Rate of accumulation of Luxol Fast Blue staining material and mitochondrial ATP synthase subunit 9 in motor neuron degeneration mice.
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.
Rodman
J S
JS
Department of Physiology, Tufts University School of Medicine, Boston, MA 02111, USA.
Lipman
R
R
Brown
A
A
Bronson
R T
RT
Dice
J F
JF
D000818:Animals; D004396:Coloring Agents / Q000378:metabolism*; D004195:Disease Models, Animal; D006624:Hippocampus / Q000201:enzymology / Q000378:metabolism; D007211:Indoles / Q000378:metabolism*; D051379:Mice; D008810:Mice, Inbred C57BL; D008817:Mice, Mutant Strains; D008928:Mitochondria / Q000201:enzymology*; D009046:Motor Neurons / Q000473:pathology*; D009472:Neuronal Ceroid-Lipofuscinoses / Q000201:enzymology / Q000378:metabolism*; D006180:Proton-Translocating ATPases / Q000378:metabolism*
D004396:Coloring Agents; D007211:Indoles; C018588:Luxol Fast Blue MBS; D006180:Proton-Translocating ATPases