J. Weimer

发表

Changes in motor behavior, neuropathology, and gut microbiota of a Batten disease mouse model following administration of acidified drinking water

Tyler B. Johnson, J. Zhao, D. Pearce, 2019, Scientific Reports.

MARCKS Is Necessary for Netrin-DCC Signaling and Corpus Callosum Formation

D. Stumpo, P. Blackshear, K. Roux, 2018, Molecular Neurobiology.

MARCKS regulates neuritogenesis and interacts with a CDC42 signaling network

H. T. Ghashghaei, K. Adler, Erika S Wittchen, 2018, Scientific Reports.

MARCKS modulates radial progenitor placement, proliferation and organization in the developing cerebral cortex

D. Stumpo, P. Blackshear, E. Anton, 2009, Development.

Elevation of Hook1 in a disease model of Batten disease does not affect a novel interaction between Ankyrin G and Hook1.

D. Pearce, Jill M Weimer, Subrata Chattopadhyay, 2005, Biochemical and biophysical research communications.

Visual deficits in a mouse model of Batten disease are the result of optic nerve degeneration and loss of dorsal lateral geniculate thalamic neurons

J. Cooper, H. Federoff, Evan P. Kingsley, 2006, Neurobiology of Disease.

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

J. Cooper, J. Weimer, H. Magee, 2013, PloS one.

Therapeutic efficacy of antisense oligonucleotides in mouse models of CLN3 Batten disease

Tyler B. Johnson, M. L. Hastings, F. Rigo, 2020, Nature Medicine.

AAV9 gene therapy restores lifespan and treats pathological and behavioral abnormalities in a mouse model of CLN8-Batten disease

Tyler B. Johnson, K. Meyer, S. Likhite, 2020, bioRxiv.

AAV9 Gene Therapy Increases Lifespan and Treats Pathological and Behavioral Abnormalities in a Mouse Model of CLN8-Batten Disease.

Tyler B. Johnson, K. Meyer, S. Likhite, 2020, Molecular therapy : the journal of the American Society of Gene Therapy.

Gene Therapy Corrects Brain and Behavioral Pathologies in CLN6-Batten Disease

Tyler B. Johnson, C. Pierson, B. Kaspar, 2019, Molecular therapy : the journal of the American Society of Gene Therapy.

A multimodal approach to identify clinically relevant biomarkers to comprehensively monitor disease progression in a mouse model of pediatric neurodegenerative disease

Tyler B. Johnson, T. Bragge, Pekka Poutiainen, 2020, Progress in Neurobiology.

A multimodal approach to identify clinically relevant parameters to monitor disease progression in a preclinical model of neuropediatric disease

Tyler B. Johnson, T. Bragge, J. Weimer, 2019, bioRxiv.

Radiomic biomarkers informative of cancerous transformation in neurofibromatosis-1 plexiform tumors.

D. Meyerholz, D. Quelle, B. Darbro, 2019, Journal of neuroradiology. Journal de neuroradiologie.

A porcine model of neurofibromatosis type 1 that mimics the human disease.

D. Meyerholz, D. Quelle, B. Darbro, 2018, JCI insight.

Immunohistochemical Markers for Prospective Studies in Neurofibromatosis-1 Porcine Models

D. Meyerholz, D. Quelle, B. Darbro, 2017, The journal of histochemistry and cytochemistry : official journal of the Histochemistry Society.

Assessment of nociception and related quality of life measures in a porcine model of Neurofibromatosis type 1.

D. Meyerholz, D. Quelle, B. Darbro, 2019, Pain.

Genetic ataxia telangiectasia porcine model phenocopies the multisystemic features of the human disease.

D. Meyerholz, D. Pearce, J. Weimer, 2017, Biochimica et biophysica acta. Molecular basis of disease.

Therapeutic landscape for Batten disease: current treatments and future prospects

Tyler B. Johnson, D. Pearce, J. Weimer, 2019, Nature Reviews Neurology.

MARCKS-dependent mucin clearance and lipid metabolism in ependymal cells are required for maintenance of forebrain homeostasis during aging

H. T. Ghashghaei, D. Stumpo, P. Blackshear, 2015, Aging cell.

The neuronal ceroid lipofuscinoses

D. Pearce, J. Weimer, Yasser M Elshatory, 2007, NeuroMolecular Medicine.

The neuronal ceroid lipofuscinoses: mutations in different proteins result in similar disease.

