Comparison of independent screens on differentially vulnerable motor neurons reveals alpha-synuclein as a common modifier in motor neuron diseases

Vapb/Amyotrophic lateral sclerosis 8 knock-in mice display slowly progressive motor behavior defects accompanying ER stress and autophagic response

(2015) Frédérique Larroquette et al.   HUMAN MOLECULAR GENETICS

Cross-disease comparison of amyotrophic lateral sclerosis and spinal muscular atrophy reveals conservation of selective vulnerability but differential neuromuscular junction pathology

(2015) Laura H. Comley et al.   JOURNAL OF COMPARATIVE NEUROLOGY

Progesterone receptor signalling in retinal photoreceptor neuroprotection

(2015) Alice C. Wyse Jackson et al.   JOURNAL OF NEUROCHEMISTRY

Progesterone attenuates Aβ 25–35 -induced neuronal toxicity via JNK inactivation and progesterone receptor membrane component 1-dependent inhibition of mitochondrial apoptotic pathway

(2015) Yabin Qin et al.   JOURNAL OF STEROID BIOCHEMISTRY AND MOLECULAR BIOLOGY

Network Analyses Reveal Novel Aspects of ALS Pathogenesis

(2015) Mario Sanhueza et al.   PLoS Genetics

Transcriptional profiling of differentially vulnerable motor neurons at pre-symptomatic stage in the Smn 2b/- mouse model of spinal muscular atrophy

(2015) Lyndsay M. Murray et al.   Acta Neuropathologica Communications

The intriguing case of motor neuron disease: ALS and SMA come closer

(2015) Tilmann Achsel et al.   BIOCHEMICAL SOCIETY TRANSACTIONS

Defining the therapeutic window in a severe animal model of spinal muscular atrophy

(2014) K. L. Robbins et al.   HUMAN MOLECULAR GENETICS

Neuronal Matrix Metalloproteinase-9 Is a Determinant of Selective Neurodegeneration

(2014) Artem Kaplan et al.   NEURON

Increased levels of phosphoinositides cause neurodegeneration in a Drosophila model of amyotrophic lateral sclerosis

(2013) Stuart Forrest et al.   HUMAN MOLECULAR GENETICS

VAPB/ALS8 MSP Ligands Regulate Striated Muscle Energy Metabolism Critical for Adult Survival in Caenorhabditis elegans

(2013) Sung Min Han et al.   PLoS Genetics

Unravelling the enigma of selective vulnerability in neurodegeneration: motor neurons resistant to degeneration in ALS show distinct gene expression characteristics and decreased susceptibility to excitotoxicity

(2012) Alice Brockington et al.   ACTA NEUROPATHOLOGICA

Decreased stathmin expression ameliorates neuromuscular defects but fails to prolong survival in a mouse model of spinal muscular atrophy

(2012) Hsin-Lan Wen et al.   NEUROBIOLOGY OF DISEASE

Defects in neuromuscular junction remodelling in the Smn2B/− mouse model of spinal muscular atrophy

(2012) Lyndsay M. Murray et al.   NEUROBIOLOGY OF DISEASE

Direct central nervous system delivery provides enhanced protection following vector mediated gene replacement in a severe model of Spinal Muscular Atrophy

(2011) Jacqueline J. Glascock et al.   BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS

Global gene expression profiling of somatic motor neuron populations with different vulnerability identify molecules and pathways of degeneration and protection

(2011) Eva Hedlund et al.   BRAIN

Severe neuromuscular denervation of clinically relevant muscles in a mouse model of spinal muscular atrophy

(2011) K. K. Y. Ling et al.   HUMAN MOLECULAR GENETICS

Combination of SMN Trans-Splicing and a Neurotrophic Factor Increases the Life Span and Body Mass in a Severe Model of Spinal Muscular Atrophy

(2010) Monir Shababi et al.   HUMAN GENE THERAPY

Characterization of the Properties of a Novel Mutation in VAPB in Familial Amyotrophic Lateral Sclerosis

(2010) Han-Jou Chen et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Alpha-Synuclein Loss in Spinal Muscular Atrophy

(2010) Gyula Acsadi et al.   JOURNAL OF MOLECULAR NEUROSCIENCE

Increased Expression of α-Synuclein Reduces Neurotransmitter Release by Inhibiting Synaptic Vesicle Reclustering after Endocytosis

(2010) Venu M. Nemani et al.   NEURON

Genetics of motor neuron disorders: new insights into pathogenic mechanisms

(2009) Patrick A. Dion et al.   NATURE REVIEWS GENETICS

Coordinated Lipid Transfer between the Endoplasmic Reticulum and the Golgi Complex Requires the VAP Proteins and Is Essential for Golgi-mediated Transport

(2008) Diego Peretti et al.   MOLECULAR BIOLOGY OF THE CELL

Intravascular AAV9 preferentially targets neonatal neurons and adult astrocytes

(2008) Kevin D Foust et al.   NATURE BIOTECHNOLOGY

Natural history of SMA IIIb: Muscle strength decreases in a predictable sequence and magnitude

(2008) F. Deymeer et al.   NEUROLOGY