Inherited neurodegenerative diseases: the one-hit model of neurodegeneration.

No abstract is available for this paper.

• Publication year

  2001

• Venue

  Human Molecular Genetics

• Publication date

  2001-10-01

• Fields of study

  Biology, Medicine

• Identifiers
  DOI 10.1093/HMG/10.20.2269PMID 11673410
• 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.

• addendum: A one-hit model of cell death in inherited neuronal degenerations

  2001cited by this paper
• Gene therapy restores vision in a canine model of childhood blindness

  2001cited by this paper
• Cause of neural death in neurodegenerative diseases attributable to expansion of glutamine repeats

  2001cited by this paper
• Oxidative stress in Alzheimer's disease.

  2000cited by this paper
• Neuronal cell death in Huntington's disease: a potential role for dopamine.

  2000cited by this paper
• A one-hit model of cell death in inherited neuronal degenerations

  2000cited by this paper
• Neurodegeneration: One-hit neuronal death

  2000cited by this paper
• Recent advances in the molecular basis of inherited photoreceptor degeneration

  2000cited by this paper
• Rom-1 is required for rod photoreceptor viability and the regulation of disk morphogenesis

  2000cited by this paper
• Oxidative stress in amyotrophic lateral sclerosis

  2000cited by this paper
• Rate of caudate atrophy in presymptomatic and symptomatic stages of Huntington's disease

  2000cited by this paper
• Molecular genetics of human retinal disease.

  1999cited by this paper
• Translating cell biology into therapeutic advances in Alzheimer's disease

  1999cited by this paper
• Light, Ca2+, and photoreceptor death: new evidence for the equivalent-light hypothesis from arrestin knockout mice.

  1999cited by this paper
• Mechanisms of photoreceptor death and survival in mammalian retina.

  1999cited by this paper
• New order from neurological disorders

  1999cited by this paper
• Non-Cell-Autonomous Photoreceptor Degeneration in rdsMutant Mice Mosaic for Expression of a Rescue Transgene

  1998influential reference
• Mechanisms of cell death in the inherited retinal degenerations.

  1998cited by this paper
• Histopathology of the human retina in retinitis pigmentosa.

  1998cited by this paper
• Rate of neuronal fallout in a transsynaptic cerebellar model.

  1998cited by this paper
• Inducible apoptosis-promoting activity in retinal cell-conditioned medium.

  1997cited by this paper
• Transgenic mice carrying the dominant rhodopsin mutation P347S: evidence for defective vectorial transport of rhodopsin to the outer segments.

  1996cited by this paper
• Support for the equivalent light hypothesis for RP

  1995cited by this paper
• Apoptosis in Retinitis Pigmentosa

  1995cited by this paper
• On the probabilistic nature of excitotoxic neuronal death in hippocampal neurons.

  1995cited by this paper
• Apoptotic photoreceptor cell death in mouse models of retinitis pigmentosa.

  1994cited by this paper
• Is idiopathic parkinsonism the consequence of an event or a process?

  1994cited by this paper
• A mathematical model of pathogenesis in idiopathic parkinsonism.

  1994cited by this paper
• Retinitis pigmentosa. The Friedenwald Lecture.

  1993cited by this paper
• Apoptosis: Final common pathway of photoreceptor death in rd, rds, and mutant mice

  1993cited by this paper
• Apoptosis: final common pathway of photoreceptor death in rd, rds, and rhodopsin mutant mice.

  1993cited by this paper
• Cellular interactions implicated in the mechanism of photoreceptor degeneration in transgenic mice expressing a mutant rhodopsin gene.

  1993influential reference
• Photoreceptor degeneration in vitamin A deprivation and retinitis pigmentosa: the equivalent light hypothesis.

  1993cited by this paper
• Oxidative stress, glutamate, and neurodegenerative disorders.

  1993cited by this paper
• The extent and time course of motoneuron involvement in amyotrophic lateral sclerosis

  1991influential reference
• Ageing and Parkinson's disease: substantia nigra regional selectivity.

  1991cited by this paper
• The extent and time course of motoneuron involvement in amyotrophic lateral sclerosis.