D. Pearce, J. Weimer, Yasser M Elshatory, 2002, Neuromolecular medicine.

Transmembrane Batten Disease Proteins Interact With a Shared Network of Vesicle Sorting Proteins, Impacting Their Synaptic Enrichment

Tyler B. Johnson, K. Roux, J. Weimer, 2022, Frontiers in Neuroscience.

Promoting validation and cross-phylogenetic integration in model organism research

Julie H. Simpson, J. Postlethwait, W. Talbot, 2022, Disease models & mechanisms.

Validating indicators of CNS disorders in a swine model of neurological disease

D. Meyerholz, D. Quelle, R. Khanna, 2019, bioRxiv.

A BAC transgenic mouse model to analyze the function of astroglial SPARCL1 (SC1) in the central nervous system

E. Anton, Jr-Gang Cheng, J. Weimer, 2008, Glia.

A novel porcine model of CLN3 Batten disease recapitulates clinical phenotypes

Tyler B. Johnson, D. Pearce, C. Rogers, 2022, bioRxiv.

A Novel Porcine Model of CLN2 Batten Disease that Recapitulates Patient Phenotypes

Tyler B. Johnson, H. Johnson, D. Pearce, 2022, Neurotherapeutics.

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

D. Meyerholz, B. Darbro, Xiao-jun Wang, 2015, Human molecular genetics.

Modulation of CRMP2 via (S)-Lacosamide shows therapeutic promise but is ultimately ineffective in a mouse model of CLN6-Batten disease

Ki Duk Park, R. Khanna, J. Weimer, 2019, Health psychology and behavioral medicine.

Endolysosomal N-glycan processing is critical to attain the most active form of the enzyme acid alpha-glucosidase

M. O. Sheikh, Nickita Mehta, J. Weimer, 2021, The Journal of biological chemistry.

High-resolution promoter interaction analysis in Type 3 Innate Lymphoid Cells implicates Batten Disease gene CLN3 in Crohn’s Disease aetiology

M. Weirauch, C. Wallace, P. Fraser, 2022, bioRxiv.

Human adipose-derived stromal vascular fraction: characterization, safety and therapeutic potential in an experimental mouse model of articular injury.

J. Weimer, Pedro Negrao de Assis, Jordan A. Dykstra, 2020, Journal of stem cells & regenerative medicine.

On the cusp of cures: Breakthroughs in Batten disease research

J. Weimer, J. Brudvig, 2021, Current Opinion in Neurobiology.

High-resolution promoter interaction analysis in Type 3 Innate Lymphoid Cells implicates Batten Disease gene CLN3 in Crohn’s Disease aetiology

M. Weirauch, C. Wallace, P. Fraser, 2022, bioRxiv.

Sex-dependent differences in pain and sleep in a porcine model of Neurofibromatosis type 1

D. Meyerholz, D. Quelle, B. Darbro, 2018 .

Sex-split analysis of pathology and motor-behavioral outcomes in a mouse model of CLN8-Batten disease reveals an increased disease burden and trajectory in female Cln8mnd mice

Tyler B. Johnson, K. Meyer, S. Likhite, 2022, Orphanet Journal of Rare Diseases.

Tracking sex-dependent differences in a mouse model of CLN6-Batten disease

Tyler B. Johnson, A. Kloth, J. Weimer, 2019, Orphanet Journal of Rare Diseases.

Mutations in VLDLR associated with ataxia with secondary vitamin E deficiency

J. Parboosingh, M. Kruer, J. Weimer, 2013, Movement disorders : official journal of the Movement Disorder Society.

Glycerophosphoinositol is Elevated in Blood Samples From CLN3Δex7-8 pigs, Cln3Δex7-8 Mice, and CLN3-Affected Individuals

Tyler B. Johnson, F. Porter, J. Weimer, 2022, Biomarker insights.

Collagen has a unique SEC24 preference for efficient export from the endoplasmic reticulum

Jinoh Kim, S. Boyadjiev, J. Weimer, 2021, bioRxiv.

Transmembrane Batten disease proteins interact with a shared network of vesicle sorting proteins to regulate synaptic composition and function

Tyler B. Johnson, K. Roux, J. Weimer, 2021, bioRxiv.

Reinduction of ErbB2 in astrocytes promotes radial glial progenitor identity in adult cerebral cortex.

