SAB1406433

Anti-SGSH antibody produced in mouse

purified immunoglobulin, buffered aqueous solution

• Sinónimos: HSS, MPS3A, SFMD
• Código UNSPSC: 12352203
• NACRES: NA.41
• conjugado: unconjugated
• application: WB
• clon: polyclonal
• reactividad de especies: human
• citations: 1
• técnicas: western blot: 1 μg/mL

Propiedades

• origen biológico: mouse
• Nivel de calidad: 100
• conjugado: unconjugated
• forma del anticuerpo: purified immunoglobulin
• tipo de anticuerpo: primary antibodies
• clon: polyclonal
• Formulario: buffered aqueous solution
• mol peso: antigen ~56.7 kDa
• reactividad de especies: human
• técnicas: western blot: 1 μg/mL
• Nº de acceso NCBI: NM_000199.2
• Nº de acceso UniProt: P51688
• Condiciones de envío: dry ice
• temp. de almacenamiento: −20°C
• modificación del objetivo postraduccional: unmodified
• Información sobre el gen: human ... SGSH(6448)

Descripción

Descripción general

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)

Inmunógeno

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

Sequence
MSCPVPACCALLLVLGLCRARPRNALLLLADDGGFESGAYNNSAIATPHLDALARRSLLFRNAFTSVSSCSPSRASLLTGLPQHQNGMYGLHQDVHHFNSFDKVRSLPLLLSQAGVRTGIIGKKHVGPETVYPFDFAYTEENGSVLQVGRNITRIKLLVRKFLQTQDDRPFFLYVAFHDPHRCGHSQPQYGTFCEKFGNGESGMGRIPDWTPQAYDPLDVLVPYFVPNTPAARADLAAQYTTVGRMDQGVGLVLQELRDAGVLNDTLVIFTSDNGIPFPSGRTNLYWPGTAEPLLVSSPEHPKRWGQVSEAYVSLLDLTPTILDWFSIPYPSYAIFGSKTIHLTGRSLLPALEAEPLWATVFGSQSHHEVTMSYPMRSVQHRHFRLVHNLNFKMPFPIDQDFYVSPTFQDLLNRTTAGQPTGWYKDLRHYYYRARWELYDRSRDPHETQNLATDPRFAQLLEMLRDQLAKWQWETHDPWVCAPDGVLEEKLSPQCQPLHNEL

Acciones bioquímicas o 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

Cláusula de descargo de responsabilidad

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.

Información de seguridad

• Código de clase de almacenamiento: 10 - Combustible liquids
• Clase de riesgo para el agua (WGK): WGK 1
• Punto de inflamabilidad (°F): Not applicable
• Punto de inflamabilidad (°C): Not applicable