  1991cited by this paper
• First order dynamics of visual field loss in retinitis pigmentosa

  1990cited by this paper

• Global Perspectives on Myopia and Pathologic Myopia: From Environmental Drivers to Precision Medicine.

  2025cites this paper
• Long-term natural history of ellipsoid zone width in USH2A-retinopathy

  2024influential citation
• Exploring Patterns of Human Mortality and Aging: A Reliability Theory Viewpoint

  2024cites this paper
• Aggregation of rhodopsin mutants in mouse models of autosomal dominant retinitis pigmentosa

  2024cites this paper
• Repeated Low-Level Red-Light Therapy for Controlling Onset and Progression of Myopia-a Review

  2023cites this paper
• A Mathematical Model for On-Target Off-Tumor Effect of CAR-T Cells on Gliomas

  2022cites this paper
• Characterizing Visual Fields in RPGR Related Retinitis Pigmentosa Using Octopus Static-Automated Perimetry

  2022cites this paper
• Mathematical Models of Retinitis Pigmentosa: The Trophic Factor Hypothesis

  2021cites this paper
• Near Infrared (NIR) Light Therapy of Eye Diseases: A Review

  2021cites this paper
• Predictive Mathematical Models for the Spread and Treatment of Hyperoxia-induced Photoreceptor Degeneration in Retinitis Pigmentosa.

  2018cites this paper
• Potential Therapeutic Benefits of Riluzole in Pre-Symptomatic Familial Amyotrophic Lateral Sclerosis

  2017cites this paper
• What causes amyotrophic lateral sclerosis?

  2017cites this paper
• Calcium dynamics change in degenerating cone photoreceptors.

  2016cites this paper
• University of Birmingham Mathematical and computational models of the retina in health, development and disease

  2016influential citation
• Stem Cell Therapy for the Central Nervous System in Lysosomal Storage Diseases

  2016cites this paper
• Mathematical and computational models of the retina in health, development and disease.

  2016influential citation
• Progression of Vision Loss in Macular Telangiectasia Type 2.

  2015cites this paper
• Photoreceptor Degeneration: Molecular Mechanisms of Photoreceptor Degeneration

  2014cites this paper
• Rare Individual Amyloid-β Oligomers Act on Astrocytes to Initiate Neuronal Damage

  2014cites this paper
• The Role of the Statistical Method of Motor Unit Number Estimation (MUNE) to Assess the Potential Therapeutic Benefits of Riluzole on Patients with Pre-symptomatic Familial Amyotrophic Lateral Sclerosis

  2013cites this paper
• Parkinson-Syndrom(e) – Neue Konzepte für eine sich ausbreitende Erkrankung?

  2013cites this paper
• Chapter 40 – Retinitis Pigmentosa and Allied Disorders

  2013cites this paper
• Changes in Motor Unit Loss and Axonal Regeneration Rate in Sporadic and Familiar Amyotrophic Lateral Sclerosis (ALS) — Possible Different Pathogenetic Mechanisms?

  2013cites this paper
• Blinded by the UV light: how the focus on transcription-coupled NER has distracted from understanding the mechanisms of Cockayne syndrome neurologic disease.

  2013cites this paper
• Protein Aggregation and Fibrillogenesis in Cerebral and Systemic Amyloid Disease

  2012cites this paper
• Modeling the polyglutamine aggregation pathway in Huntington's disease: from basic studies to clinical applications.

  2012cites this paper
• Quantitative connection between polyglutamine aggregation kinetics and neurodegenerative process in patients with Huntington’s disease

  2012cites this paper
• BDNF may play a differential role in the protective effect of the mGluR2/3 agonist LY379268 on striatal projection neurons in R6/2 Huntington's disease mice.

  2012cites this paper
• Rates of Decline in Alzheimer Disease Decrease with Age

  2012cites this paper
• Protection of Motor Neurons in Pre-Symptomatic Individuals Carrying SOD 1 Mutations: Results of Motor Unit Number Estimation (MUNE) Electrophysiology

  2012cites this paper
• Photobiomodulation protects the retina from light-induced photoreceptor degeneration.

