Article
Formation of advanced glycation end-product-modified superoxide dismutase-1 (SOD1) is one of the mechanisms responsible for inclusions common to familial amyotrophic lateral sclerosis patients with SOD1 gene mutation, and transgenic mice expressing human SOD1 gene mutation.
Neuropathology : official journal of the Japanese Society of Neuropathology - 1 Mar 2001
Kato S, Nakashima K, Horiuchi S, Nagai R, Cleveland D W, Liu J, Hirano A, Takikawa M, Kato M, Nakano I, Sakoda S, Asayama K, Ohama E
Abstract excerpt
Neuronal Lewy body-like hyaline inclusions (LBHI) and astrocytic hyaline inclusions (Ast-HI) are morphological hallmarks of certain familial amyotrophic lateral sclerosis (FALS) patients with superoxide dismutase-1 (SOD1) gene mutations, and transgenic mice expressing the human SOD1 gene mutation. The ultrastructure of inclusions in both diseases is identical: the essential common constituents are granule-coated...
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