Mutations in the Heterotopia Gene Eml1/EML1 Severely Disrupt the Formation of Primary Cilia
CELL REPORTS
Authors: Uzquiano, Ana; Cifuentes-Diaz, Carmen; Jabali, Ammar; Romero, Delfina M.; Houllier, Anne; Dingli, Florent; Maillard, Camille; Boland, Anne; Deleuze, Jean-Francois; Loew, Damarys; Mancini, Grazia M. S.; Bahi-Buisson, Nadia; Ladewig, Julia; Francis, Fiona
Abstract
Apical radial glia (aRGs) are predominant progenitors during corticogenesis. Perturbing their function leads to cortical malformations, including subcortical heterotopia (SH), characterized by the presence of neurons below the cortex. EML1/Eml1 mutations lead to SH in patients, as well as to heterotopic cortex (HeCo) mutant mice. In HeCo mice, some aRGs are abnormally positioned away from the ventricular zone (VZ). Thus, unraveling EML1/Eml1 function will clarify mechanisms maintaining aRGs in the VZ. We pinpoint an unknown EML1/Eml1 function in primary cilium formation. In HeCo aRGs, cilia are shorter, less numerous, and often found aberrantly oriented within vesicles. Patient fibroblasts and human cortical progenitors show similar defects. EML1 interacts with RPGRIP1L, a ciliary protein, and RPGRIP1L mutations were revealed in a heterotopia patient. We also identify Golgi apparatus abnormalities in EML1/Eml1 mutant cells, potentially upstream of the cilia phenotype. We thus reveal primary cilia mechanisms impacting aRG dynamics in physiological and pathological conditions.
High performance red organic electroluminescent devices based on a trivalent iridium complex with stepwise energy levels
RSC ADVANCES
Authors: Li, Yanan; Zhou, Liang; Cui, Rongzhen; Jiang, Yunlong; Zhao, Xuesen; Liu, Weiqiang; Zhu, Qi; Cui, Yingjie; Zhang, Hongjie
Abstract
In this work, electroluminescent (EL) devices with double light-emitting layers (EMLs) having stepwise energy levels were designed and fabricated to improve the EL performances of the red light-emitting trivalent iridium complex bis(2-methyldibenzo[f,h] quinoxaline)(acetylacetonate) iridium(III) [Ir(MDQ)(2)(acac)]. To broaden the recombination zone and facilitate the balance of carriers on emitter molecules, the widely used p-type material 4,40,400-tri(N-carbazolyl) triphenylamine (TcTa) and bipolar material 2,6-bis(3-(9H-carbazol-9-yl) phenyl) pyridine (26DCzPPy) were chosen as host materials of EML1 and EML2, respectively due to their well matched energy levels. Interestingly, slight decomposition of Ir(MDQ)(2)(acac) molecules was observed during the deposition of EML, which causes the rapidly decreased brightness at relatively high doping concentration. Finally, a high performance red EL device with maximum current efficiency of 44.76 cd A(-1), power efficiency of 40.19 lm W-1, and external quantum efficiency (EQE) of 15.5% was obtained by optimizing the doping concentration of Ir(MDQ)(2)(acac). Even at a high brightness of 1000 cd m(-2) (5.2 V), a current efficiency as high as 40.59 cd A(-1) (EQE-14.4%) can still be retained by the same device.