• Publications
  • Influence
Mitochondrial dysfunction and oxidative stress in neurodegenerative diseases
TLDR
Treatments targeting basic mitochondrial processes, such as energy metabolism or free-radical generation, or specific interactions of disease-related proteins with mitochondria hold great promise in ageing-related neurodegenerative diseases. Expand
Dopamine neurons derived from human ES cells efficiently engraft in animal models of Parkinson's disease.
TLDR
A novel floor-plate-based strategy for the derivation of human DA neurons that efficiently engraft in vivo is presented, suggesting that past failures were due to incomplete specification rather than a specific vulnerability of the cells. Expand
Mitochondrial pathology and muscle and dopaminergic neuron degeneration caused by inactivation of Drosophila Pink1 is rescued by Parkin.
TLDR
It is shown that inhibition of Drosophila Pink1 function results in energy depletion, shortened lifespan, and degeneration of select indirect flight muscles and dopaminergic neurons, and the level of Parkin protein is significantly reduced in dPink1 RNA interference animals. Expand
Reduced-median-network analysis of complete mitochondrial DNA coding-region sequences for the major African, Asian, and European haplogroups.
TLDR
This work used reduced-median-network approaches to analyze 560 complete European, Asian, and African mtDNA coding-region sequences from unrelated individuals to develop a more complete understanding of sequence diversity both within and between haplogroups. Expand
Mitochondria take center stage in aging and neurodegeneration
  • M. Beal
  • Biology, Medicine
  • Annals of neurology
  • 1 October 2005
TLDR
There is strong evidence from genetics and transgenic mouse models that mitochondrial dysfunction results in neurodegeneration and may contribute to the pathogenesis of Alzheimer’s disease, Parkinson's disease, Huntington's Disease, amyotrophic lateral sclerosis, hereditary spastic paraplegia, and cerebellar degenerations. Expand
Mutated Human SOD1 Causes Dysfunction of Oxidative Phosphorylation in Mitochondria of Transgenic Mice*
TLDR
The findings suggest that G93A-mutated hSOD1 in mitochondria may cause mitochondrial defects, which contribute to precipitating the neurodegenerative process in motor neurons. Expand
Neural subtype specification of fertilization and nuclear transfer embryonic stem cells and application in parkinsonian mice
TLDR
A set of coculture conditions is provided that allows rapid and efficient derivation of most central nervous system phenotypes and transplantation of ES and ntES cell–derived dopaminergic neurons corrected the phenotype of a mouse model of Parkinson disease, demonstrating an in vivo application of therapeutic cloning in neural disease. Expand
Neurochemical and histologic characterization of striatal excitotoxic lesions produced by the mitochondrial toxin 3-nitropropionic acid
TLDR
The effects of intrastriatal, subacute systemic, or chronic systemic administration of the mitochondrial toxin 3-nitropropionic acid in rats showed consistent with in vitro studies showing that mild metabolic compromise can selectively activate NMDA receptors while more severe compromise activates both NMDA and non-NMDA receptors. Expand
Functional engraftment of human ES cell–derived dopaminergic neurons enriched by coculture with telomerase-immortalized midbrain astrocytes
TLDR
The utility of recreating the cellular environment of the developing human midbrain while driving dopaminergic neurogenesis from HES cells is shown, and the potential of the resultant cells to mediate substantial functional recovery in a model of Parkinson disease is demonstrated. Expand
Pink1 regulates mitochondrial dynamics through interaction with the fission/fusion machinery
TLDR
It is shown that Pink1, a mitochondria-targeted Ser/Thr kinase linked to familial PD, genetically interacts with the mitochondrial fission/fusion machinery and modulates mitochondrial dynamics and is established as a paradigm for PD research. Expand
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