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Article

A Transgenic Pig Model With Human Mutant SOD1 Exhibits the Early Pathology of Amyotrophic Lateral Sclerosis.

Laboratory investigation; a journal of technical methods and pathology - 1 Feb 2023

Kondo Takayuki, Inoue Ikuyo, Umeyama Kazuhiro, Watanabe Masahito, Matsunari Hitomi, Uchikura Ayuko, Nakano Kazuaki, Tsukita Kayoko, Imamura Keiko, Nagashima Hiroshi, Inoue Haruhisa

Abstract excerpt

Amyotrophic lateral sclerosis (ALS) causes progressive degeneration of the motor neurons. In this study, we delivered the genetic construct including the whole locus of human mutant superoxide dismutase 1 (SOD1) with the promoter region of human SOD1 into porcine zygotes using intracytoplasmic sperm injection-mediated gene transfer, and we thereby generated a pig model of human mutant SOD1-mediated familial ALS....

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PMID: 37039150
DOI: 10.1016/j.labinv.2022.100013

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