At the Weihl Lab, our goal is to understand the molecular mechanisms of protein inclusion formation, disaggregation, and clearance in myodegenerative (skeletal muscle) and neurodegenerative diseases.

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View the most recent publications from our lab and receive an in-depth look into the research we are doing at the forefront of neurodegenerative disease.

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We believe that dysfunction in protein quality control (molecular  chaperones) and protein degradation pathways (e.g. the ubiquitin proteasome system, autophagy and endocytosis) disrupt cellular protein homeostasis resulting in degenerative phenotypes and normal aging.  We employ complementary experimental techniques such as biochemistry, cell culture, animal models, and patient tissue to achieve these goals.

Our lab is particularly interested in the pathogenesis of inclusion body myopathy, paget’s disease of the bone and frontotemporal dementia or IBMPFD, a multisystem degenerative disorder due to missense mutations in p97/VCP/cdc48.  IBMPFD muscle and brain tissue contains ubiquitinated protein inclusions associated with cellular degeneration and vacuolation.

Recent Posts

Published Research

High-protein diets increase cardiovascular risk by activating macrophage mTOR to suppress mitophagy.

Zhang X, Sergin I, Evans TD, Jeong SJ, Rodriguez-Velez A, Kapoor D, Chen S, Song E, Holloway KB, Crowley JR, Epelman S, Weihl CC, Diwan A, Fan D, Mittendorfer B, Stitziel NO, Schilling JD, Lodhi IJ, Razani B.

Nat Metab. 2020 Sep;2(9):991. doi: 10.1038/s42255-020-00291-6

PMID: 32908252

Optimizing hand-function patient outcome measures for inclusion body myositis.

Lin AY, Siener CS, Faino AV, Seiffert M, Weihl CC, Wang LH.

Neuromuscul Disord. 2020 Aug 19:S0960-8966(20)30555-1. doi: 10.1016/j.nmd.2020.08.358. Online ahead of print.

PMID: 32928647

Inhibition of DNAJ-HSP70 interaction improves strength in muscular dystrophy.

Bengoechea R, Findlay AR, Bhadra AK, Shao H, Stein KC, Pittman SK, Daw J, Gestwicki JE, True HL, Weihl CC.

J Clin Invest. 2020 May 19:136167. doi: 10.1172/JCI136167. Online ahead of print.

PMID: 32427588

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