您好,欢迎来到半岛电竞官方网址 ! [ 登录] [ 免费注册]
半岛电竞官方网址
位置: 首页> 品牌> Sigma-Aldrich> Anti-SGSH antibody produced in mouse

Anti-SGSH antibody produced in mouse

品牌
Sigma-Aldrich
货号
SAB1406433
规格纯度
purified immunoglobulin, buffered aqueous solution
价格
5045.46 *本价格含增值税费
促销
服务
  • 原厂保证
  • 包邮
  • 增值税票
数量
- +
半岛bd体育手机客户端 名称:
Anti-SGSH antibody produced in mouse
HSS
SFMD
MPS3A
半岛bd体育手机客户端 介绍:

半岛bd体育手机客户端 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

NACRES NA.41

半岛bd体育手机客户端 性质

质量水平 100
生物来源 mouse
偶联物 unconjugated
抗体形式 purified immunoglobulin
antibody product type primary antibodies
克隆 polyclonal
形式 buffered aqueous solution
分子量 antigen ~56.7 kDa
species reactivity human
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 Information human ... SGSH(6448)

安全信息

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

Sigma-Aldrich

推荐半岛bd体育手机客户端
Baidu
map