ULTRASTRUCTURAL CHANGES IN THE MITOCHONDRIA OF CEREBELLAR PURKINJE CELLS OF NERVOUS MUTANT MICE

The maturation of cerebellar Purkinje cells of normal and nervous (nr/nr) mutant mice has been studied by light and electron microscopy. In the mutant, 90% of Purkinje cells selectively degenerate between postnatal days 23 and 50. Losses are greater in lateral than medial regions. Other cerebellar neurons appear normal. The first morphological abnormality recognized is the presence of rounded mitochondria in perikarya of some Purkinje cells of the mutant at 9 days after birth. By 15 days, all nr/nr Purkinje cells contain spherical mitochondria and begin to deviate from the normal maturational sequence. Elaboration of the extensive dendritic tree halts midway and newly formed axon collateral fibers degenerate. In the perikaryon, the basal polysomal accumulation and climbing fiber-somatic spine synapses are sometimes abnormally retained. Cisternae of the Golgi apparatus and rough endoplasmic reticulum cease to form aligned stacks, and decrease in number, while polysomes dissociate into free ribosomes. These changes are progressive, culminating in cell death. Although every nr/nr Purkinje cell demonstrates spherical mitochondria, some cells survive the critical period, retain a near-normal complement of organelles, and reacquire normal-appearing mitochondria. The disorder appears intrinsic to Purkinje cells since all major classes of synapses were identified before cell death.

• Publication year

  1973

• Venue

  Journal of Cell Biology

• Publication date

  1973-06-01

• Fields of study

  Biology, Medicine

• Identifiers
  DOI 10.1083/JCB.57.3.782PMID 4698906PMCID 2109013
• 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.

• Interrelations of basket cell axons and climbing fibers in the cerebellar cortex of the rat

  2004cited by this paper
• The recurrent collaterals of Purkinje cell axons: A correlated study of the rat's cerebellar cortex with electron microscopy and the Golgi method

  2004cited by this paper
• The biosynthesis of mitochondrial cytochromes.

  1974cited by this paper
• THE BIOSYNTHESIS OF MITOCHONDRIAL CYTOCHROMES

  1974cited by this paper
• Postnatal development of the cerebellar cortex in the rat. II. Phases in the maturation of Purkinje cells and of the molecular layer

  1972cited by this paper
• Possible division of cardiac mitochondria

  1972cited by this paper
• Studies on norepinephrine-containing afferents to Purkinje cells of art cerebellum. I. Localization of the fibers and their synapses.

  1971cited by this paper
• On the projections from the locus coeruleus noradrealine neurons: the cerebellar innervation.

  1971cited by this paper
• Morphometric analysis of the rat liver cell in the perinatal period.

  1971cited by this paper
• THE EFFECTS OF IRON DEFICIENCY ON THE HEPATOCYTE: A BIOCHEMICAL AND ULTRASTRUCTURAL STUDY

  1971cited by this paper
• GENESIS OF MITOCHONDRIA IN INSECT FAT BODY

  1970cited by this paper
• Giant Hepatic Mitochondria: Production in Mice Fed with Cuprizone

  1969cited by this paper
• 'nervous', A new mutant mouse with cerebellar disease. Les mutants pathologiques chez l'animal (colloq. Internat. Cnrs no.

  1969influential reference
• RIBOFLAVIN AND MOUSE HEPATIC CELL STRUCTURE AND FUNCTION

  1969cited by this paper
• A QUANTITATIVE STEREOLOGICAL DESCRIPTION OF THE ULTRASTRUCTURE OF NORMAL RAT LIVER PARENCHYMAL CELLS

  1968cited by this paper
• Biochemical aspects of the biogenesis of mitochondria

  1968cited by this paper
• A QUANTITATIVE DESCRIPTION OF CORTISONE-INDUCED ALTERATIONS IN THE ULTRASTRUCTURE OF RAT LIVER PARENCHYMAL CELLS

  1968cited by this paper
• Riboflavin and mouse hepatic cell structure and function. I. Ultrastructural alterations in simple deficiency.

  1968cited by this paper
• Cytological changes in the fat body of Rhodnius during starvation, feeding and oxygen want.

  1967cited by this paper
• Incorporation of amino acids into the outer and inner membrane of isolated rat liver mitochondria.

  1967cited by this paper
• SYNCHRONOUS MITOCHONDRIAL DIVISION IN NEUROSPORA CRASSA

  1967cited by this paper
• Serial section analysis of mitochondrial form and membrane relationships in the neonatal rat liver cell.

