Non-human animal model for amyotrophic lateral sclerosis (ALS) with loss-of-TDP-43 function
AssigneeAcademia Sinica
InventorsChe-Kun James Shen, Lien-Szu Wu
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Abstract
A non-human animal model for amyotrophic lateral sclerosis (ALS) is disclosed. The animal model comprises a rodent whose spinal cord motor neurons have a loss of TAR-DNA binding protein-43 (TDP-43) function and phenotypes exhibit ALS-like symptoms. A method for identifying a candidate agent for treating, preventing and/or inhibiting ALS associated with a loss-of-function of TDP-43 is also disclosed.
Claims20
A conditional TDP-43 gene-knockout mouse model for amyotrophic lateral sclerosis (ALS) whose spinal cord motor neurons comprises a disrupted TDP-43 encoding gene and have a loss of TAR-DNA binding protein-43 (TDP-43) function, wherein the TDP-43-encoding gene in the spinal cord motor neurons comprises a homozygous disruption and the mouse exhibits ALS-like symptoms after the age of 13 weeks.
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