Human ALS/FTD brain organoid slice cultures display distinct early astrocyte and targetable neuronal pathology

Genetic analysis of amyotrophic lateral sclerosis identifies contributing pathways and cell types

(2021) Sara Saez-Atienzar et al.   Science Advances

Reactive astrocyte nomenclature, definitions, and future directions

(2021) Carole Escartin et al.   NATURE NEUROSCIENCE

Neurodevelopmental defects and neurodegenerative phenotypes in human brain organoids carrying Parkinson’s disease-linked DNAJC6 mutations

(2021) Noviana Wulansari et al.   Science Advances

Cell stress in cortical organoids impairs molecular subtype specification

(2020) Aparna Bhaduri et al.   NATURE

Astrocyte Unfolded Protein Response Induces a Specific Reactivity State that Causes Non-Cell-Autonomous Neuronal Degeneration

(2020) Heather L. Smith et al.   NEURON

Synthetic Analyses of Single-Cell Transcriptomes from Multiple Brain Organoids and Fetal Brain

(2020) Yoshiaki Tanaka et al.   Cell Reports

Sliced Human Cortical Organoids for Modeling Distinct Cortical Layer Formation

(2020) Xuyu Qian et al.   Cell Stem Cell

Presynaptic Homeostasis Opposes Disease Progression in Mouse Models of ALS-Like Degeneration: Evidence for Homeostatic Neuroprotection

(2020) Brian O. Orr et al.   NEURON

Cellular complexity in brain organoids: Current progress and unsolved issues

(2020) Abed AlFatah Mansour et al.   SEMINARS IN CELL & DEVELOPMENTAL BIOLOGY

Knockout of reactive astrocyte activating factors slows disease progression in an ALS mouse model

(2020) Kevin A. Guttenplan et al.   Nature Communications

Drug GRADE: An Integrated Analysis of Population Growth and Cell Death Reveals Drug-Specific and Cancer Subtype-Specific Response Profiles

(2020) Hannah R. Schwartz et al.   Cell Reports

Quantitative patterns of motor cortex proteinopathy across ALS genotypes

(2020) Matthew Nolan et al.   Acta Neuropathologica Communications

C9orf72 in myeloid cells suppresses STING-induced inflammation

(2020) Madelyn E. McCauley et al.   NATURE

Deregulated expression of a longevity gene, Klotho, in the C9orf72 deletion mice with impaired synaptic plasticity and adult hippocampal neurogenesis

(2020) Wan Yun Ho et al.   Acta Neuropathologica Communications

Cross-Comparison of Human iPSC Motor Neuron Models of Familial and Sporadic ALS Reveals Early and Convergent Transcriptomic Disease Signatures

(2020) Ritchie Ho et al.   Cell Systems

Repeat‐associated non‐AUG translation in C9orf72‐ALS/FTD is driven by neuronal excitation and stress

(2019) Thomas Westergard et al.   EMBO Molecular Medicine

HR23B pathology preferentially co-localizes with p62, pTDP-43 and poly-GA in C9ORF72-linked frontotemporal dementia and amyotrophic lateral sclerosis

(2019) Frederike W. Riemslagh et al.   Acta Neuropathologica Communications

Cerebral organoids at the air–liquid interface generate diverse nerve tracts with functional output

(2019) Stefano L. Giandomenico et al.   NATURE NEUROSCIENCE

Motor neurons from ALS patients with mutations in C9ORF72 and SOD1 exhibit distinct transcriptional landscapes

(2019) Ching-On Wong et al.   HUMAN MOLECULAR GENETICS

Human 3D cellular model of hypoxic brain injury of prematurity

(2019) Anca M. Pașca et al.   NATURE MEDICINE

A Single-Cell Transcriptomic Atlas of Human Neocortical Development during Mid-gestation

(2019) Damon Polioudakis et al.   NEURON

The Role of Altered BDNF/TrkB Signaling in Amyotrophic Lateral Sclerosis

(2019) Jonu Pradhan et al.   Frontiers in Cellular Neuroscience

Extensive transcriptomic study emphasizes importance of vesicular transport in C9orf72 expansion carriers

(2019) Dennis W. Dickson et al.   Acta Neuropathologica Communications

Chloroquine inhibits autophagic flux by decreasing autophagosome-lysosome fusion

(2018) Mario Mauthe et al.   Autophagy

Amyotrophic lateral sclerosis (ALS) and Alzheimer’s disease (AD) are characterised by differential activation of ER stress pathways: focus on UPR target genes

(2018) L. Montibeller et al.   CELL STRESS & CHAPERONES

A single-cell RNA-seq survey of the developmental landscape of the human prefrontal cortex

(2018) Suijuan Zhong et al.   NATURE

Integrating single-cell transcriptomic data across different conditions, technologies, and species

(2018) Andrew Butler et al.   NATURE BIOTECHNOLOGY

Brain organoids get vascularized

(2018) Madeline A Lancaster   NATURE BIOTECHNOLOGY

scmap: projection of single-cell RNA-seq data across data sets

(2018) Vladimir Yu Kiselev et al.   NATURE METHODS

CellProfiler 3.0: Next-generation image processing for biology

(2018) Claire McQuin et al.   PLOS BIOLOGY

Reliability of human cortical organoid generation

(2018) Se-Jin Yoon et al.   NATURE METHODS

High prevalence of focal and multi-focal somatic genetic variants in the human brain

(2018) Michael J. Keogh et al.   Nature Communications

Transcriptional mechanism of IRF8 and PU.1 governs microglial activation in neurodegenerative condition

