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Mervyn J. Monteiro, PhD

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Mervyn J. Monteiro, PhD     

University of Maryland 

Generation and characterization of ubiquilin-2 transgenic mice models of ALS

Ubiquilin-2 mutations have been shown to cause ALS. To understand the molecular mechanisms by which the mutations cause disease we propose to generate and characterize transgenic mice carrying select human ubiquilin-2 mutations that we have found affect the synthesis, folding and degradation of proteins. The mice will be characterized using a battery of biochemical, pathological and behavioral examinations. Once developed these mice will provide useful models to test and validate candidate approaches to slow and/or halt ALS.


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Steven Finkbeiner

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Gladstone Institutes, UCSF
Two recently discovered genes that have been associated with both familial and sporadic forms of ALS encode the related proteins TDP43 and FUS cause neuron death in ALS.
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