mouse model

October 30, 2018October 30, 2018

CLN3 Deletion Affects Genes Responsible for Production of Type of Fat in Brain, Mouse Study Finds

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A genetic deletion in the CLN3 gene impairs the normal function of genes involved in the production of a type of fat critical for nerve cell growth and survival in the brain, ... Read more

September 21, 2018September 21, 2018

Search for Biomarkers in CLN3 Mouse Model Fails to Yield Results, Study Reports

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An animal study using a mouse model of CLN3 disease, the juvenile form of Batten disease, failed to identify any new biomarkers that could be used to track disease progression, ... Read more

August 28, 2018August 28, 2018

Nerve Cells’ Dysfunction May Contribute to Neurodegeneration in Several Types of Batten’s, Mouse Study Reveals

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Two types of nerve cells — microglia and astrocytes — are dysfunctional and may contribute to neurodegeneration associated with different types of neuronal ceroid lipofuscinoses (NCLs, aka Batten disease), ... Read more

June 15, 2018June 15, 2018

New Mouse Model of Late Infantile Batten Disease May Help Assess Potential Therapies

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A new mouse model of late infantile Batten disease may help to efficiently investigate personalized therapies, a study suggests. The research, “A tailored mouse model of CLN2 disease: A nonsense ... Read more

May 22, 2018May 22, 2018

Protein in Specific Cells Involved in Batten Disease Neurodegeneration, Mouse Study Suggests

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Therapeutic approaches that target production of a protein called progranulin in specific brain cells may help preserve brain function in Batten disease, according to a mouse study. The study, “Conditional loss of progranulin ... Read more

April 24, 2018April 24, 2018

Several Compounds Found to Correct Brain Defects in Mouse Model of CLN3 Disease

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Several compounds were able to correct defects in the brain caused by juvenile neuronal ceroid lipofuscinosis (CLN3) in both cellular and mouse models, uncovering potential therapeutic candidates for treating the disease. Because ... Read more

April 10, 2018April 10, 2018

Gene Therapy Prevents Vision Loss Caused by CLN6 Disease in Mouse Study

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Gene therapy directly targeting a specific type of neuron in the retina of the eye was able to prevent vision loss in late infantile Batten disease caused by rare CLN6 ... Read more

March 16, 2018March 16, 2018

New Mouse Model and Gene-Editing Tool May Help Advance CLN2 Research

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A genetically modified mouse with increased levels of a specific enzyme in the brain, along with a new genetic tool, may help researchers further their understanding of late infantile neuronal ... Read more

February 23, 2018March 23, 2018

Metallothioneins Show Promise as Therapeutic Targets in Mouse Model of Batten Disease

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A family of enzymes called metallothioneins potentially could be new therapeutic agents in the treatment of lysosomal storage disorders (LSDs), including Batten disease, a mouse model shows. The study, ... Read more

December 5, 2017

Mouse Model Helps Researchers Identify Processes Underlying Batten Disease Symptoms

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Researchers found, in a new mouse model for Batten disease, that defective synapses in specific brain regions may be the cause of disease symptoms. The study “Defective synaptic transmission ... Read more

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