Faculty Research 1990 - 1999

Title

Rate of accumulation of Luxol Fast Blue staining material and mitochondrial ATP synthase subunit 9 in motor neuron degeneration mice.

Document Type

Article

Publication Date

1998

Keywords

Disease-Models-Animal, Dyes: me, H(+)-Transporting-ATP-Synthase: me, Hippocampus: en, me, Indoles: me, Mice, Mice-Inbred-C57BL, Mice-Mutant-Strains, Mitochondria: en, Motor-Neurons: pa, Neuronal-Ceroid-Lipofuscinosis: en, me, SUPPORT-U-S-GOVT-P-H-S

JAX Source

Neurochem Res 1998 Oct;23(10):1291-6

Grant

NS30153/NS/NINDS, NS30148/NS/NINDS

Abstract

The rate of accumulation of Luxol Fast Blue staining material in the hippocampus of motor neuron degeneration (mnd/mnd) mice, a model of Batten Disease, was quantitated. Stained material increased linearly up to 8 months of age. A quantitative immunoassay was used to measure levels of mitochondrial ATP synthase subunit 9 in brain and liver of mnd/mnd mice. Levels of subunit 9 increased progressively throughout the lifespan of mnd/mnd mice reaching levels approximately 5-fold higher than in control animals. The rate of accumulation of subunit 9 is not consistent with any simple complete or partial degradation defect that is constant throughout the animal's life. Two more complicated models are discussed which are consistent with the observed accumulation rate of subunit 9.

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