Developmental delay in motor skill acquisition in Niemann-Pick C1 mice reveals abnormal cerebellar morphogenesis

Altered cerebellum development and impaired motor coordination in mice lacking the Btg1 gene: Involvement of cyclin D1

(2015) Manuela Ceccarelli et al.   DEVELOPMENTAL BIOLOGY

High incidence of unrecognized visceral/neurological late-onset Niemann-Pick disease, type C1, predicted by analysis of massively parallel sequencing data sets

(2015) Christopher A. Wassif et al.   GENETICS IN MEDICINE

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

(2015) Giampiero Palladino et al.   Orphanet Journal of Rare Diseases

Intracisternal cyclodextrin prevents cerebellar dysfunction and Purkinje cell death in feline Niemann-Pick type C1 disease

(2015) Charles H. Vite et al.   Science Translational Medicine

Cholesterol metabolism and homeostasis in the brain

(2015) Juan Zhang et al.   Protein & Cell

Abnormal climbing fibre-Purkinje cell synaptic connections in the essential tremor cerebellum

(2014) Chi-Ying Lin et al.   BRAIN

Relevance of neuronal and glial NPC1 for synaptic input to cerebellar Purkinje cells

(2014) Isabelle Buard et al.   MOLECULAR AND CELLULAR NEUROSCIENCE

A marked paucity of granule cells in the developing cerebellum of the Npc1−/− mouse is corrected by a single injection of hydroxypropyl-β-cyclodextrin

(2014) S. Nusca et al.   NEUROBIOLOGY OF DISEASE

Pre-reproductive maternal enrichment influences offspring developmental trajectories: motor behavior and neurotrophin expression

(2014) Paola Caporali et al.   Frontiers in Behavioral Neuroscience

Myelin management by the 18.5-kDa and 21.5-kDa classic myelin basic protein isoforms

(2013) George Harauz et al.   JOURNAL OF NEUROCHEMISTRY

An Acute Glutamate Exposure Induces Long-Term Down Regulation of GLAST/EAAT1 Uptake Activity in Cultured Bergmann Glia Cells

(2013) Daniel Martínez et al.   NEUROCHEMICAL RESEARCH

Niemann-Pick disease type C clinical database: cognitive and coordination deficits are early disease indicators

(2013) Miriam Stampfer et al.   Orphanet Journal of Rare Diseases

Precocious cerebellum development and improved motor functions in mice lacking the astrocyte cilium-, patched 1-associated Gpr37l1 receptor

(2013) D. Marazziti et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Origin, lineage and function of cerebellar glia

(2013) Annalisa Buffo et al.   PROGRESS IN NEUROBIOLOGY

Npc1 Acting in Neurons and Glia Is Essential for the Formation and Maintenance of CNS Myelin

(2013) Ting Yu et al.   PLoS Genetics

Impaired motor coordination and disrupted cerebellar architecture in Fgfr1 and Fgfr2 double knockout mice

(2012) Karen Müller Smith et al.   BRAIN RESEARCH

Timing of developmental sequences in different brain structures: physiological and pathological implications

(2012) N. Dehorter et al.   EUROPEAN JOURNAL OF NEUROSCIENCE

Bergmann Glial AMPA Receptors Are Required for Fine Motor Coordination

(2012) A. S. Saab et al.   SCIENCE

A novel mouse model of Niemann–Pick type C disease carrying a D1005G-Npc1 mutation comparable to commonly observed human mutations

(2011) Robert A. Maue et al.   HUMAN MOLECULAR GENETICS

Unesterified Cholesterol Accumulation in Late Endosomes/Lysosomes Causes Neurodegeneration and Is Prevented by Driving Cholesterol Export from This Compartment

(2011) A. Aqul et al.   JOURNAL OF NEUROSCIENCE

Decreased expression of myelin gene regulatory factor in Niemann-Pick type C 1 mouse

(2011) Xin Yan et al.   METABOLIC BRAIN DISEASE

GFAP in health and disease

(2011) J. Middeldorp et al.   PROGRESS IN NEUROBIOLOGY

Defective cholesterol trafficking in Niemann-Pick C-deficient cells

(2010) Kyle B. Peake et al.   FEBS LETTERS

White and gray matter alterations in adults with Niemann-Pick disease type C: A cross-sectional study

(2010) M. Walterfang et al.   NEUROLOGY

Weekly Cyclodextrin Administration Normalizes Cholesterol Metabolism in Nearly Every Organ of the Niemann-Pick Type C1 Mouse and Markedly Prolongs Life

(2010) Charina M Ramirez et al.   PEDIATRIC RESEARCH

Functions of glutamate transporters in cerebellar Purkinje cell synapses

(2009) Y. Takayasu et al.   Acta Physiologica

Four distinct phases of basket/stellate cell migration after entering their final destination (the molecular layer) in the developing cerebellum

(2009) D. Bryant Cameron et al.   DEVELOPMENTAL BIOLOGY

Structure and function of the blood–brain barrier

(2009) N. Joan Abbott et al.   NEUROBIOLOGY OF DISEASE

Chronic Cyclodextrin Treatment of Murine Niemann-Pick C Disease Ameliorates Neuronal Cholesterol and Glycosphingolipid Storage and Disease Progression

(2009) Cristin D. Davidson et al.   PLoS One

Regional Differences in the Temporal Expression of Nonapoptotic Caspase-3-Positive Bergmann Glial Cells in the Developing Rat Cerebellum

(2009) VelvetLee Finckbone   Frontiers in Neuroanatomy

Down-regulation of astrocytic GLAST by microglia-related inflammation is abrogated in dibutyryl cAMP-differentiated cultures

(2008) Sébastien Tilleux et al.   JOURNAL OF NEUROCHEMISTRY

Altered Neuron Excitability and Synaptic Plasticity in the Cerebellar Granular Layer of Juvenile Prion Protein Knock-Out Mice with Impaired Motor Control

(2008) F. Prestori et al.   JOURNAL OF NEUROSCIENCE

Astrocyte-only Npc1 reduces neuronal cholesterol and triples life span ofNpc1-/-mice

(2008) Min Zhang et al.   JOURNAL OF NEUROSCIENCE RESEARCH

Brain cholesterol metabolism and neurologic disease

(2008) E. E. Benarroch   NEUROLOGY