Patterned cerebellar Purkinje cell death in a transgenic mouse model of Niemann Pick type A/B disease

No abstract is available for this paper.

• Publication year

  2001

• Venue

  European Journal of Neuroscience

• Publication date

  2001-05-01

• Fields of study

  Biology, Medicine

• Identifiers
  DOI 10.1046/j.0953-816x.2001.01564.xPMID 11403680
• 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.

• Aldolase C/zebrin II and the regionalization of the cerebellum

  2007cited by this paper
• Early sequential formation of functional GABAA and glutamatergic synapses on CA1 interneurons of the rat foetal hippocampus

  2002cited by this paper
• Expression of heat‐shock protein Hsp25 in mouse purkinje cells during development reveals novel features of cerebellar compartmentation

  2001influential reference
• Pattern formation in the cerebellar cortex.

  2000cited by this paper
• Constitutive expression of the 25‐kDa heat shock protein Hsp25 reveals novel parasagittal bands of Purkinje cells in the adult mouse cerebellar cortex

  2000influential reference
• Transverse zones in the vermis of the mouse cerebellum

  1999cited by this paper
• Ceramide Signaling Downstream of the p75 Neurotrophin Receptor Mediates the Effects of Nerve Growth Factor on Outgrowth of Cultured Hippocampal Neurons

  1999cited by this paper
• SIMULTANEOUS IDENTIFICATION OF TEMPERATURE PROFILE AND WALL EMISSIVITIES IN ONE-DIMENSIONAL SEMITRANSPARENT MEDIUM BY INVERSE RADIATION ANALYSIS

  1999cited by this paper
• Different Dystrophin-like Complexes Are Expressed in Neurons and Glia

  1999cited by this paper
• Advances in the signal transduction of ceramide and related sphingolipids.

  1999cited by this paper
• Spatial correspondence between tactile projection patterns and the distribution of the antigenic Purkinje cell markers anti-zebrin I and anti-zebrin II in the cerebellar folium crus IIA of the rat.

  1999cited by this paper
• Ceramide and Its Interconvertible Metabolite Sphingosine Function as Indispensable Lipid Factors Involved in Survival and Dendritic Differentiation of Cerebellar Purkinje Cells

  1998cited by this paper
• Genomic sequences of aldolase C (Zebrin II) direct lacZ expression exclusively in non-neuronal cells of transgenic mice.

  1998cited by this paper
• Neurodegeneration in Lurcher mice caused by mutation in δ2 glutamate receptor gene

  1997cited by this paper
• Stripes and zones: the origins of regionalization of the adult cerebellum.

  1997cited by this paper
• An anatomical model of cerebellar modules.

  1997cited by this paper
• Chapter 3 An anatomical model of cerebellar modules

  1997cited by this paper
• Absence epilepsy in tottering mutant mice is associated with calcium channel defects.

  1996influential reference
• Acid sphingomyelinase-deficient mice mimic the neurovisceral form of human lysosomal storage disease (Niemann-Pick disease).

  1995cited by this paper
• Acid sphingomyelinase deficient mice: a model of types A and B Niemann–Pick disease

  1995influential reference
• External cuneocerebellar projection and Purkinje cell zebrin II bands: a direct comparison of parasagittal banding in the mouse cerebellum.

  1994cited by this paper
• Topography of Purkinje cell compartments and mossy fiber terminal fields in lobules II and III of the rat cerebellar cortex: spinocerebellar and cuneocerebellar projections.

  1994influential reference
• Lack of Purkinje cell loss in adult rat cerebellum following protracted axotomy: Degenerative changes and regenerative attempts of the severed axons

  1994cited by this paper
• The cloning of zebrin II reveals its identity with aldolase C.

  1994cited by this paper
• Functional and antigenic maps in the rat cerebellum: Zebrin compartmentation and vibrissal receptive fields in lobule IXa

  1994cited by this paper
• Effects of nervous mutation on Purkinje cell compartments defined by Zebrin II and 9-O-acetylated gangliosides expression.

  1994cited by this paper
• Parasagittal compartmentation of adult rat Purkinje cells expressing the low-affinity nerve growth factor receptor: changes of pattern expression after a traumatic lesion.

  1994cited by this paper
• Purkinje cell loss from alternating sagittal zones in the cerebellum of leaner mutant mice.

  1994cited by this paper
• Cell interactions underlying Purkinje cell replacement by neural grafting in the pcd mutant cerebellum.

  1993cited by this paper
• Antigenic compartmentation in the mouse cerebellar cortex: Zebrin and HNK‐1 reveal a complex, overlapping molecular topography

  1993cited by this paper
• Degeneration of Purkinje cells in parasagittal zones of the cerebellar vermis after treatment with ibogaine or harmaline.

