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Mitochondrial dysfunction in neurons in Friedreich's ataxia

Molecular and cellular neuroscience, 2020-01, Vol.102, p.103419-103419, Article 103419

2020

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Imaging neuronal activity in the central and peripheral nervous systems using new Thy1.2-GCaMP6 transgenic mouse lines

Journal of neuroscience methods, 2020-03, Vol.334, p.108535-108535, Article 108535

2020

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Mitochondrial dynamics and bioenergetic dysfunction is associated with synaptic alterations in mutant SOD1 motor neurons

The Journal of neuroscience, 2012-01, Vol.32 (1), p.229-242

2012

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The mitochondrial calcium regulator cyclophilin D is an essential component of oestrogen-mediated neuroprotection in amyotrophic lateral sclerosis

Brain (London, England : 1878), 2012-09, Vol.135 (Pt 9), p.2865-2874

2012

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Early cerebellar deficits in mitochondrial biogenesis and respiratory chain complexes in the KIKO mouse model of Friedreich ataxia

Disease models & mechanisms, 2017-11, Vol.10 (11), p.1343-1352

2017

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Abnormal mitochondrial transport and morphology are common pathological denominators in SOD1 and TDP43 ALS mouse models

Human molecular genetics, 2014-03, Vol.23 (6), p.1413-1424

2014

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The Age Lipid A2E and Mitochondrial Dysfunction Synergistically Impair Phagocytosis by Retinal Pigment Epithelial Cells

The Journal of biological chemistry, 2008-09, Vol.283 (36), p.24770-24780

2008

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Imaging of mitochondrial dynamics in motor and sensory axons of living mice

Methods in enzymology, 2014, Vol.547, p.97-110

2014

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Early VGLUT1-specific parallel fiber synaptic deficits and dysregulated cerebellar circuit in the KIKO mouse model of Friedreich ataxia

Disease models & mechanisms, 2017-12, Vol.10 (12), p.1529-1538

2017

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α-Synuclein is localized to mitochondria-associated ER membranes

The Journal of neuroscience, 2014-01, Vol.34 (1), p.249-259

2014

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PINK1-Dependent Recruitment of Parkin to Mitochondria in Mitophagy

Proceedings of the National Academy of Sciences - PNAS, 2010-01, Vol.107 (1), p.378-383

2010

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Mitochondrial function, morphology, and axonal transport in amyotrophic lateral sclerosis

Antioxidants & redox signaling, 2009-07, Vol.11 (7), p.1615

2009

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Lysyl-tRNA Synthetase Is a Target for Mutant SOD1 Toxicity in Mitochondria

The Journal of biological chemistry, 2008-10, Vol.283 (42), p.28321-28328

2008

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Correction: Early cerebellar deficits in mitochondrial biogenesis and respiratory chain complexes in the KIKO mouse model of Friedreich ataxia (doi: 10.1242/dmm.030502)

Disease models & mechanisms, 2018-01, Vol.11 (1)

2018

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In vivo pathogenic role of mutant SOD1 localized in the mitochondrial intermembrane space

The Journal of neuroscience, 2011-11, Vol.31 (44), p.15826-15837

2011

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Distinct intracellular sAC-cAMP domains regulate ER Ca2+ signaling and OXPHOS function

Journal of cell science, 2017-11, Vol.130 (21), p.3713-3727

2017

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Mutant SOD1 in neuronal mitochondria causes toxicity and mitochondrial dynamics abnormalities

Human molecular genetics, 2009-12, Vol.18 (23), p.4552-4564

2009

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SOD1 targeted to the mitochondrial intermembrane space prevents motor neuropathy in the Sod1 knockout mouse

Brain (London, England : 1878), 2011-01, Vol.134 (Pt 1), p.196-209

2011

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Isolation and culture of neurons and astrocytes from the mouse brain cortex

Methods in molecular biology (Clifton, N.J.), 2011, Vol.793, p.63

2011

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A glycine zipper motif mediates the formation of toxic β-amyloid oligomers in vitro and in vivo

