(Q36521932)

14 August 2017

1 reference

title

The role of mitochondria in inherited neurodegenerative diseases (English)

1 reference

14 August 2017

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2

M Flint Beal

1 reference

14 August 2017

Jennifer Q Kwong

1

1 reference

PubMed ID

16805775

14 August 2017

Giovanni Manfredi

series ordinal

3

1 reference

PubMed ID

16805775

14 August 2017

language of work or name

English

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publication date

1 June 2006

1 reference

Journal of Neurochemistry

14 August 2017

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14 August 2017

97

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14 August 2017

6

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14 August 2017

1659-1675

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OPA1, encoding a dynamin-related GTPase, is mutated in autosomal dominant optic atrophy linked to chromosome 3q28

14 August 2017

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Mitochondrial targeting and a novel transmembrane arrest of Alzheimer's amyloid precursor protein impairs mitochondrial function in neuronal cells

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Sequence and organization of the human mitochondrial genome

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Presenilin-1 is located in rat mitochondria

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Membrane protein degradation by AAA proteases in mitochondria

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Loss of m-AAA protease in mitochondria causes complex I deficiency and increased sensitivity to oxidative stress in hereditary spastic paraplegia

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Mitochondrial DNA polymorphisms as risk factors for Parkinson's disease and Parkinson's disease dementia.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Regulation of mitochondrial iron accumulation by Yfh1p, a putative homolog of frataxin

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Mitofusin-2 determines mitochondrial network architecture and mitochondrial metabolism. A novel regulatory mechanism altered in obesity

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

p53 mediates cellular dysfunction and behavioral abnormalities in Huntington's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

High levels of mitochondrial DNA deletions in substantia nigra neurons in aging and Parkinson disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Leber hereditary optic neuropathy mtDNA mutations disrupt glutamate transport in cybrid cell lines.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Mitochondrial dysfunction due to mutant copper/zinc superoxide dismutase associated with amyotrophic lateral sclerosis is reversed by N-acetylcysteine.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Ubiquitin-proteasome system and Parkinson's diseases

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Chronic systemic pesticide exposure reproduces features of Parkinson's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Mutations in the DJ-1 gene associated with autosomal recessive early-onset parkinsonism

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

The role of mtDNA background in disease expression: a new primary LHON mutation associated with Western Eurasian haplogroup J.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Oxidative damage and metabolic dysfunction in Huntington's disease: selective vulnerability of the basal ganglia.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Mitochondrial abnormalities in Alzheimer brain: mechanistic implications

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Frataxin acts as an iron chaperone protein to modulate mitochondrial aconitase activity

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Altered metabolism of the amyloid beta precursor protein is associated with mitochondrial dysfunction in Down's syndrome

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Frataxin is reduced in Friedreich ataxia patients and is associated with mitochondrial membranes

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

The Parkinson's disease protein DJ-1 is neuroprotective due to cysteine-sulfinic acid-driven mitochondrial localization

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Pathogenic expression of homoplasmic mtDNA mutations needs a complex nuclear-mitochondrial interaction

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Bioenergetics shapes cellular death pathways in Leber's hereditary optic neuropathy: a model of mitochondrial neurodegeneration

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Expression of a Cu,Zn superoxide dismutase typical of familial amyotrophic lateral sclerosis induces mitochondrial alteration and increase of cytosolic Ca2+ concentration in transfected neuroblastoma SH-SY5Y cells

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Spastic paraplegia and OXPHOS impairment caused by mutations in paraplegin, a nuclear-encoded mitochondrial metalloprotease

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Beta-amyloid inhibits integrated mitochondrial respiration and key enzyme activities

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Mutant huntingtin aggregates impair mitochondrial movement and trafficking in cortical neurons

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Emerging functions of mammalian mitochondrial fusion and fission

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

The mitochondrial ND6 gene is a hot spot for mutations that cause Leber's hereditary optic neuropathy

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Mutant huntingtin directly increases susceptibility of mitochondria to the calcium-induced permeability transition and cytochrome c release

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Mutant huntingtin represses CBP, but not p300, by binding and protein degradation

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Truncated N-terminal fragments of huntingtin with expanded glutamine repeats form nuclear and cytoplasmic aggregates in cell culture.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Mitochondrial DNA deletions in human brain: regional variability and increase with advanced age

