1998 Fiscal Year Final Research Report Summary
Pathological characteristics of astrocytic hyaline inclusions in familial amyotrophic lateral
| Project/Area Number |
09680744
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| Research Category |
Grant-in-Aid for Scientific Research (C)
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| Allocation Type | Single-year Grants |
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| Section | 一般 |
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| Research Field |
Nerve anatomy/Neuropathology
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| Research Institution | TOTTORI UNIVERSITY |
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Principal Investigator |
KATO Shinsuke Tottori University FACULTY OF MEDICINE,ASSISTANT PROFESSOR, 医学部, 講師 (60194817)
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| Co-Investigator(Kenkyū-buntansha) |
OHAMA Eisaku Tottori University FACULTY OF MEDICINE,PROFESSOR, 医学部, 教授 (50018892)
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| Project Period (FY) |
1997 – 1998
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| Keywords | Familial amyotrophic lateral sclerosis / Astrocytic hyaline inclusion / Superoxide dismutase 1 / Granule-coated fibril / Lewy body-like hyaline inclusion / N^<epsilon>-carboxymethyl lysine / Advanced glycation endproduct / Advanced glycation endproducts |
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| Research Abstract |
To clarify the pathological characteristics of astrocytic hyaline inclusions (Ast-HI) in patients with familial amyptrophic lateral sclerosis (FALS) with the superoxide dismutase 1 (SOD1) genemutations, eight autopsies on members of four different families were analyzed. The neuronal Lewy body-like hyaline inslusion(LBHI) was also a characteristic neuropathological marker of the mutant SOD1-related FALS.Both Ast-Ill and LBHI were ultrastructurally composed of approximately 15- to 25-nm granule-coated fibrils that had immunoreactivities to SOD1 and N^<epsilon> -carboxymethyl lysine (CML, an advanced glycation endproduct). These results suggest that Ast-HI and LBHI contain SOD1 and CML, and that the formation of CML-modified SOD1 (probably CML-modified mutant SOD 1) is related to the cell degeneration.
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