Increased apoptosis of Huntington disease lymphoblasts associated with repeat length-dependent mitochondrial depolarization

No abstract is available for this paper.

• Publication year

  1999

• Venue

  Nature Medicine

• Publication date

  1999-10-01

• Fields of study

  Biology, Medicine

• Identifiers
  DOI 10.1038/13518PMID 10502825
• External record

  Open on Semantic Scholar

• Source metadata

  Semantic Scholar, PubMed

• No claims are published for this paper.

• No concepts are published for this paper.

• [Mitochondria and apoptosis].

  2000cited by this paper
• Inhibition of caspase-1 slows disease progression in a mouse model of Huntington's disease

  1999cited by this paper
• Caspase-8 is required for cell death induced by expanded polyglutamine repeats.

  1999cited by this paper
• Biochemical abnormalities and excitotoxicity in Huntington's disease brain

  1999cited by this paper
• Nuclear and Neuropil Aggregates in Huntington’s Disease: Relationship to Neuropathology

  1999cited by this paper
• In vitro evidence for both the nucleus and cytoplasm as subcellular sites of pathogenesis in Huntington's disease.

  1999cited by this paper
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  1998cited by this paper
• Huntingtin acts in the nucleus to induce apoptosis but death does not correlate with the formation of intranuclear inclusions.

  1998cited by this paper
• Reduced apoptosis and cytochrome c-mediated caspase activation in mice lacking caspase 9.

  1998cited by this paper
• The mitochondrial death/life regulator in apoptosis and necrosis.

  1998cited by this paper
• Mitochondria and apoptosis.

  1998cited by this paper
• Ataxin-1 nuclear localization and aggregation: role in polyglutamine-induced disease in SCA1 transgenic mice.

  1998cited by this paper
• Intranuclear neuronal inclusions in Huntington's disease and dentatorubral and pallidoluysian atrophy: correlation between the density of inclusions and IT15 CAG triplet repeat length.

  1998cited by this paper
• Prevention of Apoptosis by Bcl-2: Release of Cytochrome c from Mitochondria Blocked

  1997cited by this paper
• Aberrant expression of bcl-2 gene family in Down's syndrome brains.

  1997cited by this paper
• Mitochondrial function in Huntington's disease: Clues for pathogenesis and prospects for treatment

  1997cited by this paper
• Energy metabolism defects in Huntington's disease and effects of coenzyme Q10

  1997cited by this paper
• Glyceraldehyde-3-phosphate dehydrogenase: nuclear translocation participates in neuronal and nonneuronal cell death.

  1997cited by this paper
• Formation of neuronal intranuclear inclusions underlies the neurological dysfunction in mice transgenic for the HD mutation.

  1997cited by this paper
• Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neurites in brain.

  1997cited by this paper
• Oxidative damage and metabolic dysfunction in Huntington's disease: Selective vulnerability of the basal ganglia

  1997cited by this paper
• Interactions of Cyclophilin with the Mitochondrial Inner Membrane and Regulation of the Permeability Transition Pore, a Cyclosporin A-sensitive Channel (*)

  1996cited by this paper
• Huntington's disease: translating a CAG repeat into a pathogenic mechanism

  1996cited by this paper
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  1996cited by this paper
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  1996cited by this paper
• Mitochondrial defect in Huntington's disease caudate nucleus

  1996cited by this paper
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  1995cited by this paper
• Reduction in mitochondrial potential constitutes an early irreversible step of programmed lymphocyte death in vivo

  1995cited by this paper
• Glutamate-induced neuronal death: a succession of necrosis or apoptosis depending on mitochondrial function.

  1995cited by this paper
• Expression analysis of the ataxin–1 protein in tissues from normal and spinocerebellar ataxia type 1 individuals

  1995cited by this paper
• Immunophilins and nervous system

  1995cited by this paper
• Huntington's disease gene (IT15) is widely expressed in human and rat tissues.

  1993cited by this paper
• Cryopreservation of whole blood and isolated lymphocytes for B-cell immortalization

  1993cited by this paper
• Neurochemical and histologic characterization of striatal excitotoxic lesions produced by the mitochondrial toxin 3-nitropropionic acid

  1993cited by this paper
• Peripheral type benzodiazepine receptors.

  1989cited by this paper
• The peripheral-type benzodiazepine receptor. Localization to the mitochondrial outer membrane.

  1986cited by this paper

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  2018cites this paper
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  2018cites this paper
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  2018cites this paper
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