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Molecular pathways of motor neuron injury in amyotrophic lateral sclerosis

Nature reviews. Neurology, 2011-11, Vol.7 (11), p.616-630

2011

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Mutations in CHMP2B in lower motor neuron predominant amyotrophic lateral sclerosis (ALS)

PloS one, 2010-03, Vol.5 (3), p.e9872-e9872

2010

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The C9orf72 protein interacts with Rab1a and the ULK1 complex to regulate initiation of autophagy

The EMBO journal, 2016-08, Vol.35 (15), p.1656-1676

2016

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Axonal Transport Defects in a Mitofusin 2 Loss of Function Model of Charcot-Marie-Tooth Disease in Zebrafish

PloS one, 2013-06, Vol.8 (6), p.e67276-e67276

2013

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Role of Axonal Transport in Neurodegenerative Diseases

Annual review of neuroscience, 2008-01, Vol.31 (1), p.151-173

2008

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Systemic delivery of scAAV9 expressing SMN prolongs survival in a model of spinal muscular atrophy

Science translational medicine, 2010-06, Vol.2 (35), p.35ra42

2010

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The effect of SOD1 mutation on cellular bioenergetic profile and viability in response to oxidative stress and influence of mutation-type

PloS one, 2013-06, Vol.8 (6), p.e68256

2013

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Superoxide dismutase 1 mutation in a cellular model of amyotrophic lateral sclerosis shifts energy generation from oxidative phosphorylation to glycolysis

Neurobiology of aging, 2014-06, Vol.35 (6), p.1499-1509

2014

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Use of zebrafish models to investigate rare human disease

Journal of medical genetics, 2018-10, Vol.55 (10), p.641-649

2018

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Mitochondrial Function and Actin Regulate Dynamin-Related Protein 1-Dependent Mitochondrial Fission

Current biology, 2005-04, Vol.15 (7), p.678-683

2005

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Familial amyotrophic lateral sclerosis-linked SOD1 mutants perturb fast axonal transport to reduce axonal mitochondria content

Human molecular genetics, 2007-11, Vol.16 (22), p.2720-2728

2007

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An interaction between synapsin and C9orf72 regulates excitatory synapses and is impaired in ALS/FTD

Acta neuropathologica, 2022-09, Vol.144 (3), p.437-464

2022

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S[+] Apomorphine is a CNS penetrating activator of the Nrf2-ARE pathway with activity in mouse and patient fibroblast models of amyotrophic lateral sclerosis

Free radical biology & medicine, 2013-08, Vol.61, p.438-452

2013

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Neutrophils use selective autophagy receptor Sqstm1/p62 to target Staphylococcus aureus for degradation in vivo in zebrafish

Autophagy, 2021-06, Vol.17 (6), p.1448-1457

2021

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Altered age-related changes in bioenergetic properties and mitochondrial morphology in fibroblasts from sporadic amyotrophic lateral sclerosis patients

Neurobiology of aging, 2015-10, Vol.36 (10), p.2893-2903

2015

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Lamb1a regulates atrial growth by limiting second heart field addition during zebrafish heart development

Development (Cambridge), 2021-10, Vol.148 (20)

2021

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C9orf72 plays a central role in Rab GTPase-dependent regulation of autophagy

Small GTPases, 2018-09, Vol.9 (5), p.399-408

2018

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Discovery of Western European R1b1a2 Y chromosome variants in 1000 genomes project data: an online community approach

PloS one, 2012-07, Vol.7 (7), p.e41634-e41634

2012

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Loss of TMEM106B exacerbates C9ALS/FTD DPR pathology by disrupting autophagosome maturation

Frontiers in cellular neuroscience, 2022-12, Vol.16, p.1061559

2022

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Tardbpl splicing rescues motor neuron and axonal development in a mutant tardbp zebrafish

Human molecular genetics, 2013-06, Vol.22 (12), p.2376-2386

2013

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Direct evidence for axonal transport defects in a novel mouse model of mutant spastin-induced hereditary spastic paraplegia (HSP) and human HSP patients

