Krainc, Dimitri, MD, PhD

Information

Name

Krainc, Dimitri, MD, PhD

Title

Aaron Montgomery Professor and Chairman

Email

krainc@northwestern.edu

Office Phone

(312) 503-3936

Office Fax

(312) 503-0872

Department

Neurology

Office

Ward 12-140

Areas of Research

Cell Biology, Molecular Neuroscience, Neurobiology of Disease

NU Scholar Profile

https://northwestern.pure.elsevier.com/en/persons/905f798e-e476-4420-887b-c09b6d718447

Current Research

Current Research

The overarching goal of my laboratory has been to define key molecular pathways in the pathogenesis of neurodegeneration. We have focused on pathogenic mechanisms that commonly occur in neurodegenerative disorders such as accumulation and deficient degradation of aggregation-prone proteins and mitochondrial dysfunction. As a general strategy, we are studying rare genetic diseases, in particular those with mutations in genes that play a role in these common pathogenic pathways (e.g. PINK1, Parkin, ATP13A2, Gaucher) with a goal of identifying specific targets for therapeutic development in neurodegeneration. To validate and study these mechanisms in patient-specific human neurons, we are developing new tools to generate purified population of specific neuronal subtypes from reprogrammed patient fibroblasts (iPS).

Selected Publications

Selected Publications

Dunah AW, Jeong H., Griffin A., Kim MJ, Standaert DG, Hersch SM, MouradianMM, Young AB, Tanese N. and Krainc D. Sp1 and TAF130 transcriptional activity disrupted in early Huntington’s Disease. Science, 2002; 296, 2238

Cui L., Jeong H., Borovecki F. Parkhurst C., Tanese, N. and Krainc D. Transcriptional Repression of PGC-1alpha by Mutant Huntingtin Leads to Mitochondrial Dysfunction and Neurodegeneration. Cell, 2006, 126, 59-69.

Jeong H., Then F., Mazzulli JR., Melia, T. Savas J., Voisine C., Tanese, N., Hart C.A., Yamamoto A. and Krainc D. Acetylation targets mutant huntingtin to autophagosomes for degradation. Cell,2009, 137, 1-13

Seibler, P., Graziotto,J., Jeong, H., Simunovic F., Klein, C. and Krainc, D. Mitochondrial Parkin recruitment is impaired in neurons derived from mutant PINK1 iPS cells. J. Neurosci 2011, 31, 5970.

Jeong, H., Cohen, D.E., Cui, L.; Supinski, A; Bordone, L; , Guarente, L.P, and Krainc, D. Sirt1 mediates neuroprotection from mutant huntingtin by activation of TORC1 and CREB transcriptional pathway, Nature Medicine (advance online publication, December 2011, doi:10.1038/nm.2558).

Mazzulli, J.R., Sun, Y., Knight, A.L., McLean, P.J., Caldwell, G, Sidransky, E, Grabowski, G.A. and Krainc, D.: Gaucher’s Disease Glucocerebrosidase and alpha-synuclein form a bidirectional pathogenic loop in synucleinopathies. Cell, 2011, 146, 1-16.