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Titolo:
LOCALIZATION OF LAMININ SUBUNITS IN THE CENTRAL-NERVOUS-SYSTEM IN FUKUYAMA CONGENITAL MUSCULAR-DYSTROPHY - AN IMMUNOHISTOCHEMICAL INVESTIGATION
Autore:
YAMAMOTO T; SHIBATA N; KANAZAWA M; KOBAYASHI M; KOMORI T; IKEYA K; KONDO E; SAITO K; OSAWA M;
Indirizzi:
TOKYO WOMENS MED COLL,DEPT PATHOL,SHINJUKU KU,8-1 KAWADA CHO TOKYO 162 JAPAN TOKYO METROPOLITAN INST NEUROSCI,DEPT NEUROPATHOL FUCHU TOKYO 183 JAPAN TOKYO WOMENS MED COLL,DEPT PEDIAT,SHINJUKU KU TOKYO 162 JAPAN
Titolo Testata:
Acta Neuropathologica
fascicolo: 2, volume: 94, anno: 1997,
pagine: 173 - 179
SICI:
0001-6322(1997)94:2<173:LOLSIT>2.0.ZU;2-L
Fonte:
ISI
Lingua:
ENG
Soggetto:
GALLOWAY-MOWAT SYNDROME; WALKER-WARBURG SYNDROME; 50DAG A2; EXPRESSION; MUSCLE; MEROSIN; GLYCOPROTEINS; DYSPLASIA; NEPHROSIS; MIGRATION;
Keywords:
LAMININ; DYSTROPHIN-ASSOCIATED GLYCOPROTEIN; CENTRAL NERVOUS SYSTEM; FUKUYAMA CONGENITAL MUSCULAR DYSTROPHY;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Science Citation Index Expanded
Science Citation Index Expanded
Science Citation Index Expanded
Citazioni:
38
Recensione:
Indirizzi per estratti:
Citazione:
T. Yamamoto et al., "LOCALIZATION OF LAMININ SUBUNITS IN THE CENTRAL-NERVOUS-SYSTEM IN FUKUYAMA CONGENITAL MUSCULAR-DYSTROPHY - AN IMMUNOHISTOCHEMICAL INVESTIGATION", Acta Neuropathologica, 94(2), 1997, pp. 173-179

Abstract

We have undertaken an immunohistochemical study of laminin subunits in the central nervous system (CNS) of fetuses and patients with Fukuyama congenital muscular dystrophy (FCMD) and of controls including fivefetuses. Immunoreaction product deposits with antibodies to laminin alpha 1, alpha 2, beta 1 and gamma 1, and beta-dystroglycan were detected on the surface and vessels of the CNS of controls. No staining withanti-alpha-sarcoglycan antibody was detected in the CNS. Neurons and glia did not react with any of the antibodies used. In utero expression of laminin subunits and beta-dystroglycan seemed to be lower in the cerebrum than in the spinal cord. Moreover, immunostaining for lamininalpha 2 and beta 1 tended to be weak on the fetal spinal cord surface. Expression of laminin subunits and dystrophin-associated proteins inthe CNS may be modulated during development, as in the skeletal muscle. The distribution of immunoreaction product deposits was basically the same in FCMD and controls, although laminin alpha 2 and beta-dystroglycan expression appeared to be decreased in the CNS of the FCMD cases. Defects of the pial-glial barrier of the fetal brain surface have been considered the main cause of micropolygyria in FCMD, and these observations suggest that the co-localization and secondary loss of theseproteins in association with the unknown product(s) of the FCMD gene might be involved in the CNS lesions of this disorder.

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Documento generato il 27/11/20 alle ore 00:44:25