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货号： bs-11689R-Bio 基本售价： 2980.0 元 规格： 100ul
产品信息
- 产品编号
- bs-11689R-Bio
- 英文名称
- Anti-FGGY/Biotin
- 中文名称
- 生物素标记的肌萎缩侧索硬化症相关蛋白FGGY抗体
- 别 名
- fggy; FGGY carbohydrate kinase domain containing; FGGY carbohydrate kinase domain-containing protein; FGGY_HUMAN; FLJ10986; MGC94804; OTTHUMP00000010078; OTTHUMP00000010081; OTTHUMP00000010082; OTTHUMP00000202071; RP11-242B9.1.
- 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 信号转导 Alzheimers
- 抗体来源
- Rabbit
- 克隆类型
- Polyclonal
- 交叉反应
- Human, Mouse, Rat, Dog, Cow, Horse, Rabbit, Sheep,
- 产品应用
- WB=1:50-200 ELISA=1:100-1000 IHC-P=1:50-200 IHC-F=1:50-200 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
- 60kDa
- 性 状
- Lyophilized or Liquid
- 浓 度
- 1mg/ml
- 免 疫 原
- KLH conjugated synthetic peptide derived from human FGGY (151-250aa)
- 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:
FGGY is a 551 amino acid member of the FGGY kinase family that exists as four isoforms which are produced by alternative splicing events. Expressed in lung, kidney, small intestine, liver and fetal brain, FGGY is encoded by a gene that maps to chromosome 1 and, when mutated, is associated with sporadic amyotrophic lateral sclerosis (ALS). ALS is a neurodegenerative disorder that affects motor neurons and results in fatal paralysis, usually within 2 to 5 years after initial diagnosis. Chromosome 1, on which the gene encoding FGGY is located, is the largest human chromosome, spanning about 260 million base pairs and making up 8% of the human genome. There are about 3,000 genes on chromosome 1, many of which are associated with genetic diseases, including Hutchinson-Gilford progeria, familial adenomatous polyposis, Stickler syndrome, Gaucher disease and Usher syndrome.
Function:
Expressed in kidney, lung and small intestine and to a lower extent in liver and detected in cerebrospinal fluid (at protein level).
Tissue Specificity:
Expressed in fetal brain (at protein level).
DISEASE:
Defects in FGGY are associated with sporadic amyotrophic lateral sclerosis (ALS) [MIM:105400]. Amyotrophic lateral sclerosis is a neurodegenerative disorder affecting upper and lower motor neurons and resulting in fatal paralysis. Sensory abnormalities are absent. Death usually occurs within 2 to 5 years. The etiology of amyotrophic lateral sclerosis is likely to be multifactorial, involving both genetic and environmental factors.
Similarity:
Belongs to the FGGY kinase family.
Database links:
UniProtKB/Swiss-Prot: Q96C11.2
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.