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Rabbit Anti-TMEM106B/BF488 Conjugated antibody (bs-11694R-BF488)
訂購熱線:400-901-9800
訂購郵箱:sales@bioss.com.cn
訂購QQ:  400-901-9800
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說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產(chǎn)品編號 bs-11694R-BF488
英文名稱 Rabbit Anti-TMEM106B/BF488 Conjugated antibody
中文名稱 BF488標記的跨膜蛋白106B抗體
別    名 Tmem106b; Transmembrane protein 106B; 2310036D22Rik; 5830455K21Rik; 6430519M21Rik; AI428776; AI661344; FLJ11273; LRRGT00101; MGC33727; MGC94135; T106B_HUMAN.  
規(guī)格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
研究領域 細胞生物  神經(jīng)生物學  細胞膜蛋白  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 (predicted: Human, Mouse, Rat, Dog, Pig, Cow, Horse, Rabbit, Sheep, )
產(chǎn)品應用 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    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human TMEM106B (101-200aa)
亞    型 IgG
純化方法 affinity purified by Protein A
儲 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol
保存條件 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.
產(chǎn)品介紹 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.
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