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Anti-SGSH antibody produced in mouse

品牌
Sigma-Aldrich
货号
SAB1406433
规格纯度
purified immunoglobulin, buffered aqueous solution
参考价格
5045.46 *本价格含增值税费
促销
数量
-+
产品介绍:

产品说明

一般描述

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)

免疫原

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

Sequence
MSCPVPACCALLLVLGLCRARPRNALLLLADDGGFESGAYNNSAIATPHLDALARRSLLFRNAFTSVSSCSPSRASLLTGLPQHQNGMYGLHQDVHHFNSFDKVRSLPLLLSQAGVRTGIIGKKHVGPETVYPFDFAYTEENGSVLQVGRNITRIKLLVRKFLQTQDDRPFFLYVAFHDPHRCGHSQPQYGTFCEKFGNGESGMGRIPDWTPQAYDPLDVLVPYFVPNTPAARADLAAQYTTVGRMDQGVGLVLQELRDAGVLNDTLVIFTSDNGIPFPSGRTNLYWPGTAEPLLVSSPEHPKRWGQVSEAYVSLLDLTPTILDWFSIPYPSYAIFGSKTIHLTGRSLLPALEAEPLWATVFGSQSHHEVTMSYPMRSVQHRHFRLVHNLNFKMPFPIDQDFYVSPTFQDLLNRTTAGQPTGWYKDLRHYYYRARWELYDRSRDPHETQNLATDPRFAQLLEMLRDQLAKWQWETHDPWVCAPDGVLEEKLSPQCQPLHNEL

生化/生理作用

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.

外形

Solution in phosphate buffered saline, pH 7.4

免责声明

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.

基本信息

NACRESNA.41

产品性质

质量水平100
生物来源mouse
偶联物unconjugated
抗体形式purified immunoglobulin
antibody product typeprimary antibodies
克隆polyclonal
形式buffered aqueous solution
分子量antigen ~56.7 kDa
species reactivityhuman
technique(s)western blot: 1 μg/mL
NCBI登记号NM_000199.2
UniProt登记号P51688
运输dry ice
储存温度−20℃
Gene Informationhuman ... SGSH(6448)

安全信息

储存分类代码13 - Non Combustible Solids
WGKWGK 1
闪点(F)Not applicable
闪点(C)Not applicable

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