  2011cites this paper
• Differential Motor Neuron Impairment and Axonal Regeneration in Sporadic and Familiar Amyotrophic Lateral Sclerosis with SOD-1 Mutations: Lessons from Neurophysiology

  2011cites this paper
• Photoreceptor degeneration, azoospermia, leukoencephalopathy, and abnormal RPE cell function in mice expressing an early stop mutation in CLCN2.

  2010cites this paper
• Biological clues on neuronal degeneration based on theoretical fits of decay patterns: towards a mathematical neuropathology.

  2010cites this paper
• Biomarkers of disease in a case of familial lower motor neuron ALS

  2010cites this paper
• A comparison of visual field sensitivity to photoreceptor thickness in retinitis pigmentosa.

  2010cites this paper
• One-hit stochastic decline in a mechanochemical model of cytoskeleton-induced neuron death III: diffusion pulse death zones.

  2009cites this paper
• Detection of pre-clinical motor unit loss in familial amyotrophic lateral sclerosis.

  2009cites this paper
• Recent clinical findings with memantine should not mean that the idea of neuroprotection in glaucoma is abandoned

  2009cites this paper
• Nucleation of protein aggregation kinetics as a basis for genotype-phenotype correlations in polyglutamine diseases

  2009cites this paper
• Characterization of a canine model of autosomal recessive retinitis pigmentosa due to a PDE6A mutation.

  2009cites this paper
• One-hit Stochastic Decline in a Mechanochemical Model of Cytoskeleton-induced Neuron Death

  2009cites this paper
• The spectrum of retinal dystrophies caused by mutations in the peripherin/RDS gene.

  2008cites this paper
• Neonatal Neuronal Circuitry Shows Hyperexcitable Disturbance in a Mouse Model of the Adult-Onset Neurodegenerative Disease Amyotrophic Lateral Sclerosis

  2008cites this paper
• One-hit stochastic decline in a mechanochemical model of cytoskeleton-induced neuron death II: transition state metastability.

  2007cites this paper
• A Universal Mechanism Ties Genotype to Phenotype in Trinucleotide Diseases

  2007cites this paper
• One-hit stochastic decline in a mechanochemical model of cytoskeleton-induced neuron death I: cell-fate arrival times.

  2007cites this paper
• A frameshift mutation in RPGR exon ORF15 causes photoreceptor degeneration and inner retina remodeling in a model of X-linked retinitis pigmentosa.

  2006cites this paper
• Models of Systems Failure in Aging

  2006cites this paper
• THE ROLE OF THE TAM FAMILY OF RECEPTOR PTKS IN RETINAL HOMEOSTASIS

  2006cites this paper
• SLCO/OATP-like Transport of Glutathione in FasL-induced Apoptosis

  2006cites this paper
• Chapter 17 – Retinitis Pigmentosa and Allied Disorders

  2006cites this paper
• Chapter 1 – Reliability Theory of Aging and Longevity

  2005cites this paper
• Scale-free neurodegeneration: cellular heterogeneity and the stretched exponential kinetics of cell death.

  2005cites this paper
• Heterogeneous cellular environments modulate one-hit neuronal death kinetics.

  2005cites this paper
• Programmed cell deaths

  2004cites this paper
• Lifespan and mitochondrial control of neurodegeneration

  2004cites this paper
• Programmed cell deaths. Apoptosis and alternative deathstyles.

  2004cites this paper
• Alternative Programs of Cell Death in Developing Retinal Tissue*

  2003cites this paper
• Frailty models based on Lévy processes

  2003cites this paper
• Progress toward understanding the genetic and biochemical mechanisms of inherited photoreceptor degenerations.

  2003cites this paper
• The quest for a general theory of aging and longevity.

  2003cites this paper
• Photoreceptor death: Spatiotemporal patterns arising from one-hit death kinetics and a diffusible cell death factor

  2002cites this paper
• Antioxidants as treatment for neurodegenerative disorders

  2002cites this paper
• The cellular fate of mutant rhodopsin: quality control, degradation and aggresome formation.

  2002cites this paper
• Detection of preclinical motor neurone loss in SOD1 mutation carriers using motor unit number estimation

  2002cites this paper
• Exploring the formation of Alzheimer's disease senile plaques in silico.

  2002cites this paper
• The reliability theory of aging and longevity.

  2001cites this paper