ノイラミニダーゼ欠損症のげっ歯類モデル

Neuraminidases are crucial enzymes that cleave terminal sialic acid residues from sialylated glycoconjugates. Their presence varies across tissues, cellular locations, and substrate specificities in different mammalian species, assigning each neuraminidase distinct functions. Rodent models have significantly contributed to the field of sialobiology, and the absence of these enzymes allows researchers to investigate their roles in degrading oligosaccharides, glycoproteins, and glycolipids. Additionally, these models aid in understanding diseases associated with neuraminidases, such as lysosomal storage disorders and neurodegenerative diseases, thereby supporting the development of new therapies. This review highlights key discoveries from studies of neuraminidase-deficient animal models. SIGNIFICANCE STATEMENT: Neuraminidases are essential for degrading sialic acid-containing biomolecules, and their absence is associated with the development of various neurodegenerative disorders.