  1967cited by this paper
• Observations on the ultrastructure of the developing cerebellum of the Macaca mulatta

  1967cited by this paper
• Synthesis of mitochondrial proteins: Demonstration of a transfer of proteins from microsomes into mitochondria

  1967cited by this paper
• A formaldehyde-glutaraldehyde fixative of high osmolality for use in electron-microscopy

  1965influential reference
• CHANGES IN HEPATIC STRUCTURE IN RATS PRODUCED BY BREATHING PURE OXYGEN

  1965cited by this paper
• A LIGHT AND ELECTRON MICROSCOPE STUDY OF THE MORPHOLOGICAL CHANGES INDUCED IN RAT LIVER CELLS BY THE AZO DYE 2-ME-DAB

  1964cited by this paper
• MITOCHONDRIAL CHANGES IN THE LIVER OF ESSENTIAL FATTY ACID-DEFICIENT MICE

  1963cited by this paper
• The Morphological and Structural Features of Neurons in Vitro Studied by Phase-Contrast and Time-Lapse Movies

  1963cited by this paper
• The cerebellar cortex of the rabbit. An electron microscopic study

  1963cited by this paper
• The American Medical Association.

  1947cited by this paper
• Mitochondria (and other cytoplasmic structures) in tissue cultures

  1915cited by this paper

• Sexual Dimorphism of Ethanol-Induced Mitochondrial Dynamics in Purkinje Cells

  2024cites this paper
• Meshed neuronal mitochondrial networks empowered by AI-powered classifiers and immersive VR reconstruction

  2023influential citation
• Opiate Drugs with Abuse Liability Hijack the Endogenous Opioid System to Disrupt Neuronal and Glial Maturation in the Central Nervous System

  2018cites this paper
• A multi‐ingredient dietary supplement abolishes large‐scale brain cell loss, improves sensory function, and prevents neuronal atrophy in aging mice

  2016influential citation
• Purkyně and the Cerebellum Then and Now

  2016cites this paper
• Experimental neurotransplantation treatment for hereditary cerebellar ataxias

  2016cites this paper
• Cell death and neurodegeneration in the postnatal development of cerebellar vermis in normal and Reeler mice.

  2016cites this paper
• Jan Evangelista Purkyně and the cerebellum then and now.

  2015cites this paper
• Structure and function of the interphotoreceptor matrix surrounding retinal photoreceptor cells.

  2015cites this paper
• Motor dysfunction in cerebellar Purkinje cell-specific vesicular GABA transporter knockout mice

  2014cites this paper
• Complex Dietary Interventions to Slow Rates of Aging

  2014cites this paper
• From mice to men: lessons from mutant ataxic mice

  2014influential citation
• Tissue plasminogen activator regulates Purkinje neuron development and survival

  2013cites this paper
• CHARACTERIZATION AND TREATMENT OF A NOVEL MOUSE MODEL OF TSC-ASSOCIATED AUTISM

  2012cites this paper
• The Ataxic Syrian Hamster: An Animal Model Homologous to the pcd Mutant Mouse?

  2009cites this paper
• The effects of cerebellar damage on maze learning in animals

  2008cites this paper
• Purkinje cell death: Differences between developmental cell death and neurodegenerative death in mutant mice

  2008influential citation
• The robotic mouse: Unravelling the function of AF4 in the cerebellum

  2005cites this paper
• Large-scale genomic approaches to brain development and circuitry.

  2005cites this paper
• Purkinje cell neuroaxonal dystrophy similar to nervous mutant mice phenotype in two sibling kittens

  2004cites this paper
• The critical period of Purkinje cell degeneration and cerebellar hypoplasia due to bilirubin

  2004cites this paper
• An ultrastructural study on the cerebellum of the brindled mouse

  2004cites this paper
• Development and developmental disorders of the human cerebellum

  2003cites this paper
• Motor Coordination, Exploration, and Spatial Learning in a Natural Mouse Mutation (nervous) with Purkinje Cell Degeneration

  2003cites this paper
• DIMINISHING PURKINJE CELL POPULATIONS IN THE CEREBELLA OF AGING HETEROZYGOUS PURKINJE CELL DEGENERATION BUT NOT HETEROZYGOUS NERVOUS MICE

  2002influential citation
• The cerebellar granule cell

  2002cites this paper
• Retinal degeneration in the nervous mutant mouse. IV. Inner retinal changes.

