重组Anti-Fukutin抗体[EPR7913] (ab131280)
Key features and details
- Produced recombinantly (animal-free) for high batch-to-batch consistency and long term security of supply
- Rabbit monoclonal [EPR7913] to Fukutin
- Suitable for: WB, IHC-P
- Reacts with: Human
Related conjugates and formulations
概述
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产品名称
Anti-Fukutin抗体[EPR7913]
参阅全部 Fukutin 一抗 -
描述
兔单克隆抗体[EPR7913] to Fukutin -
宿主
Rabbit -
经测试应用
适用于: WB, IHC-Pmore details
不适用于: Flow Cyt or IP -
种属反应性
与反应: Human
预测可用于: Mouse, Rat -
免疫原
Synthetic peptide. This information is proprietary to Abcam and/or its suppliers.
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阳性对照
- HeLa, A549, fetal heart, and BxPC-3 lysates; Human kidney tissue.
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常规说明
This product is a recombinant monoclonal antibody, which offers several advantages including:
- - High batch-to-batch consistency and reproducibility
- - Improved sensitivity and specificity
- - Long-term security of supply
- - Animal-free production
Our RabMAb® technology is a patented hybridoma-based technology for making rabbit monoclonal antibodies. For details on our patents, please refer to RabMAb® patents.
性能
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形式
Liquid -
存放说明
Shipped at 4°C. Store at -20°C. Stable for 12 months at -20°C. -
解离常数(KD)
KD = 5.00 x 10 -11 M Learn more about KD -
存储溶液
pH: 7.20
Preservative: 0.01% Sodium azide
Constituents: 9% PBS, 40% Glycerol (glycerin, glycerine), 0.05% BSA, 50% Tissue culture supernatant -
Concentration information loading...
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纯度
Protein A purified -
克隆
单克隆 -
克隆编号
EPR7913 -
同种型
IgG -
研究领域
相关产品
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Alternative Versions
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Isotype control
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Positive Controls
应用
The Abpromise guarantee
Abpromise™承诺保证使用ab131280于以下的经测试应用
“应用说明”部分 下显示的仅为推荐的起始稀释度;实际最佳的稀释度/浓度应由使用者检定。
应用 | Ab评论 | 说明 |
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WB |
1/1000 - 1/10000. Predicted molecular weight: 53 kDa.
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IHC-P |
1/100 - 1/250. Perform heat mediated antigen retrieval with citrate buffer pH 6 before commencing with IHC staining protocol.
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说明 |
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WB
1/1000 - 1/10000. Predicted molecular weight: 53 kDa. |
IHC-P
1/100 - 1/250. Perform heat mediated antigen retrieval with citrate buffer pH 6 before commencing with IHC staining protocol. |
靶标
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功能
May be a glycosyltransferase which participates in glycosylation of alpha-dystroglycan/DAG1. May interact with and reinforce a large complex encompassing the outside and inside of muscle membranes. Could be involved in brain development. -
组织特异性
Widely expressed with highest expression in brain, heart, pancreas and skeletal muscle. Expressed at similar levels in control fetal and adult brain, but is much reduced in Fukuyama-type congenital dystrophy (FCMD) brains. Expressed in migrating neurons, including Cajar-Retzius cells and adult cortical neurons, as well as hippocampal pyramidal cells and cerebellar Purkinje cells. No expression observed in the glia limitans, the subpial astrocytes (which contribute to basement membrane formation) or other glial cells. In the FCMD brain, neurons in regions with no dysplasia show fair expression, whereas transcripts are nearly undetectable in the overmigrated dysplastic region. -
疾病相关
Defects in FKTN are the cause of muscular dystrophy-dystroglycanopathy congenital with brain and eye anomalies type A4 (MDDGA4) [MIM:253800]; also called congenital muscular dystrophy Fukuyama type (FCMD) or Walker-Warburg syndrome FKTN-related. MDDGA4 is an autosomal recessive disorder characterized by congenital muscular dystrophy associated with cobblestone lissencephaly and other brain anomalies. Patients suffer from generalized skeletal muscle weakness and hypotonia from early infancy, mental retardation and seizures. Occasional features include optic atrophy, retinal detachment, cardiomyopathy.
Defects in FKTN are the cause of muscular dystrophy-dystroglycanopathy congenital without mental retardation type B4 (MDDGB4) [MIM:613152]. An autosomal recessive disorder characterized by congenital muscular dystrophy and evidence of dystroglycanopathy. Features included increased serum creatine kinase, generalized weakness, mild white matter changes on brain MRI in some cases, and absence of mental retardation.
Defects in FKTN are the cause of muscular dystrophy-dystroglycanopathy limb-girdle type C4 (MDDGC4) [MIM:611588]. MDDGC4 is an autosomal recessive degenerative myopathy characterized by progressive weakness of the pelvic and shoulder girdle muscles and elevated serum creatine kinase. The severity of the disease depends on age at onset which may vary from early to late childhood or even adulthood. MDDGC4 is a novel form of LGMD2 and has no brain involvement and a remarkable clinical response to corticosteroids.
Defects in FKTN are the cause of cardiomyopathy dilated type 1X (CMD1X) [MIM:611615]; also called dilated cardiomyopathy with mild or no proximal muscle weakness. Dilated cardiomyopathy is a disorder characterized by ventricular dilation and impaired systolic function, resulting in congestive heart failure and arrhythmia. Patients are at risk of premature death. -
序列相似性
Belongs to the licD transferase family. -
细胞定位
Golgi apparatus membrane. - Information by UniProt
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数据库链接
- Entrez Gene: 2218 Human
- Entrez Gene: 246179 Mouse
- Entrez Gene: 362520 Rat
- Omim: 607440 Human
- SwissProt: O75072 Human
- SwissProt: Q8R507 Mouse
- Unigene: 55777 Human
- Unigene: 247210 Mouse
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别名
- CMD1X antibody
- FCMD antibody
- FCMD gene antibody
see all
图片
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All lanes : Anti-Fukutin antibody [EPR7913] (ab131280) at 1/1000 dilution
Lane 1 : HeLa cell lysate
Lane 2 : A549 cell lysate
Lane 3 : Fetal heart tissue lysate
Lane 4 : BxPC-3 cell lysate
Lysates/proteins at 10 µg per lane.
Secondary
All lanes : HRP labelled goat anti-rabbit at 1/2000 dilution
Predicted band size: 53 kDa -
Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-Fukutin antibody [EPR7913] (ab131280)
Immunohistochemical analysis of paraffin-embedded Human kidney tissue labelling Fukutin with ab131280 at 1/100 dilution.
Perform heat mediated antigen retrieval with citrate buffer pH 6 before commencing with IHC staining protocol.
数据表及文件
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SDS download
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Datasheet download
文献 (4)
ab131280 被引用在 4 文献中.
- Lesurf R et al. Whole genome sequencing delineates regulatory, copy number, and cryptic splice variants in early onset cardiomyopathy. NPJ Genom Med 7:18 (2022). PubMed: 35288587
- Pham TT et al. Characteristic expression of fukutin in gastric cancer among atomic bomb survivors. Oncol Lett 13:937-941 (2017). PubMed: 28356981
- Gerin I et al. ISPD produces CDP-ribitol used by FKTN and FKRP to transfer ribitol phosphate onto a-dystroglycan. Nat Commun 7:11534 (2016). PubMed: 27194101
- Oo HZ et al. Fukutin, identified by the Escherichia coli ampicillin secretion trap (CAST) method, participates in tumor progression in gastric cancer. Gastric Cancer 19:443-452 (2016). PubMed: 26223471