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Hoffmann, R. A wiki for the life sciences where authorship matters. Nature Genetics (2008)
 

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Decline in brainstem auditory-evoked potentials coincides with loss of spiral ganglion cells in arylsulfatase A-deficient mice.

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.[1]

References

  1. Decline in brainstem auditory-evoked potentials coincides with loss of spiral ganglion cells in arylsulfatase A-deficient mice. D'Hooge, R., Coenen, R., Gieselmann, V., Lüllmann-Rauch, R., De Deyn, P.P. Brain Res. (1999) [Pubmed]
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