PMID- 10575108
OWN - NLM
STAT- MEDLINE
DCOM- 19991221
LR  - 20190614
IS  - 0006-8993 (Print)
IS  - 0006-8993 (Linking)
VI  - 847
IP  - 2
DP  - 1999 Nov 20
TI  - Decline in brainstem auditory-evoked potentials coincides with loss of spiral 
      ganglion cells in arylsulfatase A-deficient mice.
PG  - 352-6
AB  - 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 and 12 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.
FAU - D'Hooge, R
AU  - D'Hooge R
AD  - Laboratory of Neurochemistry and Behaviour, Born-Bunge Foundation, and Memory 
      Clinic, Neurology Department, Middelheim Hospital, University of Antwerp, 
      Universiteitsplein 1, Antwerp, Belgium.
FAU - Coenen, R
AU  - Coenen R
FAU - Gieselmann, V
AU  - Gieselmann V
FAU - Lullmann-Rauch, R
AU  - Lullmann-Rauch R
FAU - De Deyn, P P
AU  - De Deyn PP
LA  - eng
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PL  - Netherlands
TA  - Brain Res
JT  - Brain research
JID - 0045503
RN  - EC 3.1.6.8 (Cerebroside-Sulfatase)
SB  - IM
MH  - Age Factors
MH  - Animals
MH  - Cerebroside-Sulfatase/*genetics/metabolism
MH  - Evoked Potentials, Auditory, Brain Stem/*genetics
MH  - Leukodystrophy, Metachromatic/*genetics/metabolism
MH  - Mice
MH  - Mice, Transgenic
MH  - Spiral Ganglion/metabolism/*pathology
EDAT- 1999/11/27 00:00
MHDA- 1999/11/27 00:01
CRDT- 1999/11/27 00:00
PHST- 1999/11/27 00:00 [pubmed]
PHST- 1999/11/27 00:01 [medline]
PHST- 1999/11/27 00:00 [entrez]
AID - S0006-8993(99)02085-5 [pii]
AID - 10.1016/s0006-8993(99)02085-5 [doi]
PST - ppublish
SO  - Brain Res. 1999 Nov 20;847(2):352-6. doi: 10.1016/s0006-8993(99)02085-5.