in TBST.Detection: ECL Basic Kit (AbGn00020).Exposure time: 90s.
