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货号： bs-16092R 基本售价： 1380.0 元 规格： 100ul
- 规格：100ul
- 价格：1380.00元
- 规格：200ul
- 价格：2200.00元
产品信息
- 产品编号
- bs-16092R
- 英文名称
- FKBP14
- 中文名称
- FKBP14蛋白抗体
- 别 名
- 22 kDa FK506 binding protein; 22 kDa FK506-binding protein; 22 kDa FKBP; FK506 binding protein 14 (22 kDa); FK506 binding protein 14; FK506-binding protein 14; FKB14_HUMAN; FKBP 22; FKBP-14; FKBP-22; FKBP14; FKBP22; FLJ20731; Peptidyl prolyl cis trans isomerase; Peptidyl-prolyl cis-trans isomerase FKBP14; PPIase; PPIase FKBP14; Rotamase.
- 规格价格
- 100ul/1380元购买 200ul/2200元购买 大包装/询价
- 说 明 书
- 100ul 200ul
- 研究领域
- 细胞生物 免疫学
- 抗体来源
- Rabbit
- 克隆类型
- Polyclonal
- 交叉反应
- Human, Mouse, Rat, Pig, Cow, Horse, Rabbit,
- 产品应用
- WB=1:500-2000 ELISA=1:500-1000 IHC-P=1:400-800 IHC-F=1:400-800 ICC=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复）
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
- 分 子 量
- 24kDa
- 细胞定位
- 细胞浆
- 性 状
- Lyophilized or Liquid
- 浓 度
- 1mg/ml
- 免 疫 原
- KLH conjugated synthetic peptide derived from human FKBP14:31-130/211
- 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.
- PubMed
- PubMed
- 产品介绍
- background:
The protein encoded by this gene is a member of the FK506-binding protein family of peptidyl-prolyl cis-trans isomerases. The encoded protein is found in the lumen of the endoplasmic reticulum, where it is thought to accelerate protein folding. Defects in this gene are a cause of a type of Ehlers-Danlos syndrome (EDS). Both a protein-coding variant and noncoding variants are transcribed from this gene. [provided by RefSeq, Mar 2012]
Function:
PPIases accelerate the folding of proteins during protein synthesis.
Subcellular Location:
Endoplasmic reticulum lumen.
DISEASE:
Defects in FKBP14 are the cause of Ehlers-Danlos syndrome with progressive kyphoscoliosis, myopathy, and hearing loss (EDSKMH) [MIM:614557]. A syndrome with features of Ehlers-Danlos syndrome types VIA and VIB on the one hand, and the collagen VI-related congenital myopathies Ullrich congenital muscular dystrophy and Bethlem myopathy on the other hand. Clinically, this disorder is characterized by the following features: severe generalized hypotonia at birth with marked muscle weakness that improve in infancy; early-onset progressive kyphoscoliosis; joint hypermobility without contractures; hyperelastic skin with follicular hyperkeratosis, easy bruising, and occasional abnormal scarring; myopathy as confirmed by muscle MRI, histology, and electron microscopy; hearing impairment, which is predominantly sensorineural; and a normal ratio of lysyl pyridinoline to hydroxylysyl pyridinoline (LP/HP) in urine.
Similarity:
Contains 2 EF-hand domains.
Contains 1 PPIase FKBP-type domain.
SWISS:
Q9NWM8
Gene ID:
55033
Database links:Entrez Gene: 55033Human
Entrez Gene: 231997Mouse
Entrez Gene: 362366Rat
Omim: 614505Human
SwissProt: Q9NWM8Human
SwissProt: P59024Mouse
Unigene: 390838Human
Unigene: 274693Mouse
Unigene: 12713Rat
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.