Molecular neurodegeneration, 2011-08, Vol.6 (1), p.61-61, Article 61

2011

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PINK1 defect causes mitochondrial dysfunction, proteasomal deficit and alpha-synuclein aggregation in cell culture models of Parkinson's disease

PloS one, 2009-02, Vol.4 (2), p.e4597-e4597

2009

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Autosomal‐dominant Alzheimer's disease mutations at the same codon of amyloid precursor protein differentially alter Aβ production

Journal of neurochemistry, 2014-01, Vol.128 (2), p.330-339

2014

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Aβ42 generation is toxic to endothelial cells and inhibits eNOS function through an Akt/GSK-3β signaling-dependent mechanism

Neurobiology of aging, 2003-06, Vol.24 (3), p.437-451

2003

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adPEO mutations in ANT1 impair ADP-ATP translocation in muscle mitochondria

Human molecular genetics, 2011-08, Vol.20 (15), p.2964-2974

2011

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Internalized Antibodies to the Aβ Domain of APP Reduce Neuronal Aβ and Protect against Synaptic Alterations

The Journal of biological chemistry, 2007-06, Vol.282 (26), p.18895-18906

2007

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Mitochondrial dysfunction in neurons in Friedreich's ataxia

Imaging neuronal activity in the central and peripheral nervous systems using new Thy1.2-GCaMP6 transgenic mouse lines

Mitochondrial dynamics and bioenergetic dysfunction is associated with synaptic alterations in mutant SOD1 motor neurons

The mitochondrial calcium regulator cyclophilin D is an essential component of oestrogen-mediated neuroprotection in amyotrophic lateral sclerosis

Early cerebellar deficits in mitochondrial biogenesis and respiratory chain complexes in the KIKO mouse model of Friedreich ataxia

Abnormal mitochondrial transport and morphology are common pathological denominators in SOD1 and TDP43 ALS mouse models

The Age Lipid A2E and Mitochondrial Dysfunction Synergistically Impair Phagocytosis by Retinal Pigment Epithelial Cells

Imaging of mitochondrial dynamics in motor and sensory axons of living mice

Early VGLUT1-specific parallel fiber synaptic deficits and dysregulated cerebellar circuit in the KIKO mouse model of Friedreich ataxia

α-Synuclein is localized to mitochondria-associated ER membranes

PINK1-Dependent Recruitment of Parkin to Mitochondria in Mitophagy

Mitochondrial function, morphology, and axonal transport in amyotrophic lateral sclerosis

Lysyl-tRNA Synthetase Is a Target for Mutant SOD1 Toxicity in Mitochondria

Correction: Early cerebellar deficits in mitochondrial biogenesis and respiratory chain complexes in the KIKO mouse model of Friedreich ataxia (doi: 10.1242/dmm.030502)

In vivo pathogenic role of mutant SOD1 localized in the mitochondrial intermembrane space

Distinct intracellular sAC-cAMP domains regulate ER Ca2+ signaling and OXPHOS function

Mutant SOD1 in neuronal mitochondria causes toxicity and mitochondrial dynamics abnormalities

SOD1 targeted to the mitochondrial intermembrane space prevents motor neuropathy in the Sod1 knockout mouse

Isolation and culture of neurons and astrocytes from the mouse brain cortex

A glycine zipper motif mediates the formation of toxic β-amyloid oligomers in vitro and in vivo

PINK1 defect causes mitochondrial dysfunction, proteasomal deficit and alpha-synuclein aggregation in cell culture models of Parkinson's disease

Autosomal‐dominant Alzheimer's disease mutations at the same codon of amyloid precursor protein differentially alter Aβ production

Aβ42 generation is toxic to endothelial cells and inhibits eNOS function through an Akt/GSK-3β signaling-dependent mechanism

adPEO mutations in ANT1 impair ADP-ATP translocation in muscle mitochondria

Internalized Antibodies to the Aβ Domain of APP Reduce Neuronal Aβ and Protect against Synaptic Alterations

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