Journal of neurochemistry, 2009-07, Vol.110 (1), p.34-44

2009

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AAV9-mediated central nervous system-targeted gene delivery via cisterna magna route in mice

Molecular therapy. Methods & clinical development, 2016-01, Vol.3 (C), p.15055-15055, Article 15055

2016

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Impairment of the tRNA-splicing endonuclease subunit 54 (tsen54) gene causes neurological abnormalities and larval death in zebrafish models of pontocerebellar hypoplasia

Human molecular genetics, 2011-04, Vol.20 (8), p.1574-1584

2011

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Adipose-derived stem cells protect motor neurons and reduce glial activation in both in vitro and in vivo models of ALS

Molecular therapy. Methods & clinical development, 2021-06, Vol.21, p.413-433

2021

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The GLP-1 receptor agonist, liraglutide, fails to slow disease progression in SOD1G93A and TDP-43Q331K transgenic mouse models of ALS

Scientific reports, 2021-08, Vol.11 (1), p.17027-17027, Article 17027

2021

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Molecular pathways of motor neuron injury in amyotrophic lateral sclerosis

Mutations in CHMP2B in lower motor neuron predominant amyotrophic lateral sclerosis (ALS)

The C9orf72 protein interacts with Rab1a and the ULK1 complex to regulate initiation of autophagy

Axonal Transport Defects in a Mitofusin 2 Loss of Function Model of Charcot-Marie-Tooth Disease in Zebrafish

Role of Axonal Transport in Neurodegenerative Diseases

Systemic delivery of scAAV9 expressing SMN prolongs survival in a model of spinal muscular atrophy

The effect of SOD1 mutation on cellular bioenergetic profile and viability in response to oxidative stress and influence of mutation-type

Superoxide dismutase 1 mutation in a cellular model of amyotrophic lateral sclerosis shifts energy generation from oxidative phosphorylation to glycolysis

Use of zebrafish models to investigate rare human disease

Mitochondrial Function and Actin Regulate Dynamin-Related Protein 1-Dependent Mitochondrial Fission

Familial amyotrophic lateral sclerosis-linked SOD1 mutants perturb fast axonal transport to reduce axonal mitochondria content

An interaction between synapsin and C9orf72 regulates excitatory synapses and is impaired in ALS/FTD

S[+] Apomorphine is a CNS penetrating activator of the Nrf2-ARE pathway with activity in mouse and patient fibroblast models of amyotrophic lateral sclerosis

Neutrophils use selective autophagy receptor Sqstm1/p62 to target Staphylococcus aureus for degradation in vivo in zebrafish

Altered age-related changes in bioenergetic properties and mitochondrial morphology in fibroblasts from sporadic amyotrophic lateral sclerosis patients

Lamb1a regulates atrial growth by limiting second heart field addition during zebrafish heart development

C9orf72 plays a central role in Rab GTPase-dependent regulation of autophagy

Discovery of Western European R1b1a2 Y chromosome variants in 1000 genomes project data: an online community approach

Loss of TMEM106B exacerbates C9ALS/FTD DPR pathology by disrupting autophagosome maturation

Tardbpl splicing rescues motor neuron and axonal development in a mutant tardbp zebrafish

Direct evidence for axonal transport defects in a novel mouse model of mutant spastin-induced hereditary spastic paraplegia (HSP) and human HSP patients

AAV9-mediated central nervous system-targeted gene delivery via cisterna magna route in mice

Impairment of the tRNA-splicing endonuclease subunit 54 (tsen54) gene causes neurological abnormalities and larval death in zebrafish models of pontocerebellar hypoplasia

Adipose-derived stem cells protect motor neurons and reduce glial activation in both in vitro and in vivo models of ALS

The GLP-1 receptor agonist, liraglutide, fails to slow disease progression in SOD1G93A and TDP-43Q331K transgenic mouse models of ALS

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