Article
A GM1 gangliosidosis mutant mouse model exhibits activated microglia and disturbed autophagy.
Experimental biology and medicine (Maywood, N.J.) - 1 Jun 2021
Liu Sichi, Feng Yuyu, Huang Yonglan, Jiang Xiaoling, Tang Chengfang, Tang Fang, Zeng Chunhua, Liu Li
Abstract excerpt
GM1 gangliosidosis is a rare lysosomal storage disease caused by a deficiency of β-galactosidase due to mutations in the GLB1 gene. We established a C57BL/6 mouse model with Glb1G455R mutation using CRISPR/Cas9 genome editing. The β-galactosidase enzyme activity of Glb1G455R mice measured by fluorometric assay was negligible throughout the whole body. Mutant mice displayed no marked phenotype at eight weeks....
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