  1993cited by this paper
• Purkinje Cell Heterogeneity: Its Role in Organizing the Topography of the Cerebellar Cortex Connections

  1992cited by this paper
• Zebrins: Molecular Markers of Compartmentation in the Cerebellum

  1992cited by this paper
• Novel developmental boundary in the cerebellum revealed by zebrin expression in the Lurcher (Lc/+) mutant mouse

  1992cited by this paper
• Compartmentation in mammalian cerebellum: Zebrin II and P-path antibodies define three classes of sagittally organized bands of Purkinje cells.

  1992cited by this paper
• The modular cerebellum.

  1991cited by this paper
• Parasagittal organization of the rat cerebellar cortex: Direct comparison of purkinje cell compartments and the organization of the spinocerebellar projection

  1990cited by this paper
• Zebrin II: A polypeptide antigen expressed selectively by purkinje cells reveals compartments in rat and fish cerebellum

  1990influential reference
• Development of parasagittal zonation in the rat cerebellar cortex: MabQ113 antigenic bands are created postnatally by the suppression of antigen expression in a subset of Purkinje cells

  1988cited by this paper
• Effects of the murine mutation ‘nervous’ on neurons in cerebellum and dorsal cochlear nucleus

  1988cited by this paper
• Parasagittal organization of the rat cerebellar cortex: Direct correlation between antigenic purkinje cell bands revealed by mabQ113 and the organization of the olivocerebellar projection

  1987cited by this paper
• Cerebellar mutations affecting the postnatal survival of Purkinje cells in the mouse disclose a longitudinal pattern of differentially sensitive cells.

  1987cited by this paper
• Embryonic and adult neurons interact to allow Purkinje cell replacement in mutant cerebellum

  1987cited by this paper
• Antigenic map of the rat cerebellar cortex: The distribution of parasagittal bands as revealed by monoclonal anti‐purkinje cell antibody mabQ113

  1987cited by this paper
• Harmaline-induced changes in plasma membrane of Purkinje cells: a trans-synaptic effect mediated by climbing fibers.

  1986cited by this paper
• Anatomy of cerebellar Purkinje cells in the rat determined by a specific immunohistochemical marker.

  1984cited by this paper
• Immunohistochemical localization of calcium-binding protein in the cerebellum, hippocampal formation and olfactory bulb of the rat.

  1982cited by this paper
• Cerebellar cell degeneration in the leaner mutant mouse.

  1982cited by this paper
• Structural and quantitative studies on the normal C3H and Lurcher mutant mouse.

  1979cited by this paper

• Cerebellar Purkinje cell stripe patterns reveal a differential vulnerability and resistance to cell loss during normal aging in mice

  2025cites this paper
• Cerebellar Purkinje cell stripe patterns reveal a differential vulnerability and resistance to cell loss during normal aging in mice

  2025cites this paper
• Autonomous STING signaling in Purkinje cells drives neurodegeneration independent of type I interferon

  2025cites this paper
• Retention of lysosomal acid sphingomyelinase protects from Niemann-Pick Disease

  2025cites this paper
• Dysregulation of zebrin-II cell subtypes in the cerebellum is a shared feature across polyglutamine ataxia mouse models and patients.

  2024cites this paper
• The Niemann-Pick type diseases - A synopsis of inborn errors in sphingolipid and cholesterol metabolism.

  2023cites this paper
• Lobe X of the Cerebellum: A Natural Neuro-Resistant Region

  2023cites this paper
• Spinal muscular atrophy-like phenotype in a mouse model of acid ceramidase deficiency

  2023cites this paper
• Region‐specific preservation of Purkinje cell morphology and motor behavior in the ATXN1[82Q] mouse model of spinocerebellar ataxia 1

  2021cites this paper
• Robotic Mouse

  2021cites this paper
• Cerebellar Patterning Defects in Mutant Mice

  2021cites this paper
• Longitudinal single-cell transcriptional dynamics throughout neurodegeneration in SCA1

  2021cites this paper
• Aged Tmem106b knockout mice display gait deficits in coincidence with Purkinje cell loss and only limited signs of non‐motor dysfunction

  2020cites this paper
• Apolipoprotein D-mediated preservation of lysosomal function promotes cell survival and delays motor impairment in Niemann-Pick type A disease.

  2020influential citation
• Genetic defects in the sphingolipid degradation pathway and their effects on microglia in neurodegenerative disease.

  2020influential citation
• Brain pathology and cerebellar purkinje cell loss in a mouse model of chronic neuronopathic Gaucher disease.

