SAB1406433

Anti-SGSH antibody produced in mouse

purified immunoglobulin, buffered aqueous solution

• Sinônimo(s): HSS, MPS3A, SFMD
• Código UNSPSC: 12352203
• NACRES: NA.41

Propriedades

• fonte biológica: mouse
• Nível de qualidade: 100
• conjugado: unconjugated
• forma do anticorpo: purified immunoglobulin
• tipo de produto de anticorpo: primary antibodies
• clone: polyclonal
• forma: buffered aqueous solution
• peso molecular: antigen ~56.7 kDa
• reatividade de espécies: human
• técnica(s): western blot: 1 μg/mL
• nº de adesão NCBI: NM_000199.2
• nº de adesão UniProt: P51688
• Condições de expedição: dry ice
• temperatura de armazenamento: −20°C
• modificação pós-traducional do alvo: unmodified
• Informações sobre genes: human ... SGSH(6448)

Descrição

Descrição geral

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)

Imunogênio

SGSH (NP_000190.1, 1 a.a. ~ 502 a.a) full-length human protein.

Sequence
MSCPVPACCALLLVLGLCRARPRNALLLLADDGGFESGAYNNSAIATPHLDALARRSLLFRNAFTSVSSCSPSRASLLTGLPQHQNGMYGLHQDVHHFNSFDKVRSLPLLLSQAGVRTGIIGKKHVGPETVYPFDFAYTEENGSVLQVGRNITRIKLLVRKFLQTQDDRPFFLYVAFHDPHRCGHSQPQYGTFCEKFGNGESGMGRIPDWTPQAYDPLDVLVPYFVPNTPAARADLAAQYTTVGRMDQGVGLVLQELRDAGVLNDTLVIFTSDNGIPFPSGRTNLYWPGTAEPLLVSSPEHPKRWGQVSEAYVSLLDLTPTILDWFSIPYPSYAIFGSKTIHLTGRSLLPALEAEPLWATVFGSQSHHEVTMSYPMRSVQHRHFRLVHNLNFKMPFPIDQDFYVSPTFQDLLNRTTAGQPTGWYKDLRHYYYRARWELYDRSRDPHETQNLATDPRFAQLLEMLRDQLAKWQWETHDPWVCAPDGVLEEKLSPQCQPLHNEL

Ações bioquímicas/fisiológicas

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.

forma física

Solution in phosphate buffered saline, pH 7.4

Exoneração de responsabilidade

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.

Informações de segurança

• Código de classe de armazenamento: 10 - Combustible liquids
• Classe de risco de água (WGK): WGK 1
• Ponto de fulgor (°F): Not applicable
• Ponto de fulgor (°C): Not applicable