Intracellularly produces malonate, a reversible succinate dehydrogenase inhibitor used to generate Huntington's disease models. Induces apoptotic cell death in striatal neurons.
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Methylmalonate toxicity in primary neuronal cultures.
McLaughlin et al.
Huntington's disease and the striatal medium spiny neuron: cell-autonomous and non-cell-autonomous mechanisms of disease.
Experimental therapeutics in transgenic mouse models of Huntington's disease.
Beal and Ferrante