(2018) Nan Zhou et al.   Protein & Cell

C9orf72 poly GA RAN-translated protein plays a key role in amyotrophic lateral sclerosis via aggregation and toxicity

(2017) Youn-Bok Lee et al.   HUMAN MOLECULAR GENETICS

C9ORF72 hexanucleotide repeat exerts toxicity in a stable, inducible motor neuronal cell model, which is rescued by partial depletion of Pten

(2017) Matthew J. Stopford et al.   HUMAN MOLECULAR GENETICS

The DNA damage response (DDR) is induced by the C9orf72 repeat expansion in amyotrophic lateral sclerosis

(2017) Manal A. Farg et al.   HUMAN MOLECULAR GENETICS

Cell diversity and network dynamics in photosensitive human brain organoids

(2017) Giorgia Quadrato et al.   NATURE

Guided self-organization and cortical plate formation in human brain organoids

(2017) Madeline A Lancaster et al.   NATURE BIOTECHNOLOGY

KLF4-dependent perivascular cell plasticity mediates pre-metastatic niche formation and metastasis

(2017) Meera Murgai et al.   NATURE MEDICINE

SCENIC: single-cell regulatory network inference and clustering

(2017) Sara Aibar et al.   NATURE METHODS

C9orf72 expansion disrupts ATM-mediated chromosomal break repair

(2017) Callum Walker et al.   NATURE NEUROSCIENCE

Spatiotemporal gene expression trajectories reveal developmental hierarchies of the human cortex

(2017) Tomasz J. Nowakowski et al.   SCIENCE

A neuroprotective astrocyte state is induced by neuronal signal EphB1 but fails in ALS models

(2017) Giulia E. Tyzack et al.   Nature Communications

Guidelines for the use and interpretation of assays for monitoring autophagy (3rd edition)

(2016) Daniel J Klionsky et al.   Autophagy

Decoding ALS: from genes to mechanism

(2016) J. Paul Taylor et al.   NATURE

C9ORF72 poly(GA) aggregates sequester and impair HR23 and nucleocytoplasmic transport proteins

(2016) Yong-Jie Zhang et al.   NATURE NEUROSCIENCE

Topoisomerases and the regulation of neural function

(2016) Peter J. McKinnon   NATURE REVIEWS NEUROSCIENCE

Gene expression profiles and protein-protein interaction networks in amyotrophic lateral sclerosis patients with C9orf72 mutation

(2016) Meena Kumari Kotni et al.   Orphanet Journal of Rare Diseases

Semi-automated quantification of C9orf72 expansion size reveals inverse correlation between hexanucleotide repeat number and disease duration in frontotemporal degeneration

(2015) EunRan Suh et al.   ACTA NEUROPATHOLOGICA

Distinct brain transcriptome profiles in C9orf72-associated and sporadic ALS

(2015) Mercedes Prudencio et al.   NATURE NEUROSCIENCE

Partial restoration of protein synthesis rates by the small molecule ISRIB prevents neurodegeneration without pancreatic toxicity

(2015) M Halliday et al.   Cell Death & Disease

Aggregation-prone c9FTD/ALS poly(GA) RAN-translated proteins cause neurotoxicity by inducing ER stress

(2014) Yong-Jie Zhang et al.   ACTA NEUROPATHOLOGICA

Amyotrophic lateral sclerosis: a long preclinical period?

(2014) A. Eisen et al.   JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY

Poly-A Binding Protein-1 Localization to a Subset of TDP-43 Inclusions in Amyotrophic Lateral Sclerosis Occurs More Frequently in Patients Harboring an Expansion inC9orf72

(2014) Leeanne McGurk et al.   JOURNAL OF NEUROPATHOLOGY AND EXPERIMENTAL NEUROLOGY

Detecting Pairwise Correlations in Spike Trains: An Objective Comparison of Methods and Application to the Study of Retinal Waves

(2014) C. S. Cutts et al.   JOURNAL OF NEUROSCIENCE

Organogenesis in a dish: Modeling development and disease using organoid technologies

(2014) M. A. Lancaster et al.   SCIENCE

The neuropathology associated with repeat expansions in the C9ORF72 gene

(2013) Ian R. A. Mackenzie et al.   ACTA NEUROPATHOLOGICA

Cerebral organoids model human brain development and microcephaly

(2013) Madeline A. Lancaster et al.   NATURE

SIRT1 collaborates with ATM and HDAC1 to maintain genomic stability in neurons

(2013) Matthew M Dobbin et al.   NATURE NEUROSCIENCE

Polymerase chain reaction and Southern blot-based analysis of the C9orf72 hexanucleotide repeat in different motor neuron diseases

(2013) Annemarie Hübers et al.   NEUROBIOLOGY OF AGING

53BP1: pro choice in DNA repair

(2013) Michal Zimmermann et al.   TRENDS IN CELL BIOLOGY

Clinico-pathological features in amyotrophic lateral sclerosis with expansions in C9ORF72

(2012) Johnathan Cooper-Knock et al.   BRAIN

Dynamics of DNA damage response proteins at DNA breaks: a focus on protein modifications

(2011) S. E. Polo et al.   GENES & DEVELOPMENT

Selective Neuronal Vulnerability in Neurodegenerative Diseases: from Stressor Thresholds to Degeneration

(2011) Smita Saxena et al.   NEURON

Expanded GGGGCC Hexanucleotide Repeat in Noncoding Region of C9ORF72 Causes Chromosome 9p-Linked FTD and ALS

(2011) Mariely DeJesus-Hernandez et al.   NEURON

WGCNA: an R package for weighted correlation network analysis

(2008) Peter Langfelder et al.   BMC BIOINFORMATICS