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Copper-dependent inhibition of human cytochrome c oxidase by a dimeric conformer of amyloid-beta1-42.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Neural mitochondrial Ca2+ capacity impairment precedes the onset of motor symptoms in G93A Cu/Zn-superoxide dismutase mutant mice

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Cell death: critical control points

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Parkin prevents mitochondrial swelling and cytochrome c release in mitochondria-dependent cell death.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Resistance of alpha -synuclein null mice to the parkinsonian neurotoxin MPTP.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Nuclear gene OPA1, encoding a mitochondrial dynamin-related protein, is mutated in dominant optic atrophy

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Mitochondrial encephalomyopathies: an update

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Sp1 and TAFII130 transcriptional activity disrupted in early Huntington's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Clinical and genetic abnormalities in patients with Friedreich's ataxia

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Reconstitution of gamma-secretase activity

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

The hereditary spastic paraplegias: nine genes and counting

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Antioxidant defences in cybrids harboring mtDNA mutations associated with Leber's hereditary optic neuropathy

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Deletion of the yeast homologue of the human gene associated with Friedreich's ataxia elicits iron accumulation in mitochondria.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Mitochondrial iron detoxification is a primary function of frataxin that limits oxidative damage and preserves cell longevity

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Juvenile Huntington chorea: Clinical, ultrastructural, and biochemical studies

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Alpha-synuclein and neurodegenerative diseases

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

The GAA*TTC triplet repeat expanded in Friedreich's ataxia impedes transcription elongation by T7 RNA polymerase in a length and supercoil dependent manner

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Mitochondrial pathology and apoptotic muscle degeneration in Drosophila parkin mutants

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Genetic and genomic studies of Drosophila parkin mutants implicate oxidative stress and innate immune responses in pathogenesis.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Hereditary spastic paraplegia SPG13 is associated with a mutation in the gene encoding the mitochondrial chaperonin Hsp60

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Nicastrin, presenilin, APH-1, and PEN-2 form active gamma-secretase complexes in mitochondria

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Idebenone and reduced cardiac hypertrophy in Friedreich's ataxia

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Rapid aggregate formation of the huntingtin N-terminal fragment carrying an expanded polyglutamine tract

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Abundant type 10 17 beta-hydroxysteroid dehydrogenase in the hippocampus of mouse Alzheimer's disease model

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

UCHL-1 is not a Parkinson's disease susceptibility gene

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

ALS-associated mutant SOD1G93A causes mitochondrial vacuolation by expansion of the intermembrane space and by involvement of SOD1 aggregation and peroxisomes

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Mutational analysis of action of mitochondrial fusion factor mitofusin-2.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

alpha-synuclein promotes mitochondrial deficit and oxidative stress

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Inhibition of the alpha-ketoglutarate dehydrogenase complex alters mitochondrial function and cellular calcium regulation

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

The crucial role of caspase-9 in the disease progression of a transgenic ALS mouse model

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Functional integrity of mitochondrial genomes in human platelets and autopsied brain tissues from elderly patients with Alzheimer's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Evidence for impairment of energy metabolism in vivo in Huntington's disease using localized 1H NMR spectroscopy

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Mitochondrial electron transport chain complex dysfunction in a transgenic mouse model for amyotrophic lateral sclerosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Transcriptional regulatory circuits controlling mitochondrial biogenesis and function

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Hypersensitivity of DJ-1-deficient mice to 1-methyl-4-phenyl-1,2,3,6-tetrahydropyrindine (MPTP) and oxidative stress.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Cytochrome c association with the inner mitochondrial membrane is impaired in the CNS of G93A-SOD1 mice.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Mutations in the parkin gene cause autosomal recessive juvenile parkinsonism

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Mice lacking alpha-synuclein are resistant to mitochondrial toxins

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Energy metabolism defects in Huntington's disease and effects of coenzyme Q10.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Mitochondrial DNA deletions are abundant and cause functional impairment in aged human substantia nigra neurons.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

ALS-linked Cu/Zn-SOD mutation increases vulnerability of motor neurons to excitotoxicity by a mechanism involving increased oxidative stress and perturbed calcium homeostasis

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Parkin enhances mitochondrial biogenesis in proliferating cells

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Mitochondrial cyclic AMP response element-binding protein (CREB) mediates mitochondrial gene expression and neuronal survival

