产品编号Rs-9542R 英文名称C7orf27 中文名称7号染色体开放阅读框27抗体 别 名BAAT 1; BAAT1; BRAT-1; brat1; BRAT1_HUMAN; BRCA1-associated ATM activator 1; BRCA1-associated protein required for ATM activation protein 1; BRCA1-associated protein required for ATM activation-1; C7orf27; chromosome 7 open reading frame 27; HEAT repeat containing protein C7orf27. 说 明 书0.2ml 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, 产品应用WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 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88kDa 细胞定位细胞核 性 状Lyophilized or Liquid 浓 度1mg/1ml 免 疫 原KLH conjugated synthetic peptide derived from human BAAT1/C7orf27 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. PubMedPubMed 产品介绍Function: Required for activation of ATM following ionizing radiation. May act by regulating dephosphorylation of ATM.
Subunit: Interacts with BRCA1 ａnd ATM.
Subcellular Location: Nucleus. Present at double strand breaks (DSBs) following ionizing radiation treatment.
Tissue Specificity: Ubiquitously expressed.
DISEASE: Defects in BRAT1 are the cause of rigidity ａnd multifocal seizure syndrome, lethal neonatal (RMFSL) [MIM:614498]. A lethal, neonatal, neurologic disorder characterized by episodic jerking that is apparent in utero, lack of psychomotor development, axial ａnd limb rigidity, frequent multifocal seizures, ａnd dysautonomia. At birth, affected individuals have small heads, overlapping cranial sutures, small or absent fontanels, ａnd depressed frontal bones. Infants show poorly responsive focal jerks of the tongue, face ａnd arms in a nearly continuous sequence throughout life.
Similarity: Contains 2 HEAT repeats.
Database links: Entrez Gene: 221927 Human Entrez Gene: 231841 Mouse SwissProt: Q6PJG6 Human SwissProt: Q8C3R1 Mouse SwissProt: D3ZUM1 Rat Unigene: 520623 Human Unigene: 271932 Mouse
Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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