H. T. Ghashghaei, E. Anton, K. McCarthy, 2007, Genes & development.

identity in adult cerebral cortex Reinduction of ErbB 2 in astrocytes promotes radial glial progenitor data

H. T. Ghashghaei, E. Anton, K. McCarthy, 2007 .

Cerebellar defects in a mouse model of juvenile neuronal ceroid lipofuscinosis

J. Cooper, D. Pearce, Charlie C. Pontikis, 2009, Brain Research.

Concise Review: Fat and Furious: Harnessing the Full Potential of Adipose‐Derived Stromal Vascular Fraction

K. Francis, J. Weimer, Jordan A. Dykstra, 2017, Stem cells translational medicine.

CLN3, the protein associated with batten disease: Structure, function and localization

D. Pearce, J. Weimer, Jared W. Benedict, 2005, Journal of neuroscience research.

Longitudinal phenotype development in a minipig model of neurofibromatosis type 1

D. Meyerholz, D. Quelle, B. Darbro, 2020, Scientific Reports.

A human model of Batten disease shows role of CLN3 in phagocytosis at the photoreceptor–RPE interface

Tyler B. Johnson, J. Mink, V. Bonilha, 2021, Communications Biology.

A human model of Batten disease shows role of CLN3 in phagocytosis at the photoreceptor–RPE interface

Tyler B. Johnson, J. Mink, David S. Williams, 2021, Communications biology.

Opening Pandora's jar: a primer on the putative roles of CRMP2 in a panoply of neurodegenerative, sensory and motor neuron, and central disorders.

R. Sultana, Sarah M. Wilson, R. Khanna, 2012, Future neurology.

Novel CLN3 mutation predicted to cause complete loss of protein function does not modify the classical JNCL phenotype

J. Mink, P. Rothberg, A. Leman, 2005, Neuroscience Letters.

Intracranial delivery of AAV9 gene therapy partially prevents retinal degeneration and visual deficits in CLN6-Batten disease mice

Tyler B. Johnson, J. Cooper, B. Kaspar, 2021, Molecular therapy. Methods & clinical development.

A tailored Cln3Q352X mouse model for testing therapeutic interventions in CLN3 Batten disease

Tyler B. Johnson, D. Pearce, Logan M Langin, 2020, Scientific Reports.

Short-Term Administration of Mycophenolate Is Well-Tolerated in CLN3 Disease (Juvenile Neuronal Ceroid Lipofuscinosis).

J. Mink, F. Marshall, E. Augustine, 2019, JIMD reports.

Searching for novel biomarkers using a mouse model of CLN3-Batten disease

T. Kielian, D. Pearce, J. Weimer, 2018, PloS one.

Selectively increased sensitivity of cerebellar granule cells to AMPA receptor-mediated excitotoxicity in a mouse model of Batten disease

D. Pearce, J. Weimer, A. Kovács, 2006, Neurobiology of Disease.

Moving towards effective therapeutic strategies for Neuronal Ceroid Lipofuscinosis

T. Kielian, D. Pearce, J. Weimer, 2016, Orphanet Journal of Rare Diseases.

Neuronal Ceroid Lipofuscinosis Type 6 (CLN6) clinical findings and molecular diagnosis: Costa Rica’s experience

J. Weimer, J. Brudvig, A. Sanabria-Castro, 2022, Orphanet Journal of Rare Diseases.

Characterization of a novel porcine model of CLN3-Batten disease

Tyler B. Johnson, J. Cooper, A. Drack, 2019, Molecular Genetics and Metabolism.

A tailored mouse model of CLN2 disease: A nonsense mutant for testing personalized therapies

D. Pearce, Jill M Weimer, David A Pearce, 2017, PloS one.

Tumor-infiltrating nerves create an electro-physiologically active microenvironment and contribute to treatment resistance

Alex P. Rickel, Zhongkui Hong, R. Drapkin, 2020, bioRxiv.

Abbreviated Exposure to Hypoxia Is Sufficient to Induce CNS Dysmyelination, Modulate Spinal Motor Neuron Composition, and Impair Motor Development in Neonatal Mice

Moses Rodriguez, A. Denic, J. Weimer, 2015, PloS one.

Characterization of a recurrent missense mutation in the forkhead DNA-binding domain of FOXP1

Tyler B. Johnson, Ruthellen H. Anderson, K. Roux, 2018, Scientific Reports.

Phosphorylation-dependent proteome of Marcks in ependyma during aging and behavioral homeostasis in the mouse forebrain

H. T. Ghashghaei, K. Adler, D. Muddiman, 2022, GeroScience.

Alterations in striatal dopamine catabolism precede loss of substantia nigra neurons in a mouse model of juvenile neuronal ceroid lipofuscinosis

Deborah A. Ryan, J. Cooper, H. Federoff, 2007, Brain Research.

