CRISPR/Cas9 mediated generation of an ovine model for infantile neuronal ceroid lipofuscinosis (CLN1 disease)

A Huntingtin Knockin Pig Model Recapitulates Features of Selective Neurodegeneration in Huntington’s Disease

(2018) Sen Yan et al.   CELL

Longitudinal in vivo monitoring of CNS demonstrates the efficacy of gene therapy in a sheep model of CLN5 Batten disease

(2018) Nadia L. Mitchell et al.   MOLECULAR THERAPY

Bridging the gap: large animal models in neurodegenerative research

(2017) S. L. Eaton et al.   MAMMALIAN GENOME

A girl with infantile neuronal ceroid lipofuscinosis caused by novel PPT1 mutation and paternal uniparental isodisomy of chromosome 1

(2016) Yo Niida et al.   BRAIN & DEVELOPMENT

HomozygousPPT1Splice Donor Mutation in a Cane Corso Dog With Neuronal Ceroid Lipofuscinosis

(2016) A. Kolicheski et al.   JOURNAL OF VETERINARY INTERNAL MEDICINE

Development and Implementation of a Corriedale Ovine Brain Atlas for Use in Atlas-Based Segmentation

(2016) Kishan Andre Liyanage et al.   PLoS One

How Does the Accuracy of Intracranial Volume Measurements Affect Normalized Brain Volumes? Sample Size Estimates Based on 966 Subjects from the HUNT MRI Cohort

(2015) T.I. Hansen et al.   AMERICAN JOURNAL OF NEURORADIOLOGY

Genetics of the neuronal ceroid lipofuscinoses (Batten disease)

(2015) Sara E. Mole et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

A novel porcine model of ataxia telangiectasia reproduces neurological features and motor deficits of human disease

(2015) Rosanna Beraldi et al.   HUMAN MOLECULAR GENETICS

Diagnosing limb paresis and paralysis in sheep

(2015) James Patrick Crilly et al.   IN PRACTICE

Engineering large animal models of human disease

(2015) C Bruce A Whitelaw et al.   JOURNAL OF PATHOLOGY

Construction of an MRI 3D high resolution sheep brain template

(2015) Arsène Ella et al.   MAGNETIC RESONANCE IMAGING

Nonclinical evaluation of CNS-administered TPP1 enzyme replacement in canine CLN2 neuronal ceroid lipofuscinosis

(2015) Brian R. Vuillemenot et al.   MOLECULAR GENETICS AND METABOLISM

Large animal models of rare genetic disorders: sheep as phenotypically relevant models of human genetic disease

(2015) Ashish R. Pinnapureddy et al.   Orphanet Journal of Rare Diseases

Rapid and Progressive Regional Brain Atrophy in CLN6 Batten Disease Affected Sheep Measured with Longitudinal Magnetic Resonance Imaging

(2015) Stephen J. Sawiak et al.   PLoS One

Molecular neuropathology of the synapse in sheep with CLN5 Batten disease

(2015) Inês S. Amorim et al.   Brain and Behavior

Enzyme replacement therapy attenuates disease progression in a canine model of late-infantile neuronal ceroid lipofuscinosis (CLN2 disease)

(2014) Martin L. Katz et al.   JOURNAL OF NEUROSCIENCE RESEARCH

Oral cysteamine bitartrate and N-acetylcysteine for patients with infantile neuronal ceroid lipofuscinosis: a pilot study

(2014) Sondra W Levin et al.   LANCET NEUROLOGY

Genome edited sheep and cattle

(2014) Chris Proudfoot et al.   TRANSGENIC RESEARCH

Pathogenesis and therapies for infantile neuronal ceroid lipofuscinosis (infantile CLN1 disease)

(2013) Jacqueline A. Hawkins-Salsbury et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

Pupillary light reflex deficits in a canine model of late infantile neuronal ceroid lipofuscinosis

(2013) Rebecca E.H. Whiting et al.   EXPERIMENTAL EYE RESEARCH

A Murine Model of Variant Late Infantile Ceroid Lipofuscinosis Recapitulates Behavioral and Pathological Phenotypes of Human Disease

(2013) Jeremy P. Morgan et al.   PLoS One

Symmetric diffeomorphic modeling of longitudinal structural MRI

(2013) John Ashburner   Frontiers in Neuroscience

Human pathology in NCL

(2012) Glenn W. Anderson et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

A 3D wavelet fusion approach for the reconstruction of isotropic-resolution MR images from orthogonal anisotropic-resolution scans

(2011) Iman Aganj et al.   MAGNETIC RESONANCE IN MEDICINE

Diffeomorphic registration using geodesic shooting and Gauss–Newton optimisation

(2011) John Ashburner et al.   NEUROIMAGE

A mutation in canine PPT1 causes early onset neuronal ceroid lipofuscinosis in a Dachshund

(2010) Douglas N. Sanders et al.   MOLECULAR GENETICS AND METABOLISM

Biodemography of human ageing

(2010) James W. Vaupel   NATURE

A reproducible evaluation of ANTs similarity metric performance in brain image registration

(2010) Brian B. Avants et al.   NEUROIMAGE

Removing Obstacles in Neuroscience Drug Discovery: The Future Path for Animal Models

(2008) Athina Markou et al.   NEUROPSYCHOPHARMACOLOGY

Symmetric diffeomorphic image registration with cross-correlation: Evaluating automated labeling of elderly and neurodegenerative brain

(2007) B AVANTS et al.   MEDICAL IMAGE ANALYSIS