The role of mitochondria in inherited neurodegenerative diseases (Q36521932)

14 August 2017

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title

The role of mitochondria in inherited neurodegenerative diseases (English)

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14 August 2017

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2

M Flint Beal

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14 August 2017

Jennifer Q Kwong

1

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14 August 2017

Giovanni Manfredi

series ordinal

3

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14 August 2017

language of work or name

English

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publication date

1 June 2006

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Journal of Neurochemistry

14 August 2017

published in

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14 August 2017

volume

97

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14 August 2017

issue

6

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14 August 2017

page(s)

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

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Beta-amyloid inhibits integrated mitochondrial respiration and key enzyme activities

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21 January 2018

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

21 January 2018

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Cell death: critical control points

21 January 2018

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

21 January 2018

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

21 January 2018

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

21 January 2018

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21 January 2018

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

21 January 2018

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Clinical and genetic abnormalities in patients with Friedreich's ataxia

21 January 2018

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Reconstitution of gamma-secretase activity

21 January 2018

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The hereditary spastic paraplegias: nine genes and counting

21 January 2018

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

21 January 2018

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

21 January 2018

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21 January 2018

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21 January 2018

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21 January 2018

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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

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Mitochondrial pathology and apoptotic muscle degeneration in Drosophila parkin mutants

21 January 2018

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

21 January 2018

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

21 January 2018

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

21 January 2018

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Idebenone and reduced cardiac hypertrophy in Friedreich's ataxia

21 January 2018

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

21 January 2018

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

21 January 2018

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

21 January 2018

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

21 January 2018

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

21 January 2018

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alpha-synuclein promotes mitochondrial deficit and oxidative stress

21 January 2018

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

21 January 2018

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

21 January 2018

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

21 January 2018

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

21 January 2018

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

21 January 2018

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Transcriptional regulatory circuits controlling mitochondrial biogenesis and function

21 January 2018

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21 January 2018

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

21 January 2018

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Mutations in the parkin gene cause autosomal recessive juvenile parkinsonism

21 January 2018

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Mice lacking alpha-synuclein are resistant to mitochondrial toxins

21 January 2018

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

21 January 2018

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

21 January 2018

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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

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Parkin enhances mitochondrial biogenesis in proliferating cells

21 January 2018

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

21 January 2018

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The ubiquitin pathway in Parkinson's disease

21 January 2018

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Interaction of Huntington disease protein with transcriptional activator Sp1

21 January 2018

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

21 January 2018

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

21 January 2018

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

21 January 2018

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

21 January 2018

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

21 January 2018

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

21 January 2018

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21 January 2018

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21 January 2018

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

21 January 2018

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

21 January 2018

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

21 January 2018

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

21 January 2018

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

21 January 2018

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

21 January 2018

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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

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

21 January 2018

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Oxidative damage is the earliest event in Alzheimer disease

21 January 2018

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

21 January 2018

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Amyotrophic lateral sclerosis: a proposed mechanism

21 January 2018

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

21 January 2018

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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

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Mitochondrial dysfunction and oxidative damage in parkin-deficient mice

21 January 2018

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

21 January 2018

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

21 January 2018

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https://api.crossref.org/works/10.1111/J.1471-4159.2006.03990.X

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

21 January 2018

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

21 January 2018

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

21 January 2018

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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

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

21 January 2018

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Mitochondrial DNA haplogroup cluster UKJT reduces the risk of PD.

21 January 2018

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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

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https://api.crossref.org/works/10.1111/J.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/J.1471-4159.2006.03990.X

Excitotoxicity hypothesis

21 January 2018

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

21 January 2018

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Oxidative stress in patients with Friedreich ataxia.

21 January 2018

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https://api.crossref.org/works/10.1111/J.1471-4159.2006.03990.X

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

21 January 2018

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Friedreich ataxia: the oxidative stress paradox

21 January 2018

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1111/J.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

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

21 January 2018

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

21 January 2018

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

21 January 2018

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

21 January 2018

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

21 January 2018

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

21 January 2018

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

21 January 2018

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

21 January 2018

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

21 January 2018

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Biochemical abnormalities and excitotoxicity in Huntington's disease brain.

21 January 2018

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1111/J.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/J.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/J.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/J.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/J.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/J.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/J.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/J.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/J.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/J.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/J.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/J.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/J.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/J.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/J.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/J.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/J.1471-4159.2006.03990.X

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1111/J.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/J.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/J.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/J.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/J.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/J.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/J.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/J.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/J.1471-4159.2006.03990.X

Comparative biochemical studies in fibroblasts from patients with different forms of Leigh syndrome

21 January 2018

1 reference

9 November 2024

https://opencitations.net/index/api/v1/citations/10.1007/BF01799347

Identifiers

DOI

10.1111/J.1471-4159.2006.03990.X

1 reference

14 August 2017

1 reference