VCP mediated mutagenesis in Danio rerio to model motor neuron degeneration in ALS

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2015-04-14
Authors
Jones, Stephen
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Altmetrics
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Genetics, Development and Cell Biology
Abstract

Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterized by the death of motor neurons. Once these nerve cells die, the patient’s muscles waste away, resulting in paralysis and eventually death. Two primary forms of ALS exist: Familial and Sporadic. Familial constitutes 5-10% of cases and is defined by ALS being present in one or more cases in a family’s lineage. Sporadic makes up 90-95% of ALS cases and is essentially when no family history exists with ALS but an individual has ALS. Mutations in SOD1 have been the most studied in regards to ALS. However there are many other genes linked to ALS that have not been studied. VCP is a gene that has been linked to several different diseases including familial versions of ALS. The protein has been linked to many different cellular processes including protein degradation and programmed cell death. To gain a better understanding into the development and eventual death of motor neurons, we are using both TAL-effector nuclease (TALEN) mediated mutagenesis and a VCP CRSPR to create zebrafish that are mutant for VCP. These mutant fish will hopefully allow us to create a new model of motor neuron degeneration or ALS.

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