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

The ubiquitin pathway in Parkinson's disease.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Interaction of Huntington disease protein with transcriptional activator Sp1

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

The importance of dendritic mitochondria in the morphogenesis and plasticity of spines and synapses

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

High aggregate burden of somatic mtDNA point mutations in aging and Alzheimer's disease brain

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Defects in adaptive energy metabolism with CNS-linked hyperactivity in PGC-1alpha null mice

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Toxicity of familial ALS-linked SOD1 mutants from selective recruitment to spinal mitochondria.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

High-throughput profiling of the mitochondrial proteome using affinity fractionation and automation

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Parkinsonism, premature menopause, and mitochondrial DNA polymerase gamma mutations: clinical and molecular genetic study.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

ABAD directly links Abeta to mitochondrial toxicity in Alzheimer's disease.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Mitochondria are a direct site of A beta accumulation in Alzheimer's disease neurons: implications for free radical generation and oxidative damage in disease progression

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Oligomycin induces a decrease in the cellular content of a pathogenic mutation in the human mitochondrial ATPase 6 gene.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Parkinson's disease alpha-synuclein transgenic mice develop neuronal mitochondrial degeneration and cell death.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Neuroprotective role of the Reaper-related serine protease HtrA2/Omi revealed by targeted deletion in mice

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Mutated human SOD1 causes dysfunction of oxidative phosphorylation in mitochondria of transgenic mice.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

The mtDNA T8993G (NARP) mutation results in an impairment of oxidative phosphorylation that can be improved by antioxidants

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Cellular localization and development of neuronal intranuclear inclusions in striatal and cortical neurons in R6/2 transgenic mice

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Mitochondrial dysfunction in a cell culture model of familial amyotrophic lateral sclerosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Mitochondrial complex I and IV activities in leukocytes from patients with parkin mutations.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Interactions between mitochondrial bioenergetics and cytoplasmic calcium in cultured cerebellar granule cells.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Inhibition of NADH-linked oxidation in brain mitochondria by 1-methyl-4-phenyl-pyridine, a metabolite of the neurotoxin, 1-methyl-4-phenyl-1,2,5,6-tetrahydropyridine.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

The m-AAA protease defective in hereditary spastic paraplegia controls ribosome assembly in mitochondria

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Oxidative damage is the earliest event in Alzheimer disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Subcellular distribution of superoxide dismutases (SOD) in rat liver: Cu,Zn-SOD in mitochondria

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Amyotrophic lateral sclerosis: a proposed mechanism

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

The human dynamin-related protein OPA1 is anchored to the mitochondrial inner membrane facing the inter-membrane space

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Endogenous oxidative stress in sporadic Alzheimer's disease neuronal cybrids reduces viability by increasing apoptosis through pro-death signaling pathways and is mimicked by oxidant exposure of control cybrids

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Mitochondrial dysfunction and oxidative damage in parkin-deficient mice

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Reduced platelet cytochrome c oxidase activity in Alzheimer's disease.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Amyotrophic lateral sclerosis-associated SOD1 mutant proteins bind and aggregate with Bcl-2 in spinal cord mitochondria

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Drosophila parkin mutants have decreased mass and cell size and increased sensitivity to oxygen radical stress

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Wild-type PINK1 prevents basal and induced neuronal apoptosis, a protective effect abrogated by Parkinson disease-related mutations

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Mutation in the alpha-synuclein gene identified in families with Parkinson's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Mouse models for Friedreich ataxia exhibit cardiomyopathy, sensory nerve defect and Fe-S enzyme deficiency followed by intramitochondrial iron deposits

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Peroxisome proliferator-activated receptor-gamma coactivator 1 alpha (PGC-1 alpha): transcriptional coactivator and metabolic regulator

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Mitochondrial DNA haplogroup cluster UKJT reduces the risk of PD.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Mutations associated with amyotrophic lateral sclerosis convert superoxide dismutase from an antiapoptotic gene to a proapoptotic gene: studies in yeast and neural cells

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Mutations in Cu/Zn superoxide dismutase gene are associated with familial amyotrophic lateral sclerosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Excitotoxicity hypothesis

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Sticky DNA: self-association properties of long GAA.TTC repeats in R.R.Y triplex structures from Friedreich's ataxia.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Oxidative stress in patients with Friedreich ataxia.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

HD CAG repeat implicates a dominant property of huntingtin in mitochondrial energy metabolism

