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货号： bs-11694R-RBITC 基本售价： 2980.0 元 规格： 100ul
产品信息
- 产品编号
- bs-11694R-RBITC
- 英文名称
- Anti-TMEM106B/RBITC
- 中文名称
- 罗丹明（RBITC）标记的跨膜蛋白106B抗体
- 别 名
- Tmem106b; Transmembrane protein 106B; 2310036D22Rik; 5830455K21Rik; 6430519M21Rik; AI428776; AI661344; FLJ11273; LRRGT00101; MGC33727; MGC94135; T106B_HUMAN.
- 规格价格
- 100ul/2980元购买 大包装/询价
- 说 明 书
- 100ul
- 研究领域
- 细胞生物 神经生物学 细胞膜蛋白
- 抗体来源
- Rabbit
- 克隆类型
- Polyclonal
- 交叉反应
- Human, Mouse, Rat, Dog, Pig, Cow, Horse, 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
- 31kDa
- 性 状
- Lyophilized or Liquid
- 浓 度
- 1mg/ml
- 免 疫 原
- KLH conjugated synthetic peptide derived from human TMEM106B (101-200aa)
- 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:
TMEM106B is a 274 amino acid single-pass membrane protein that is encoded by a gene which maps to human chromosome 7. Chromosome 7 houses over 1,000 genes and comprises nearly 5% of the human genome. Defects in some of the genes localized to chromosome 7 have been linked to Osteogenesis imperfecta, Pendred syndrome, Lissencephaly, Citrullinemia and Shwachman-Diamond syndrome. The deletion of a portion of the q arm of chromosome 7 is associated with Williams-Beuren syndrome, a condition characterized by mild mental retardation, an unusual comfort and friendliness with strangers and an elfin appearance. Deletions of portions of the q arm of chromosome 7 are also seen in a number of myeloid disorders, including cases of acute myelogenous leukemia and myelodysplasia.
Subcellular Location:
Late endosome membrane; Single-pass type II membrane protein. Lysosome membrane; Single-pass type II membrane protein.
Tissue Specificity:
Expressed in frontal cortex.
DISEASE:
Note=TMEM106B genotype, when containing 3 particular single-nucleotide polymorphisms, is strongly correlated with frontotemporal lobar degeneration with TAR DNA-binding protein (TDP-43) inclusions (FTLD-TDP). Frontotemporal lobar degeneration (FTLD) is the second most common cause of presenile dementia and 20% of patients with this neurodegenerative disease have autosomal dominant GRN mutations. Expression of TMEM106B associated with these polymorphisms is increased in frontal cortex of patients with FTLD-TDP compared to unaffected controls. Thus, increased TMEM106B expression in the brain may be linked to mechanisms of disease in FTLD-TDP and risk alleles confer genetic susceptibility by increasing gene expression.
Similarity:
Belongs to the TMEM106 family.
Database links:Entrez Gene: 54664 Human
Entrez Gene: 71900 Mouse
Entrez Gene: 312132 Rat
Omim: 613413 Human
SwissProt: Q9NUM4 Human
SwissProt: Q80X71 Mouse
SwissProt: Q6AYA5 Rat
Unigene: 396358 Human
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.