跨膜蛋白43抗体
规格:1mg/1ml
英文名: TMEM43
别名: ARVC5; ARVD5; EDMD7; LUMA; Protein LUMA; Tmem43; TMM43_HUMAN; Transmembrane protein 43,
分子量: 45kDa
储存液:0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型:Polyclonal
亚型:IgG
纯化方法:affinity purified by Protein A
**原:KLH conjugated synthetic peptide derived from human TMEM43
交叉反应:Human, Mouse, Rat,
细胞定位:细胞核
跨膜蛋白43抗体产品介绍:background: This gene belongs to the TMEM43 family. Defects in this gene are the cause of familial arrhythmogenic right ventricular dysplasia type 5 (ARVD5), also known as arrhythmogenic right ventricular cardiomyopathy type 5 (ARVC5). Arrhythmogenic right ventricular dysplasia is an inherited disorder, often involving both ventricles, and is characterized by ventricular tachycardia, heart failure, sudden cardiac death, and fibrofatty replacement of cardiomyocytes. This gene contains a response element for PPAR gamma (an adipogenic transcription factor), which may explain the fibrofatty replacement of the myocardium, a characteristic pathological finding in ARVC. [provided by RefSeq, Oct 2008] Function: May have an important role in maintaining nuclear envelope structure by organizing protein complexes at the inner nuclear membrane. Required for retaining emerin at the inner nuclear membrane. Subcellular Location: Endoplasmic reticulum. Nucleus inner 跨膜蛋白43抗体membrane. Retained in the inner nuclear membrane through interaction with EMD and A-and B-lamins. The N- and C-termini are orientated towards the nucleoplasm. The majority of the hydrophilic domain resides in the endoplasmic reticulum lumen. Tissue Specificity: Highest expression in placenta. Also found at lower levels in heart, ovary, spleen, small intestine, thymus, prostate and testis. DISEASE: Defects in TMEM43 are the cause of familial arrhythmogenic right ventricular dysplasia type 5 (ARVD5) [MIM:604400]; also known as arrhythmogenic right ventricular cardiomyopathy (ARVC5). ARVD is an autosomal dominant disease characterized by partial degeneration of the myocardium of the right ventricle, electrical instability, and sudden death. It is clinically defined by electrocardiographic and angiographic criteria; pathologic findings, replacement of跨膜蛋白43抗体 ventricular myocardium with fatty and fibrous elements, preferentially involve the right ventricular free wall. Similarity: Belongs to the TMEM43 family. Database links: Entrez Gene: 79188 Human Entrez Gene: 74122 Mouse Entrez Gene: 362401 Rat Omim: 612048 Human SwissProt: Q9BTV4 Human SwissProt: Q9DBS1 Mouse SwissProt: Q5XIP9 Rat Unigene: 517817 Human Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
跨膜蛋白43抗体产品应用: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.
研究领域:心血管 细胞生物 染色质和核信号 信号转导
储存条件: Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源: Rabbit
外观: Lyophilized or Liquid