Doubling Up on Microtubule Stabilizers: Synergistic Functions of Doublecortin-like Kinase and Doublecortin in the Developing Cerebral Cortex

E. Anton, J. Weimer, E. S. Anton, 2006, Neuron.

Mutations in QARS, encoding glutaminyl-tRNA synthetase, cause progressive microcephaly, cerebral-cerebellar atrophy, and intractable seizures.

C. Walsh, L. Zon, A. Munnich, 2014, American journal of human genetics.

Dysregulation of CRMP2 Post-Translational Modifications Drive Its Pathological Functions

D. Feinstein, R. Khanna, J. Weimer, 2019, Molecular Neurobiology.

Cancer exosomes induce tumor innervation

R. Dantzer, Alex P. Rickel, Zhongkui Hong, 2018, Nature Communications.

Diet-induced obesity prolongs neuroinflammation and recruits CCR2+ monocytes to the brain following herpes simplex virus (HSV)-1 latency in mice

J. Weimer, P. Sheridan, K. White, 2016, Brain, Behavior, and Immunity.

Deficient NRG1-ERBB signaling alters social approach: relevance to genetic mouse models of schizophrenia

E. Anton, D. Threadgill, Daekee Lee, 2009, Journal of Neurodevelopmental Disorders.

Direct visualization of microtubules using a genetic tool to analyse radial progenitor-astrocyte continuum in mice

E. Anton, J. Weimer, Kristen Stabingas, 2011, Nature communications.

X MARCKS the spot: myristoylated alanine-rich C kinase substrate in neuronal function and disease

J. Weimer, J. Brudvig, 2015, Front. Cell. Neurosci..

Expression profiles of multiple genes in single neurons of Alzheimer's disease.

P. Coleman, C. Cox, N. Chow, 1998, Proceedings of the National Academy of Sciences of the United States of America.

RABL6A Is an Essential Driver of MPNSTs that Negatively Regulates the RB1 Pathway and Sensitizes Tumor Cells to CDK4/6 Inhibitors

D. Meyerholz, D. Quelle, B. Darbro, 2020, Clinical Cancer Research.

Immune transgene-dependent myocarditis in macaques after systemic administration of adeno-associated virus expressing human acid alpha-glucosidase

S. Tuske, James M. Wilson, J. Chichester, 2023, Frontiers in Immunology.

Phosphorylated CRMP2 Regulates Spinal Nociceptive Neurotransmission

Ki Duk Park, R. Khanna, J. Weimer, 2018, Molecular Neurobiology.

Early postnatal administration of an AAV9 gene therapy is safe and efficacious in CLN3 disease

Tyler B. Johnson, B. Kaspar, K. Meyer, 2023, Frontiers in Genetics.

Nonsense pathogenic variants in exon 1 of PHOX2B lead to translational reinitiation in congenital central hypoventilation syndrome

K. Roux, D. Kim, M. Landsverk, 2017, American journal of medical genetics. Part A.

Cancer exosomes induce tumor innervation

R. Dantzer, Alex P. Rickel, Zhongkui Hong, 2018, Nature Communications.

MARCKS Is Necessary for Netrin-DCC Signaling and Corpus Callosum Formation

D. Stumpo, P. Blackshear, K. Roux, 2018, Molecular Neurobiology.

Magnetic resonance brain volumetry biomarkers of CLN2 Batten disease identified with miniswine model

H. Johnson, J. Sieren, C. Rogers, 2023, Scientific Reports.

Phosphorylated CRMP2 Regulates Spinal Nociceptive Neurotransmission

Ki Duk Park, R. Khanna, J. Weimer, 2018, Molecular Neurobiology.

Protracted CLN3 Batten disease in mice that genetically model an exon-skipping therapeutic approach

D. Wallace, J. Weimer, Jessica L. Centa, 2023, Molecular therapy. Nucleic acids.

Longitudinal phenotype development in a minipig model of neurofibromatosis type 1

D. Meyerholz, D. Quelle, B. Darbro, 2020, Scientific Reports.

Novel variants identified in CKAP2L in two siblings with Filippi syndrome

M. Landsverk, J. Weimer, Ryan J. Patrick, 2021, Cold Spring Harbor molecular case studies.

Clathrin assembly protein AP-2 is detected in both neurons and glia, and its reduction is prominent in layer II of frontal cortex in Alzheimer’s disease

P. Coleman, P. Yao, J. Weimer, 2000, Neurobiology of Aging.