无胸腺症关键蛋白6样抗体
规格：1mg/1ml
英文名： DGCR6L
别名： DGC6L_HUMAN; DGCR6L; DiGeorge syndrome critical region 6-like protein; Protein DGCR6L.
分子量： 25kDa
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 DGCR6L
交叉反应：Human, Mouse, Rat, Cow, Rabbit,
细胞定位：细胞核
无胸腺症关键蛋白6样抗体产品介绍：background: Neural crest cell migration to the third and fourth pharyngeal pouches is a critical step in the structural formation of organs that are affected in DiGeorge syndrome. DGCR6 (DiGeorge syndrome critical region 6) is a nuclear protein that plays a role in neural crest cell migration and is located at the DiGeorge syndrome critical region (DGCR) on chromosome 22. Expressed ubiquitously with highest levels in heart, liver and skeletal muscle, DGCR6 shares high homology with the Drosophila gonadal (gdl) protein and with human Laminin-1, both of which are involved in early tissue development. The gene encoding DGCR6, along with other DGCR genes, is deleted in DiGeorge syndrome; a developmental disorder characterized by improper facial, cardiac and palate formation. Upregulation of DGCR6 is implicated in无胸腺症关键蛋白6样抗体 lung and colon adenocarcinomas, as well as in Burkitt’s lymphoma and lymphocytes transformed by EBV. Due to a duplication of the ancestral DGCR6 locus, there are two functional, highly homologous copies of the DGCR6 gene (designated DGCR6 and DGCR6L) on chromosome 22. Function: May play a role in neural crest cell migration into the third and fourth pharyngeal pouches. Subcellular Location: Nucleus. Predominantly nuclear. Tissue Specificity: Widely expressed in fetal and ***** tissues. Highest expression in liver, heart and skeletal muscle. Lower levels in pancreas and placenta. Weak expression in brain. Similarity: Belongs to the gonadal family. Database links: Entrez Gene: 85359 Human Omim: 无胸腺症关键蛋白6样抗体609459 Human SwissProt: Q9BY27 Human Unigene: 410965 Human Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. DiGeorge’syndrome为先天性胸腺发育**所致原发性T细胞缺陷病，严重低钙和主动脉弓畸形。 DiGeorge’syndrome为非遗传无家族性。由于胚胎期第三、第四咽囊发育障碍，使胸腺和甲状旁腺缺如或发育不全而引起先天性异常。患儿常伴其他先天性畸形本综合征是多基因遗传性**，但染色体22q11区域缺失是主要原因，发病机制不很清楚，可能与**内的一种变异有关。
无胸腺症关键蛋白6样抗体产品应用：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：肿瘤 细胞生物 **学 神经生物学
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid