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货号： bs-18906R-FITC 基本售价： 2980.0 元 规格： 100ul
产品信息
- 产品编号
- bs-18906R-FITC
- 英文名称
- Anti-MFSD8/FITC
- 中文名称
- FITC标记的MFSD8蛋白抗体
- 别 名
- Ceroid-lipofuscinosis neuronal protein 7; CLN7; Major facilitator superfamily domain-containing protein 8; MFSD8; MFSD8_HUMAN.
- 规格价格
- 100ul/2980元购买 大包装/询价
- 说 明 书
- 100ul
- 研究领域
- 肿瘤 细胞生物 信号转导
- 抗体来源
- Rabbit
- 克隆类型
- Polyclonal
- 交叉反应
- Human, Mouse, Rat, Pig, Cow, Rabbit, Sheep,
- 产品应用
- ICC=1:50-200 IF=1:50-200
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
- 分 子 量
- 57kDa
- 性 状
- Lyophilized or Liquid
- 浓 度
- 1mg/ml
- 免 疫 原
- KLH conjugated synthetic peptide derived from human MFSD8
- 亚 型
- IgG
- 纯化方法
- affinity purified by Protein A
- 储 存 液
- 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
- 保存条件
- Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
- 产品介绍
- background:
This gene encodes a ubiquitous integral membrane protein that contains a transporter domain and a major facilitator superfamily (MFS) domain. Other members of the major facilitator superfamily transport small solutes through chemiosmotic ion gradients. The substrate transported by this protein is unknown. The protein likely localizes to lysosomal membranes. Mutations in this gene are correlated with a variant form of late infantile-onset neuronal ceroid lipofuscinoses (vLINCL). [provided by RefSeq, Oct 2008]
Function:
May be a carrier that transport small solutes by using chemiosmotic ion gradients.
Subcellular Location:
Lysosome membrane.
Tissue Specificity:
Expressed at very low levels in all tissues tested.
DISEASE:
Defects in MFSD8 are the cause of neuronal ceroid lipofuscinosis type 7 (CLN7) [MIM:610951]. A form of late infantile neuronal ceroid lipofuscinosis. CNL are a clinically and genetically heterogeneous group of neurodegenerative disorders characterized by the intracellular accumulation of autofluorescent lipopigment storage material in different patterns ultrastructurally. The patterns most often observed CLN7 are mixed combinations of granular, curvilinear, fingerprint, and rectilinear profiles. The clinical course includes progressive dementia, seizures, and progressive visual failure.
Similarity:
Belongs to the major facilitator superfamily.
Database links:Entrez Gene: 256471Human
Entrez Gene: 72175Mouse
Entrez Gene: 361939Rat
Omim: 611124Human
SwissProt: Q8NHS3Human
SwissProt: Q8BH31Mouse
Unigene: 480701Human
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.