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Titolo:
Decline in brainstem auditory-evoked potentials coincides with loss of spiral ganglion cells in arylsulfatase A-deficient mice
Autore:
DHooge, R; Coenen, R; Gieselmann, V; Lullmann-Rauch, R; De Deyn, PP;
Indirizzi:
Born Bunge Fdn, Lab Neurochem & Behav, B-2610 Antwerp, Belgium Born Bunge Fdn Antwerp Belgium B-2610 m & Behav, B-2610 Antwerp, Belgium Univ Antwerp, Dept Neurol, Middelheim Hosp, Memory Clin, B-2610 Antwerp, Belgium Univ Antwerp Antwerp Belgium B-2610 Memory Clin, B-2610 Antwerp, Belgium Univ Kiel, Dept Anat, Kiel, Germany Univ Kiel Kiel GermanyUniv Kiel, Dept Anat, Kiel, Germany Univ Kiel, Dept Biochem, Kiel, Germany Univ Kiel Kiel GermanyUniv Kiel, Dept Biochem, Kiel, Germany
Titolo Testata:
BRAIN RESEARCH
fascicolo: 2, volume: 847, anno: 1999,
pagine: 352 - 356
SICI:
0006-8993(19991120)847:2<352:DIBAPC>2.0.ZU;2-S
Fonte:
ISI
Lingua:
ENG
Soggetto:
METACHROMATIC LEUKODYSTROPHY; MOLECULAR-GENETICS; STEM; RESPONSES;
Keywords:
metachromatic leukodystrophy; arylsulfatase A; brainstem auditory-evoked potential; spiral ganglion; transgenic mouse;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Life Sciences
Citazioni:
14
Recensione:
Indirizzi per estratti:
Indirizzo: De Deyn, PP Born Bunge Fdn, Lab Neurochem & Behav, B-2610 Antwerp, BelgiumBorn Bunge Fdn Antwerp Belgium B-2610 -2610 Antwerp, Belgium
Citazione:
R. D'Hooge et al., "Decline in brainstem auditory-evoked potentials coincides with loss of spiral ganglion cells in arylsulfatase A-deficient mice", BRAIN RES, 847(2), 1999, pp. 352-356

Abstract

Arylsulfatase A (ASA)-deficient mice constitute an animal model for the inherited lysosomal storage disease, metachromatic leukodystrophy (MLD). Brainstem auditory-evoked potentials (BAEPs) were recorded in control and ASA-deficient mice of 3, 6, 9 and 12 months. BAEPs were evoked in control mice of all ages studied, but were completely absent in ASA (- / -) mice of 9 and12 months. A significant delay in the wave pattern was noted in 6-month-old ASA (- / -) mice, Histological examination and morphometric analysis showed that the decline of BAEPs in ASA (- / -) mice was paralleled by a decrease in spiral ganglion cell numbers. (C) 1999 Elsevier Science B.V. All rights reserved.

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Documento generato il 19/01/20 alle ore 09:08:29