Elongator mutation in mice induces neurodegeneration and ataxia-like behavior

Elongator subunit 3 (ELP3) modifies ALS through tRNA modification

(2018) Andre Bento-Abreu et al.   HUMAN MOLECULAR GENETICS

Elongator and codon bias regulate protein levels in mammalian peripheral neurons

(2018) Joy Goffena et al.   Nature Communications

Loss of Elp3 Impairs the Acetylation and Distribution of Connexin-43 in the Developing Cerebral Cortex

(2017) Sophie Laguesse et al.   Frontiers in Cellular Neuroscience

Architecture of the yeast Elongator complex

(2016) Maria I Dauden et al.   EMBO REPORTS

Structural basis for tRNA modification by Elp3 from Dehalococcoides mccartyi

(2016) Sebastian Glatt et al.   NATURE STRUCTURAL & MOLECULAR BIOLOGY

Structural basis for tRNA modification by Elp3 from Dehalococcoides mccartyi

(2016) Sebastian Glatt et al.   NATURE STRUCTURAL & MOLECULAR BIOLOGY

Phosphatidylserine Ameliorates Neurodegenerative Symptoms and Enhances Axonal Transport in a Mouse Model of Familial Dysautonomia

(2016) Shiran Naftelberg et al.   PLoS Genetics

The Many Faces of Elongator in Neurodevelopment and Disease

(2016) Marija Kojic et al.   Frontiers in Molecular Neuroscience

ELP2is a novel gene implicated in neurodevelopmental disabilities

(2015) Julie S. Cohen et al.   AMERICAN JOURNAL OF MEDICAL GENETICS PART A

First effects of rising amyloid-β in transgenic mouse brain: synaptic transmission and gene expression

(2015) Damian M. Cummings et al.   BRAIN

NLRP3 inflammasome is associated with the response to IFN-β in patients with multiple sclerosis

(2015) Sunny Malhotra et al.   BRAIN

Optimization of Codon Translation Rates via tRNA Modifications Maintains Proteome Integrity

(2015) Danny D. Nedialkova et al.   CELL

A Dynamic Unfolded Protein Response Contributes to the Control of Cortical Neurogenesis

(2015) Sophie Laguesse et al.   DEVELOPMENTAL CELL

A small-molecule inhibitor of the NLRP3 inflammasome for the treatment of inflammatory diseases

(2015) Rebecca C Coll et al.   NATURE MEDICINE

Inflammasomes: mechanism of action, role in disease and therapeutics

(2015) Haitao Guo et al.   NATURE MEDICINE

Early activation of microglia and astrocytes in mouse models of spinocerebellar ataxia type 1

(2015) M. Cvetanovic et al.   NEUROSCIENCE

Cerebellar transcriptional alterations with Purkinje cell dysfunction and loss in mice lacking PGC-1α

(2015) Elizabeth K. Lucas et al.   Frontiers in Cellular Neuroscience

NLRP3 Inflammasome Is Expressed and Functional in Mouse Brain Microglia but Not in Astrocytes

(2015) Audrey Gustin et al.   PLoS One

Familial dysautonomia (FD) patients have reduced levels of the modified wobble nucleoside mcm5s2U in tRNA

(2014) Tony Karlsborn et al.   BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS

A neuron autonomous role for the familial dysautonomia gene ELP1 in sympathetic and sensory target tissue innervation

(2014) M. Z. Jackson et al.   DEVELOPMENT

Functional Properties and Projections of Neurons in the Medial Amygdala

(2014) S. Keshavarzi et al.   JOURNAL OF NEUROSCIENCE

Quantitative analysis of ribonucleoside modifications in tRNA by HPLC-coupled mass spectrometry

(2014) Dan Su et al.   Nature Protocols

Inflammasomes in the CNS

(2014) John G. Walsh et al.   NATURE REVIEWS NEUROSCIENCE

Disturbance of endoplasmic reticulum proteostasis in neurodegenerative diseases

(2014) Claudio Hetz et al.   NATURE REVIEWS NEUROSCIENCE

Structural insights into Elongator function

(2013) Sebastian Glatt et al.   CURRENT OPINION IN STRUCTURAL BIOLOGY

Caspase-1: The inflammasome and beyond

(2013) Gabriel Sollberger et al.   Innate Immunity

Self-propagation of pathogenic protein aggregates in neurodegenerative diseases

(2013) Mathias Jucker et al.   NATURE

Optimization of protein purification and characterization using Thermofluor screens

(2013) Stephane Boivin et al.   PROTEIN EXPRESSION AND PURIFICATION

A coordinated codon-dependent regulation of translation by Elongator

(2012) Fanelie Bauer et al.   CELL CYCLE

NLRP3 is activated in Alzheimer’s disease and contributes to pathology in APP/PS1 mice

(2012) Michael T. Heneka et al.   NATURE

The Elongator subcomplex Elp456 is a hexameric RecA-like ATPase

(2012) Sebastian Glatt et al.   NATURE STRUCTURAL & MOLECULAR BIOLOGY

MODOMICS: a database of RNA modification pathways—2013 update

(2012) Magdalena A. Machnicka et al.   NUCLEIC ACIDS RESEARCH

Brain regions and genes affecting limb-clasping responses

(2011) R. Lalonde et al.   BRAIN RESEARCH REVIEWS

Patterned Purkinje cell loss in the ataxic sticky mouse

(2011) Justyna R. Sarna et al.   EUROPEAN JOURNAL OF NEUROSCIENCE

Deep sequencing reveals 50 novel genes for recessive cognitive disorders

(2011) Hossein Najmabadi et al.   NATURE

Microglia in neurodegenerative disease

(2010) V. Hugh Perry et al.   Nature Reviews Neurology

Apoptosis inducing factor deficiency causes reduced mitofusion 1 expression and patterned Purkinje cell degeneration

(2010) Seung-Hyuk Chung et al.   NEUROBIOLOGY OF DISEASE

Locus ceruleus controls Alzheimer's disease pathology by modulating microglial functions through norepinephrine

(2010) M. T. Heneka et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

A Simple Composite Phenotype Scoring System for Evaluating Mouse Models of Cerebellar Ataxia

(2010) Stephan J. Guyenet et al.   Jove-Journal of Visualized Experiments

Centrotemporal sharp wave EEG trait in rolandic epilepsy maps to Elongator Protein Complex 4 (ELP4)

(2009) Lisa J Strug et al.   EUROPEAN JOURNAL OF HUMAN GENETICS

Protein structure prediction on the Web: a case study using the Phyre server

(2009) Lawrence A Kelley et al.   Nature Protocols

Variants of the elongator protein 3 ( ELP3 ) gene are associated with motor neuron degeneration

(2008) Claire L. Simpson et al.   HUMAN MOLECULAR GENETICS

Neuroinflammation mediated by IL-1β increases susceptibility of dopamine neurons to degeneration in an animal model of Parkinson's disease

(2008) James B Koprich et al.   Journal of Neuroinflammation

Microglial Dysfunction and Defective  -Amyloid Clearance Pathways in Aging Alzheimer's Disease Mice

(2008) S. E. Hickman et al.   JOURNAL OF NEUROSCIENCE

Loss of Mouse Ikbkap, a Subunit of Elongator, Leads to Transcriptional Deficits and Embryonic Lethality That Can Be Rescued by Human IKBKAP

(2008) Y.-T. Chen et al.   MOLECULAR AND CELLULAR BIOLOGY