  2020cites this paper
• Identification of Novel Pathways Associated with Patterned Cerebellar Purkinje Neuron Degeneration in Niemann-Pick Disease, Type C1

  2019cites this paper
• Aberrant cerebellar Purkinje cell function repaired in vivo by fusion with infiltrating bone marrow-derived cells

  2018cites this paper
• Cerebellar compartmentation of prion pathogenesis

  2018cites this paper
• Lipid‐induced lysosomal damage after demyelination corrupts microglia protective function in lysosomal storage disorders

  2018influential citation
• Patterned expression of Pannexin 1 channels in the adult cerebellar Purkinje cells

  2018cites this paper
• Zebrin II Is Ectopically Expressed in Microglia in the Cerebellum of Neurogenin 2 Null Mice

  2018cites this paper
• Ten plus one challenges in diseases of the lysosomal system.

  2017cites this paper
• Lipid Involvement in Neurodegenerative Diseases of the Motor System: Insights from Lysosomal Storage Diseases

  2017cites this paper
• Overview of Inflammation in Neurometabolic Diseases.

  2017cites this paper
• Sphingomyelin-induced inhibition of the plasma membrane calcium ATPase causes neurodegeneration in type A Niemann–Pick disease

  2017cites this paper
• Decreased calcium flux in Niemann‐Pick type C1 patient‐specific iPSC‐derived neurons due to higher amount of calcium‐impermeable AMPA receptors

  2017cites this paper
• Heat Shock Protein Beta-1 Modifies Anterior to Posterior Purkinje Cell Vulnerability in a Mouse Model of Niemann-Pick Type C Disease

  2016cites this paper
• Synaptic failure: The achilles tendon of sphingolipidoses

  2016cites this paper
• X-linked Christianson syndrome: heterozygous female Slc9a6 knockout mice develop mosaic neuropathological changes and related behavioral abnormalities

  2016cites this paper
• Niemann-Pick Type C Disease: Protective Mechanisms from Neurodegeneration and Targets for Therapeutic Intervention.

  2016influential citation
• Neuroinflammation, mitochondrial defects and neurodegeneration in mucopolysaccharidosis III type C mouse model.

  2015cites this paper
• Visual evoked potentials of Niemann-Pick type C1 mice reveal an impairment of the visual pathway that is rescued by 2-hydroxypropyl-ß-cyclodextrin

  2015cites this paper
• The neuroplastins: multifunctional neuronal adhesion molecules--involvement in behaviour and disease.

  2014cites this paper
• Niemann-Pick C disease and mobilization of lysosomal cholesterol by cyclodextrin

  2014cites this paper
• Development of cerebellar pathology in the canine model of mucopolysaccharidosis type IIIA (MPS IIIA).

  2014cites this paper
• The Neuroplastin adhesion molecules: key regulators of neuronal plasticity and synaptic function

  2014cites this paper
• Sphingolipid Disorders and the Neuronal Ceroid Lipofuscinoses or Batten Disease (Wolman Disease, Cholesteryl Ester Storage Disease, and Cerebrotendinous Xanthomatosis)

  2013cites this paper
• Autophagy and Cell Autonomous Neurodegeneration in Niemann-Pick type C Disease

  2013cites this paper
• Neuronopathic lysosomal storage diseases: clinical and pathologic findings.

  2013cites this paper
• Antigenic Compartmentation of the Cerebellar Cortex in an Australian Marsupial, the Tammar Wallaby Macropus eugenii

  2012cites this paper
• Brain pathology in Niemann Pick disease type A: insights from the acid sphingomyelinase knockout mice

  2011influential citation
• Apoptosis inducing factor deficiency causes reduced mitofusion 1 expression and patterned Purkinje cell degeneration.

  2011cites this paper
• Neural Stem Cell Transplantation as a Therapeutic Approach for Treating Lysosomal Storage Diseases

  2011cites this paper
• Patterned Purkinje cell loss in the ataxic sticky mouse

  2011cites this paper
• Cell Death as a Regulator of Cerebellar Histogenesis and Compartmentation

  2011cites this paper
• Mechanisms of Compartmental Purkinje Cell Death and Survival in the Lurcher Mutant Mouse

  2011cites this paper
• Heat shock protein 25 expression and preferential Purkinje cell survival in the lurcher mutant mouse cerebellum

  2010cites this paper
• Improvement in Lipid and Protein Trafficking in Niemann‐Pick C1 Cells by Correction of a Secondary Enzyme Defect

  2010cites this paper
• Local insulin-like growth factor I expression is essential for Purkinje neuron survival at birth

  2010cites this paper
• Cerebellar pathology and motor deficits in the palmitoyl protein thioesterase 1-deficient mouse.

  2009cites this paper
• The Role of Astrocyte Activation in Infantile Neuronal Ceroid Lipofuscinosis

  2009cites this paper
• Promising CNS-directed enzyme replacement therapy for lysosomal storage diseases.

