Depletion of WFS1 compromises mitochondrial function in hiPSC-derived neuronal models of Wolfram syndrome [0.03%]
WFS1的耗竭损害 wolfram 综合征诱导型 pluricaneral 细胞系衍生的神经元模型中的线粒体功能
Malgorzata Zatyka,Tatiana R Rosenstock,Congxin Sun et al.
Malgorzata Zatyka et al.
Mitochondrial dysfunction involving mitochondria-associated ER membrane (MAM) dysregulation is implicated in the pathogenesis of late-onset neurodegenerative diseases, but understanding is limited for rare early-onset conditions. Loss of th...
Cellular and electrophysiological characterization of triadin knockout syndrome using induced pluripotent stem cell-derived cardiomyocytes [0.03%]
利用诱导多能干细胞源性心肌细胞表征三聚体蛋白缺乏综合症的细胞及电生理学特征
Daniel J Clemens,Dan Ye,Lili Wang et al.
Daniel J Clemens et al.
Triadin knockout syndrome (TKOS) is a malignant arrhythmia disorder caused by recessive null variants in TRDN-encoded cardiac triadin. Induced pluripotent stem cell-derived cardiomyocytes (iPSC-CMs) were generated from two unrelated TKOS pa...
Neuronal silence as a prosurvival factor for adult-born olfactory bulb interneurons [0.03%]
神经元沉默作为成年出生嗅球间神经元的促存活因素
Xin Su,Yury Kovalchuk,Nima Mojtahedi et al.
Xin Su et al.
Adult-born cells, arriving daily into the rodent olfactory bulb, either integrate into the neural circuitry or get eliminated. However, whether these two populations differ in their morphological or functional properties remains unclear. Us...
Generation, genomic characterization, and differentiation of triploid human embryonic stem cells [0.03%]
人三倍体胚胎干细胞系的建系、基因组特征及分化能力研究
Guy Haim-Abadi,Tamar Golan-Lev,Amnon Koren et al.
Guy Haim-Abadi et al.
Humans are diploid organisms, and triploidy in human embryos is responsible for ∼10% of spontaneous miscarriages. Surprisingly, some pregnancies proceed to triploid newborns that suffer from many neuro-developmental disorders. To investiga...
IRX5 promotes DNA damage repair and activation of hair follicle stem cells [0.03%]
IRX5促进DNA损伤修复及毛乳头细胞活化
Jefferson K Chen,Julie Wiedemann,Ly Nguyen et al.
Jefferson K Chen et al.
The molecular mechanisms allowing hair follicles to periodically activate their stem cells (HFSCs) are incompletely characterized. Here, we identify the transcription factor IRX5 as a promoter of HFSC activation. Irx5-/- mice have delayed a...
AAV-mediated gene augmentation therapy of CRB1 patient-derived retinal organoids restores the histological and transcriptional retinal phenotype [0.03%]
利用AAV介导的基因矫正疗法修复CRB1视网膜类器官患者的视网膜转录和组织表型
Nanda Boon,Xuefei Lu,Charlotte A Andriessen et al.
Nanda Boon et al.
Retinitis pigmentosa and Leber congenital amaurosis are inherited retinal dystrophies that can be caused by mutations in the Crumbs homolog 1 (CRB1) gene. CRB1 is required for organizing apical-basal polarity and adhesion between photorecep...
Organotypic human lung bud microarrays identify BMP-dependent SARS-CoV-2 infection in lung cells [0.03%]
人源肺芽类器官微阵列鉴定出BMP依赖性的SARS-CoV-2肺细胞感染
E A Rosado-Olivieri,B Razooky,J Le Pen et al.
E A Rosado-Olivieri et al.
Although lung disease is the primary clinical outcome in COVID-19 patients, how SARS-CoV-2 induces lung pathology remains elusive. Here we describe a high-throughput platform to generate self-organizing and commensurate human lung buds deri...
Human iPSC-derived neural progenitor cells secreting GDNF provide protection in rodent models of ALS and retinal degeneration [0.03%]
人诱导多能干细胞衍生的分泌GDNF的神经前体细胞可保护 ALS和视网膜变性动物模型
Alexander H Laperle,V Alexandra Moser,Pablo Avalos et al.
Alexander H Laperle et al.
Human induced pluripotent stem cells (iPSCs) are a renewable cell source that can be differentiated into neural progenitor cells (iNPCs) and transduced with glial cell line-derived neurotrophic factor (iNPC-GDNFs). The goal of the current s...
Organoids from mouse molar and incisor as new tools to study tooth-specific biology and development [0.03%]
小鼠磨牙和门牙类器官——研究牙齿特异性生物学和发育的新工具
Florian Hermans,Lara Hemeryck,Celine Bueds et al.
Florian Hermans et al.
Organoid models provide powerful tools to study tissue biology and development in a dish. Presently, organoids have not yet been developed from mouse tooth. Here, we established tooth organoids (TOs) from early-postnatal mouse molar and inc...
Neuro-mesodermal assembloids recapitulate aspects of peripheral nervous system development in vitro [0.03%]
神经-中胚层组装体可再现外周神经系统发育的某些方面
Anna F Rockel,Nicole Wagner,Peter Spenger et al.
Anna F Rockel et al.
Here we describe a novel neuro-mesodermal assembloid model that recapitulates aspects of peripheral nervous system (PNS) development such as neural crest cell (NCC) induction, migration, and sensory as well as sympathetic ganglion formation...