- ¥1380 - 2200
- LMAI Bio
- LM-4827R
- 进口/国产
- 2026年03月05日
- 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）
- Rabbit
- Human, Mouse, Rat, Dog, Pig, Cow, Sheep,
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- 详细信息
- 文献和实验
- 技术资料
- 供应商：
上海联迈生物工程有限公司
- 库存：
大量
- 目录编号：
LM-4827R
- 克隆性：
多克隆
- 抗原来源：
Rabbit
- 保质期：
1年
- 抗体英文名：
FHL1
- 抗体名：
骨骼肌蛋白FHL1抗体
- 宿主：
Rabbit
- 适应物种：
Human, Mouse, Rat, Dog, Pig, Cow, Sheep,
- 免疫原：
KLH conjugated synthetic peptide derived from human FHL1:151-250/323
- 亚型：
IgG
- 形态：
Lyophilized or Liquid
- 应用范围：
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）
- 浓度：
1mg/ml
- 保存条件：
Store at -20 °C
- 规格：
100ul 200ul
| 英文名称 | FHL1 |
| 中文名称 | 骨骼肌蛋白FHL1抗体 |
| 别 名 | bA535K18.1; FHL 1; FHL 1B; FHL-1; FHL1; FHL1 protein; FHL1_HUMAN; FHL1A; FHL1B; FLH1A; Four and a half LIM domains 1; Four and a half LIM domains protein 1; Four and a half Lin11 Isl 1 and Mec 3 domains 1; KYO T; LIM protein SLIMMER; MGC111107; RAM14-1; RBP associated molecule 14-1; Skeletal muscle LIM protein 1; Skeletal muscle LIM-protein 1; SLIM 1; SLIM; SLIM-1; SLIM1; SLIMMER; XMPMA. |
| 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 | 心血管 转录调节因子 锌指蛋白 表观遗传学 |
| 抗体来源 | Rabbit |
| 克隆类型 | Polyclonal |
| 交叉反应 | Human, Mouse, Rat, Dog, Pig, Cow, Sheep, |
| 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 | 35kDa |
| 细胞定位 | 细胞核 细胞浆 |
| 性 状 | Lyophilized or Liquid |
| 浓 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human FHL1:151-250/323 |
| 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: This gene encodes a member of the four-and-a-half-LIM-only protein family. Family members contain two highly conserved, tandemly arranged, zinc finger domains with four highly conserved cysteines binding a zinc atom in each zinc finger. Expression of these family members occurs in a cell- and tissue-specific mode and these proteins are involved in many cellular processes. Mutations in this gene have been found in patients with Emery-Dreifuss muscular dystrophy. Multiple alternately spliced transcript variants which encode different protein isoforms have been described.[provided by RefSeq, Nov 2009] Function: May have an involvement in muscle development or hypertrophy. Subcellular Location: Isoform 1: Cytoplasm. Isoform 3: Cytoplasm. Nucleus. Isoform 2: Nucleus. Cytoplasm, cytosol. Note=Predominantly nuclear in myoblasts but is cytosolic in differentiated myotubes. Tissue Specificity: Isoform 1 is highly expressed in skeletal muscle and to a lesser extent in heart, placenta, ovary, prostate, testis, small intestine, colon and spleen. Expression is barely detectable in brain, lung, liver, kidney, pancreas, thymus and peripheral blood leukocytes. Isoform 2 is expressed in brain, skeletal muscle and to a lesser extent in heart, colon, prostate and small intestine. Isoform 3 is expressed in testis, heart and skeletal muscle. DISEASE: Defects in FHL1 are the cause of X-linked dominant scapuloperoneal myopathy (SPM) [MIM:300695]. Scapuloperoneal syndrome (SPS) was initially described more than 120 years ago by Jules Broussard as 'une forme hereditaire d'atrophie musculaire progressive' beginning in the lower legs and affecting the shoulder region earlier and more severely than distal arm. The etiology of this condition remains unclear. Defects in FHL1 are the cause of X-linked myopathy with postural muscle atrophy (XMPMA) [MIM:300696]. Myopathies are inherited muscle disorders characterized by weakness and atrophy of voluntary skeletal muscle, and several types of myopathy also show involvement of cardiac muscle. XMPMA is a distinct form of adult-onset X-linked recessive myopathy with several features in common with other myopathies, but the presentation of a pseudoathletic phenotype, scapuloperoneal weakness, and bent spine is unique and might render the clinical phenotype distinguishable from other myopathies. Defects in FHL1 are the cause of X-linked severe early-onset reducing body myopathy (RBM) [MIM:300717]. RBM is a rare muscle disorder causing progressive muscular weakness and characteristic intracytoplasmic inclusions in myofibers. Clinical presentations of RBM have ranged from early onset fatal to childhood onset to adult onset cases. Defects in FHL1 are the cause of X-linked childhood-onset reducing body myopathy (CO-RBM) [MIM:300718]. This disorder is allelic to severe early-onset reducing body myopathy (RBM) [MIM:300717]. Similarity: Contains 3 LIM zinc-binding domains. SWISS: Q13642 Gene ID: 2273 Database links: Entrez Gene: 509056 Cow Entrez Gene: 2273 Human Entrez Gene: 14199 Mouse Entrez Gene: 25177 Rat Omim: 300163 Human SwissProt: Q13642 Human SwissProt: P97447 Mouse SwissProt: Q9WUH4 Rat Unigene: 435369 Human Unigene: 3126 Mouse Unigene: 54261 Rat Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| 产品图片 |  Sample: testis (Mouse) Lysate at 40 ug Primary: Anti-FHL1 (bs-4827R) at 1/300 dilution Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution Predicted band size: 35 kD Observed band size: 35 kD |
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