FITC标记的FKBP14蛋白抗体
产品名称： FITC标记的FKBP14蛋白抗体
英文名称： Anti-FKBP14/FITC
产品编号： HZ-16092R-FITC
产品价格： null
产品产地： 中国/上海
品牌商标： HZbscience
更新时间： 2023-08-17T10:24:20
使用范围： ICC=1:50-200 IF=1:50-200
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Rabbit Anti-FKBP14/FITC Conjugated antibody
FITC标记的FKBP14蛋白抗体
| 英文名称 | Anti-FKBP14/FITC |
| 中文名称 | FITC标记的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/2980元 购买 大包装/询价 |
| 说 明 书 | 100ul |
| 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 | 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 | 24kDa |
| 性 状 | Lyophilized or Liquid |
| 浓 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human FKBP14 |
| 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: 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. Database links: Entrez Gene: 55033 Human Entrez Gene: 231997 Mouse Entrez Gene: 362366 Rat Omim: 614505 Human SwissProt: Q9NWM8 Human SwissProt: P59024 Mouse Unigene: 390838 Human Unigene: 274693 Mouse Unigene: 12713 Rat Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications |
由该基因编码的蛋白是肽基脯氨酰顺反异构酶的FK506结合蛋白家族的成员。编码的蛋白质被发现在内质网的内腔中，在那里被认为加速蛋白质折叠。这种基因的缺陷是埃勒斯-丹洛斯综合征（EDS）的一个原因。蛋白质编码变体和非编码变体都是从该基因转录而来的。〔RefSeq，2012〕