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货号： bs-13226R-PE-Cy7 基本售价： 2980.0 元 规格： 100ul
产品信息
- 产品编号
- bs-13226R-PE-Cy7
- 英文名称
- Anti-Fukutin/PE-Cy7
- 中文名称
- PE-Cy7标记的岩藻糖变旋酶/Fucose mutarotase抗体
- 别 名
- C10orf125; Chromosome 10 open reading frame 125; FUCM_HUMAN; Fucose mutarotase; FUCU; Protein fucU homolog.
- 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 信号转导
- 抗体来源
- Rabbit
- 克隆类型
- Polyclonal
- 交叉反应
- Human, Mouse, Rat, Dog, Pig, Cow,
- 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
- 17kDa
- 性 状
- Lyophilized or Liquid
- 浓 度
- 1mg/ml
- 免 疫 原
- KLH conjugated synthetic peptide derived from human Fukutin
- 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:
Fukutin, a secreted protein, is expressed in various tissues in normal individuals. Fukutin colocalizes with a Golgi marker and a granular cytoplasmic distribution, suggesting that fukutin passes through the Golgi before being packaged into secretory vesicles. Fukutin may be located in the extracellular matrix, where it interacts with and reinforces a large complex encompassing the outside and inside of muscle membranes; alternatively, as a secreted protein, fukutin may cause muscular dystrophy by an unknown mechanism. The fukutin gene is expressed at similar levels in control fetal and adult brain, but is much reduced in Fukuyama congenital muscular dystrophy (FCMD) brains. Fukutin deficiency affects the modification of glycosylation of DAG1 (α-dystroglycan), which then cannot localize or function properly and may be degraded or eluted from the extracellular surface membrane of the muscle fiber. FCMD is the first human disease known to be caused by an ancient retrotransposal integration. The gene which encodes fukutin maps to human chromosome 9q31.
Function:
Involved in the interconversion between alpha- and beta-L-fucoses. L-Fucose (6-deoxy-L-galactose) exists as alpha-L-fucose (29.5%) and beta-L-fucose (70.5%), the beta-form is metabolized through the salvage pathway. GDP-L-fucose formed either by the de novo or salvage pathways is transported into the endoplasmic reticulum, where it serves as a substrate for N- and O-glycosylations by fucosyltransferases. Fucosylated structures expressed on cell surfaces or secreted in biological fluids are believed to play a critical role in cell-cell adhesion and recognition processes.
Subunit:
Mainly homodimer, but exists also as homotetramer, homooctamer, and homodecamer. The homodimeric form seems catalytically inactive (By similarity).
Similarity:
Belongs to the rbsD / fucU family.
Database links:Entrez Gene: 282969Human
SwissProt: A2VDF0Human
Unigene: 155823Human
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.