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Titolo:
Dendritic anomalies in disorders associated with mental retardation
Autore:
Kaufmann, WE; Moser, HW;
Indirizzi:
Kennedy Krieger Inst, Dept Dev Cognit Neurol, Baltimore, MD 21205 USA Kennedy Krieger Inst Baltimore MD USA 21205 urol, Baltimore, MD 21205 USA Johns Hopkins Univ, Sch Med, Dept Pathol, Baltimore, MD 21205 USA Johns Hopkins Univ Baltimore MD USA 21205 Pathol, Baltimore, MD 21205 USA Johns Hopkins Univ, Sch Med, Dept Neurol, Baltimore, MD 21205 USA Johns Hopkins Univ Baltimore MD USA 21205 Neurol, Baltimore, MD 21205 USA Johns Hopkins Univ, Sch Med, Dept Pediat, Baltimore, MD 21205 USA Johns Hopkins Univ Baltimore MD USA 21205 Pediat, Baltimore, MD 21205 USA Johns Hopkins Univ, Sch Med, Dept Psychiat & Behav Sci, Baltimore, MD 21205 USA Johns Hopkins Univ Baltimore MD USA 21205 av Sci, Baltimore, MD 21205 USA
Titolo Testata:
CEREBRAL CORTEX
fascicolo: 10, volume: 10, anno: 2000,
pagine: 981 - 991
SICI:
1047-3211(200010)10:10<981:DAIDAW>2.0.ZU;2-#
Fonte:
ISI
Lingua:
ENG
Soggetto:
RUBINSTEIN-TAYBI SYNDROME; FRAGILE-X-SYNDROME; CPG-BINDING PROTEIN-2; RETT-SYNDROME; DOWNS-SYNDROME; WILLIAMS-SYNDROME; CEREBRAL-CORTEX; TUBEROUS SCLEROSIS; PYRAMIDAL NEURONS; BRAIN-DEVELOPMENT;
Tipo documento:
Review
Natura:
Periodico
Settore Disciplinare:
Life Sciences
Citazioni:
126
Recensione:
Indirizzi per estratti:
Indirizzo: Kaufmann, WE Kennedy Krieger Inst, Dept Dev Cognit Neurol, Room 522,707 N Broadway, Baltimore, MD 21205 USA Kennedy Krieger Inst Room 522,707 N Broadway Baltimore MD USA 21205
Citazione:
W.E. Kaufmann e H.W. Moser, "Dendritic anomalies in disorders associated with mental retardation", CEREB CORT, 10(10), 2000, pp. 981-991

Abstract

Dendritic abnormalities are the most consistent anatomical correlates of mental retardation (MR). Earliest descriptions included dendritic spine dysgenesis. which was first associated with unclassified MR, but can also be found in genetic syndromes associated with MR. Genetic disorders with well-defined dendritic anomalies involving branches and/or spines include Down. Rett and fragile-X syndromes. Cytoarchitectonic analyses also suggest dendritic pathology in Williams and Rubinstein-Taybi syndromes. Dendritic abnormalities appear to have syndrome-specific pathogenesis and evolution, which correlate to some extent with their cognitive profile. The significance of dendritic pathology in synaptic circuitry and the role of animal models in the study of MR-associated dendritic abnormalities are also discussed. Finally, a model of genotype to neurologic phenotype pathway in MR, centered in dendritic abnormalities, is postulated.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 19/01/20 alle ore 09:02:58