  2001cites this paper
• Insights from mouse models into the molecular basis of neurodegeneration.

  2000cites this paper
• Retinal degeneration in the nervous mutant mouse. III. Electrophysiological studies of the visual pathway.

  2000cites this paper
• Cerebellar defect and impaired motor coordination in mice lacking vimentin

  1999cites this paper
• A high-resolution genetic map of the nervous locus on mouse chromosome 8.

  1998cites this paper
• Light and Electron Microscopic Analysis of the Central and Peripheral Nervous Systems of Acid Sphingomyelinase‐deficient Mice Resulting from Gene Targeting

  1997cites this paper
• Cerebellar Mutants in the Laboratory Mouse

  1997cites this paper
• A stereological study on the nuclear volume of cerebellar granule cells in aging rats.

  1997cites this paper
• Parallin, a cerebellar granule cell protein the expression of which is developmentally regulated by Purkinje cells: evidence from mutant mice.

  1997cites this paper
• Structural Integration of Cerebellar Grafts in Ataxic Mouse Mutants

  1997cites this paper
• Diverse effects of Purkinje cell loss on deep cerebellar and vestibular nuclei neurons in Purkinje cell degeneration mutant mice: A possible compensatory mechanism

  1997cites this paper
• The cerebellar model of neural grafting: structural integration and functional recovery.

  1996cites this paper
• Autoradiographic and electron microscopic studies of retinal rosettes in genetically microphthalmic mice.

  1996cites this paper
• Tissue distribution, genomic structure, and chromosome mapping of mouse and human eukaryotic initiation factor 4E-binding proteins 1 and 2.

  1996cites this paper
• Target neuron controls the integrity of afferent axon phenotype: a study on the Purkinje cell-climbing fiber system in cerebellar mutant mice

  1995influential citation
• Effects of nervous mutation on Purkinje cell compartments defined by Zebrin II and 9-O-acetylated gangliosides expression.

  1994cites this paper
• Altered 9-O acetylation of disialogangliosides in cerebellar Purkinje cells of the nervous mutant mouse.

  1994cites this paper
• Cerebellar contributions to instrumental learning.

  1994cites this paper
• Retinal degeneration in the nervous mutant mouse. II. Electron microscopic analysis

  1993cites this paper
• Identification of cannabinoid receptors in cultures of rat cerebellar granule cells.

  1993cites this paper
• The distribution of dystrophin in the murine central nervous system: an immunocytochemical study.

  1993cites this paper
• Alterations in the Dorsal Cochlear Nucleus of Cerebellar Mutant Mice

  1993cites this paper
• Retinal degeneration in the nervous mutant mouse. I. Light microscopic cytopathology and changes in the interphotoreceptor matrix

  1993cites this paper
• Cerebellar Grafting as a Tool to Analyze New Aspects of Cerebellar Development and Plasticity

  1992cites this paper
• Glutamate Receptors in Mammalian Cerebellum: Alterations in Human Ataxic Disorders and Cerebellar Mutant Mice

  1992cites this paper
• Purkinje Cell Heterogeneity: Its Role in Organizing the Topography of the Cerebellar Cortex Connections

  1992cites this paper
• Neuronal localization of cannabinoid receptors and second messengers in mutant mouse cerebellum.

  1991cites this paper
• The fine structure of the Purkinje cell and its afferents in lurcher chimeric mice

  1991cites this paper
• Autoradiographic localization of binding sites for second messengers on neurones and astrocytes of cultured rat cerebellum.

  1991cites this paper
• Calcium binding protein (calbindin-D28k) gene expression in the developing and aging mouse cerebellum.

  1990cites this paper
• Loss of Sulfoglucuronyl and Other Neolactoglycolipids in Purkinje Cell Abnormality Murine Mutants

  1990cites this paper
• Two‐Dimensional Gel Electrophoretic Analysis of the Cerebella from Neuro‐Pathologically‐Mutant Weaver, Nervous and Staggerer Mice 1

  1990cites this paper
• Axonal abnormalities in cerebellar Purkinje cells of the ‘hyperspiny Purkinje cell’ mutant mouse

  1990cites this paper
• The Purkinje cell class may extend beyond the cerebellum

  1990cites this paper
• Cerebellar synaptogenesis: what we can learn from mutant mice.

  1990cites this paper
• Regional and topographic organization of the olivocerebellar projection in homozygous staggerer (sg/sg) mutant mice: an anterograde and retrograde tracing study.

