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位置: 首页> 品牌> Sigma-Aldrich> Anti-CLN8 (ab1) antibody produced in rabbit

Anti-CLN8 (ab1) antibody produced in rabbit

品牌
Sigma-Aldrich
货号
SAB2100448
规格纯度
affinity isolated antibody
价格
6305.78 *本价格含增值税费
促销
服务
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  • 包邮
  • 增值税票
数量
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半岛bd体育手机客户端 名称:
Anti-CLN8 (ab1) antibody produced in rabbit
Anti-EPMR
Anti-C8orf61
Anti-Ceroid-lipofuscinosis, neuronal 8 (epilepsy, progressive with mental retardation)
Anti-FLJ39417
半岛bd体育手机客户端 介绍:

半岛bd体育手机客户端 说明

免疫原

Synthetic peptide directed towards the N terminal region of human CLN8

生化/生理作用

CLN8 is a transmembrane protein belonging to a family of proteins containing TLC domains, which are postulated to function in lipid synthesis, transport, or sensing. The protein localizes to the endoplasmic reticulum (ER), and may recycle between the ER and ER-Golgi intermediate compartment. Mutations in this gene are associated with progressive epilepsy with mental retardation (EMPR), which is a subtype of neuronal ceroid lipofuscinoses (NCL). Patients with mutations in this gene have altered levels of sphingolipid and phospholipids in the brain. Childhood-onset NCL are a group of autosomal recessive progressive encephalopathies characterized by the accumulation of autofluorescent material, mainly ATP synthase subunit C, in various tissues, notably in neurons. Based on clinical features, the country of origin of patients, and the molecular genetic background of the disorder, at least seven different forms are thought to exist. CLN8 is characterized by normal early development, onset of generalized seizures between 5 and 10 years, and subsequent progressive mental retardation.This gene encodes a transmembrane protein belonging to a family of proteins containing TLC domains, which are postulated to function in lipid synthesis, transport, or sensing. The protein localizes to the endoplasmic reticulum (ER), and may recycle between the ER and ER-Golgi intermediate compartment. Mutations in this gene are associated with progressive epilepsy with mental retardation (EMPR), which is a subtype of neuronal ceroid lipofuscinoses (NCL). Patients with mutations in this gene have altered levels of sphingolipid and phospholipids in the brain.

序列

Synthetic peptide located within the following region: MNPASDGGTSESIFDLDYASWGIRSTLMVAGFVFYLGVFVVCHQLSSSLN

外形

Purified antibody supplied in 1x PBS buffer with 0.09% (w/v) sodium azide and 2% sucrose.

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

生物来源 rabbit
质量水平 100
偶联物 unconjugated
抗体形式 affinity isolated antibody
antibody product type primary antibodies
克隆 polyclonal
形式 buffered aqueous solution
分子量 33 kDa
species reactivity dog, human, horse
浓度 0.5 mg - 1 mg/mL
technique(s) western blot: suitable
UniProt登记号 Q9UBY8
运输 wet ice
储存温度 ?20℃
Gene Information human ... CLN8(2055)

安全信息

储存分类代码 12 - Non Combustible Liquids
WGK WGK 3
闪点(F) Not applicable
闪点(C) Not applicable

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