  2009cites this paper
• Synaptic Vesicle Docking: Sphingosine Regulates Syntaxin1 Interaction with Munc18

  2009cites this paper
• Neuropathological changes in the peripheral nervous system and spinal cord in a transgenic mouse model of Niemann-Pick disease type A.

  2009cites this paper
• Neuropathology of the acid sphingomyelinase knockout mouse model of Niemann-Pick A disease including structure-function studies associated with cerebellar Purkinje cell degeneration.

  2008cites this paper
• The function of sphingolipids in the nervous system: lessons learnt from mouse models of specific sphingolipid activator protein deficiencies

  2007influential citation
• Phospholipase cβ4 expression reveals the continuity of cerebellar topography through development

  2007cites this paper
• Anomalous surface distribution of glycosyl phosphatidyl inositol-anchored proteins in neurons lacking acid sphingomyelinase.

  2007cites this paper
• Injection of mouse and human neural stem cells into neonatal Niemann-Pick A model mice.

  2007cites this paper
• Aldolase C‐positive cerebellar Purkinje cells are resistant to delayed death after cerebral trauma and AMPA‐mediated excitotoxicity

  2007cites this paper
• Behavioural characterisation of the robotic mouse mutant.

  2007cites this paper
• Sphingolipids in apoptosis, survival and regeneration in the nervous system.

  2006cites this paper
• Identification of novel biomarkers for Niemann-Pick disease using gene expression analysis of acid sphingomyelinase knockout mice.

  2006cites this paper
• Sphingolipids in apoptosis, survival and regeneration in the nervous system.

  2006cites this paper
• Complementary stripes of phospholipase Cβ3 and Cβ4 expression by Purkinje cell subsets in the mouse cerebellum

  2006cites this paper
• Spatiotemporal Analysis of Purkinje Cell Degeneration Relative to Parasagittal Expression Domains in a Model of Neonatal Viral Infection

  2006cites this paper
• The robotic mouse: Unravelling the function of AF4 in the cerebellum

  2005cites this paper
• Analysis of human neurological disorders using mutagenesis in the mouse.

  2005cites this paper
• Defective calcium homeostasis in the cerebellum in a mouse model of Niemann–Pick A disease

  2005cites this paper
• AAV vector-mediated correction of brain pathology in a mouse model of Niemann-Pick A disease.

  2005cites this paper
• Gene transfer of human acid sphingomyelinase corrects neuropathology and motor deficits in a mouse model of Niemann-Pick type A disease.

  2005cites this paper
• The organization of the corticonuclear and olivocerebellar climbing fiber projections to the rat cerebellar vermis: The congruence of projection zones and the zebrin pattern

  2004cites this paper
• Intracerebral Transplantation of Adult Mouse Neural Progenitor Cells into the Niemann-Pick-A Mouse Leads to a Marked Decrease in Lysosomal Storage Pathology

  2004cites this paper
• The pathogenesis of glycosphingolipid storage disorders.

  2004cites this paper
• Sphingomyelin and cholesterol: from membrane biophysics and rafts to potential medical applications.

  2004cites this paper
• The toppler mouse: A novel mutant exhibiting loss of Purkinje cells

  2004cites this paper
• Compartmentation of the mouse cerebellar cortex by sphingosine kinase

  2004cites this paper
• Mutation in saposin D domain of sphingolipid activator protein gene causes urinary system defects and cerebellar Purkinje cell degeneration with accumulation of hydroxy fatty acid-containing ceramide in mouse.

  2004cites this paper
• A Riddle Wrapped in a Mystery: Understanding Niemann-Pick Disease, Type C

  2003cites this paper
• Expression of the immunoglobulin superfamily neuroplastin adhesion molecules in adult and developing mouse cerebellum and their localisation to parasagittal stripes

  2003cites this paper
• Antigenic compartmentation of the cerebellar cortex in the syrian hamster Mesocricetus auratus.

  2003cites this paper
• Sphingomyelin and ceramide in brain aging, neuronal plasticity and neurodegenerative disorders

  2003cites this paper
• Patterned Purkinje cell death in the cerebellum.

  2003cites this paper
• A Mutation in Af4 Is Predicted to Cause Cerebellar Ataxia and Cataracts in the Robotic Mouse

  2003cites this paper
• Distinctive cerebellar immunoreactivity for the prion protein in familial (E200K) Creutzfeldt-Jakob disease

  2003cites this paper
• Patterned Purkinje cell degeneration in mouse models of Niemann‐Pick type C disease

  2003cites this paper
• Reproductive pathology and sperm physiology in acid sphingomyelinase-deficient mice.

  2002cites this paper
• Whole-mount Immunohistochemistry: A High-throughput Screen for Patterning Defects in the Mouse Cerebellum

  2002cites this paper
• Neuroinflammation, mitochondrial defects and neurodegeneration in mucopolysaccharidosis III type C mouse model

  year unknowncites this paper