  1989cites this paper
• Primary structure and functional expression of the inositol 1,4,5-trisphosphate-binding protein P400

  1989cites this paper
• Development of Cerebellar Hypoplasia in Jaundiced Gunn Rats: A Morphological Study

  1989cites this paper
• Purkinje cell abnormalities and synaptogenesis in genetically jaundiced rats (Gunn rats).

  1989cites this paper
• Autoradiographic localization of particulate cyclic AMP-dependent protein kinase in mammalian brain using [3H]cyclic AMP: implications for organization of second messenger systems.

  1989cites this paper
• Cerebellin and related postsynaptic peptides in the brain of normal and neurodevelopmentally mutant vertebrates

  1988cites this paper
• Neuronal influence on glial enzyme expression: evidence from chimeric mouse cerebellum.

  1988cites this paper
• Cerebellar Involvement in Murine Sphingomyelinosis: A New Model of Niemann-Pick Disease

  1988cites this paper
• Dendritic and axonal distribution of the microtubule-associated proteins MAP2 and tau in the cerebellum of the nervous mutant mouse.

  1988cites this paper
• The Neurobiology of Cerebellar Senescence

  1988cites this paper
• Cerebellar benzodiazepine receptors: cellular localization and consequences of neurological mutations in mice.

  1988cites this paper
• The cellular neurobiology of neuronal development: the cerebellar granule cell.

  1988cites this paper
• cDNA cloning and characterization of three genes uniquely expressed in cerebellum by Purkinje neurons

  1988cites this paper
• Light and electron microscopic studies in the cerebellum of "trembler" mutant of chickens.

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

  1988cites this paper
• Late-onset cerebellar degeneration in mice induced transplacentally by methylnitrosourea.

  1987cites this paper
• Effect of purkinje cell loss on cerebellar gangliosides in nervous mutant mice

  1987cites this paper
• l‐Aspartate and l‐glutamate binding sites in developing normal and ‘nervous’ mutant mouse cerebellum

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

  1987cites this paper
• Regional differences in cytoarchitecture of the weaver cerebellum suggest a new model for weaver gene action.

  1987cites this paper
• Neurogenetics and Behavior Related to Higher Brain Functions

  1987cites this paper
• Spontaneous alternation and habituation in a t-maze in nervous mutant mice.

  1986cites this paper
• GENETIC MOSAICS AS TOOLS FOR THE STUDY OF THE RETINA

  1986cites this paper
• Cerebellar benzodiazepine receptor distribution: an autoradiographic study of the normal C57BL/6J and Purkinje cell degeneration mutant mouse.

  1986cites this paper
• Heterogeneous localization of protein kinase C in rat brain: autoradiographic analysis of phorbol ester receptor binding

  1986cites this paper
• Degeneration of thalamic neurons in “Purkinje cell degeneration” mutant mice. I. Distribution of neuron loss

  1985cites this paper
• On the location of γ-aminobutyrate and benzodiazepine receptors in the cerebellum of the normal C3H and Lurcher mutant mouse

  1985cites this paper
• Fine Structure of Hereditary Defects of the Central Nervous System in Mice

  1985cites this paper
• Exploration of a hole-board matrix in nervous mutant mice.

  1985cites this paper
• Cerebellar hypoplasia in the Gunn rat is associated with quantitative changes in neurotypic and gliotypic proteins.

  1985cites this paper
• 28 K cholecalcin (CaBP) levels in abnormal cerebella: studies on mutant mice and harmaline- and 3-acetylpyridine-treated rats.

  1985cites this paper
• Canine Inherited Ataxia: Ultrastructural Observations

  1985cites this paper
• Ectopic parvalbumin-positive cells in the cerebellum of the adult mutant mouse 'nervous'.

  1985cites this paper
• Degeneration of thalamic neurons in “Purkinje cell degeneration” mutant mice. II. Cytology of neuron loss

  1985cites this paper
• Exploration and habituation in nervous mutant mice.

  1985cites this paper
• Sites of Gene Action in Rodents with Hereditary Retinal Degenerations

  1985cites this paper
• gamma-aminobutyric acid B receptors are negatively coupled to adenylate cyclase in brain, and in the cerebellum these receptors may be associated with granule cells.

  1984cites this paper
• Neuronal influence on glial enzyme expression: evidence from mutant mouse cerebella.

  1984cites this paper
• Localization in mammalian brain of G-substrate, a specific substrate for guanosine 3',5'-cyclic monophosphate-dependent protein kinase

  1984cites this paper