1992 Fiscal Year Final Research Report Summary
Immunological Study for the Pathogenesis of Adrenoleukodystrophy
| Project/Area Number |
03670413
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| Research Category |
Grant-in-Aid for General Scientific Research (C)
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| Allocation Type | Single-year Grants |
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| Research Field |
Neurology
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| Research Institution | Niigata University |
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Principal Investigator |
TANAKA Keiko Niigata University Department of Neurology, 医学部附属病院, 助手 (30217020)
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| Co-Investigator(Kenkyū-buntansha) |
MIYATAKE Tadashi Tokyo Medical & Dental University Department of Neurology Professor, 医学部附属病院, 教授 (50048998)
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| Project Period (FY) |
1991 – 1992
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| Keywords | adrenoleukodystrophy / demyelination / monoclonal antibody / lymphocyte / macrophage / immunohistochemistry / neopterin / Xq28 |
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| Research Abstract |
Adrenoleukodystrophy(ALD) is a rare disorder caused by the defective metabolism of very long chain fatty aced. The pathological alterations in ALD are rapidly progressing demyelination in the central nervous system with prominent mononuclear cell infiltration. We tried to characterize the infiltrating cells immunohistochemically using several kinds of monoclonal antibodies for lymphocytes and macrophages which are responsible for demyelination and revealed that T8 and macrophages are prominently seen around the vessels. The infiltrating macrophages are thought to be activated and have a primary role for progressing demyelination. Activated macrophages are known to release large amount of neopterin as the result of GTP cyclohydrolase activation. We measured the neopterin content in the cerebrospinal fluid of ALD patients in the rapidly progressing stage using high performance liquid chromatography which resulted no increase of neopterin. These results suggested that the macrophages did not have the primary role for demyelination, rather the secondary role for clearing tissue debris.
It is important to investigate the responsible gene for ALD which might solve the pathophysiological mechanism of demyelination in ALD brain. ALD gene in known to locate on the Xq28, also suggested to be near the red color pigment gene. Positional cloning focusing on this area is in progress.
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[Publications] Koike,R.,Tsuji,S.,Ohono,T.,Suzuki,Y.,Orii,T.,Miyatake,T.: "Physiological sionificance of fatty acid elongation system in adrenoleukodystrophy." J.Neurol.Sci.103. 188-194 (1991)
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[Publications] Kondo,R.,Wakamatsu,N.,Yoshino,H.,Fukuhara,N.,Miyatake,T.,Tsuji,S.: "Identification of a mutation in the arylsulfatse A gene of a patient with adult-type metachromatic leukodystrophy." Am J Hum Genet.48. 971-978 (1991)
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[Publications] Tanno,Y.,Yoneda,M.,Nonaka,I.,Tanaka,K.,Miyatake,T.,Tsuji,S.: "Quantitation of mitochondrial DNA carrying tRNA^<Lys> mutation in MERRF patients." Biochem Biophys Res Commun. 179. 880-885 (1991)
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