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Friedreich ataxia: the oxidative stress paradox

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Familial Parkinson disease gene product, parkin, is a ubiquitin-protein ligase

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Mutant Cu/Zn-superoxide dismutase proteins have altered solubility and interact with heat shock/stress proteins in models of amyotrophic lateral sclerosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Inherent calcineurin inhibitor FKBP38 targets Bcl-2 to mitochondria and inhibits apoptosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Mitochondrial import and enzymatic activity of PINK1 mutants associated to recessive parkinsonism.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Friedreich ataxia mouse models with progressive cerebellar and sensory ataxia reveal autophagic neurodegeneration in dorsal root ganglia

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Leucine-rich repeat kinase 2 (LRRK2) interacts with parkin, and mutant LRRK2 induces neuronal degeneration

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Enhanced substantia nigra mitochondrial pathology in human alpha-synuclein transgenic mice after treatment with MPTP.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Mosaicism for a specific somatic mitochondrial DNA mutation in adult human brain

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Axonopathy and transport deficits early in the pathogenesis of Alzheimer's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Loss of function mutations in the gene encoding Omi/HtrA2 in Parkinson's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

A serine protease, HtrA2, is released from the mitochondria and interacts with XIAP, inducing cell death

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Biochemical abnormalities and excitotoxicity in Huntington's disease brain.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

DJ-1 has a role in antioxidative stress to prevent cell death

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Crystal structure of human DJ-1, a protein associated with early onset Parkinson's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

The mitochondrial DNA mutation at 8993 associated with NARP slows the rate of ATP synthesis in isolated lymphoblast mitochondria

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington's disease chromosomes.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Targeted disruption of hepatic frataxin expression causes impaired mitochondrial function, decreased life span and tumor growth in mice

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

A novel mitochondrial 12SrRNA point mutation in parkinsonism, deafness, and neuropathy.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Cytoplasmic transfer of the mtDNA nt 8993 T-->G (ATP6) point mutation associated with Leigh syndrome into mtDNA-less cells demonstrates cosegregation with a decrease in state III respiration and ADP/O ratio

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Hereditary early-onset Parkinson's disease caused by mutations in PINK1

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Mutant superoxide dismutase 1 forms aggregates in the brain mitochondrial matrix of amyotrophic lateral sclerosis mice.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Vesicle permeabilization by protofibrillar alpha-synuclein is sensitive to Parkinson's disease-linked mutations and occurs by a pore-like mechanism.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Mitochondrial Polymorphisms Significantly Reduce the Risk of Parkinson Disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Altered distribution of mitochondria impairs calcium homeostasis in rat hippocampal neurons in culture

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Interaction of presenilins with FKBP38 promotes apoptosis by reducing mitochondrial Bcl-2.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Parkinson's disease-associated mutations in leucine-rich repeat kinase 2 augment kinase activity

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Increased glutathione S-transferase activity rescues dopaminergic neuron loss in a Drosophila model of Parkinson's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

An adverse property of a familial ALS-linked SOD1 mutation causes motor neuron disease characterized by vacuolar degeneration of mitochondria

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

The Friedreich's ataxia mutation confers cellular sensitivity to oxidant stress which is rescued by chelators of iron and calcium and inhibitors of apoptosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

An intracellular protein that binds amyloid-beta peptide and mediates neurotoxicity in Alzheimer's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Inactivation of Drosophila DJ-1 leads to impairments of oxidative stress response and phosphatidylinositol 3-kinase/Akt signaling

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Mitochondrial localization of the Parkinson's disease related protein DJ-1: implications for pathogenesis

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Minocycline inhibits cytochrome c release and delays progression of amyotrophic lateral sclerosis in mice

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Mutations in LRRK2 cause autosomal-dominant parkinsonism with pleomorphic pathology

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

No mitochondrial haplotype was found to increase risk for Alzheimer's disease.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Mutations in the mitochondrial GTPase mitofusin 2 cause Charcot-Marie-Tooth neuropathy type 2A

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Axonal neuropathy with optic atrophy is caused by mutations in mitofusin 2

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

Frataxin deficiency in pancreatic islets causes diabetes due to loss of β cell mass

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03990.X

10.1111/J.1471-4159.2006.03990.X

21 January 2018

Identifiers

DOI

1 reference

14 August 2017

1 reference

PubMed ID

16805775