Grants per year
Personal profile
Research Interests
Our research program is focused on the structure, function, pharmacology, and molecular genetics of ion channels. His laboratory has contributed greatly to understanding the mechanisms by which ion channel mutations cause a variety of inherited disorders of membrane excitability including congenital cardiac arrhythmia susceptibility and epilepsy. These basic and translational investigations have provided many opportunities to investigate the relationship between structure and function in ion channels and have helped establish important genotype-phenotype correlations for several human diseases and certain animal models. We were first to elucidate the functional consequences of an ion channel mutation linked to an inherited cardiac arrhythmia (Long-QT Syndrome). This discovery contributed greatly to understanding arrhythmia susceptibility in the disease and inspired use of drugs targeting persistent sodium current as a therapeutic strategy. Recently, the use of exome sequencing enabled us to discover human calmodulin mutations in congenital arrhythmia syndromes. Our studies of the molecular basis for genetic epilepsy have revealed new targets for antiepileptic drug development.
Training Experience
1986 | Residency, Vanderbilt University |
1987 | Fellowship, University of Pennsylvania |
1988 | Postdoctoral Fellowship, Institut Suisse de Recherches Experimentales sur le Cancer, Lausanne |
1992 | Postdoctoral Fellowship, University of Pennsylvania |
Expertise related to UN Sustainable Development Goals
In 2015, UN member states agreed to 17 global Sustainable Development Goals (SDGs) to end poverty, protect the planet and ensure prosperity for all. This person’s work contributes towards the following SDG(s):
Education/Academic qualification
MD, Medicine, University of Rochester
… → 1982
Research interests keywords
- Arrhythmias
- Cellular Electrophysiology
- Epilepsy
- Genetics
- Genomics
- Ion Channels
- Neurogenetics
- Pharmacology
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Collaborations and top research areas from the last five years
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Role of Endothelin-1 signaling in the development and progression of atrial fibrillation
Pfenniger, A., Arora, R. K., George, A. L., Lee, D. C., Passman, R. S., Roy-Puckelwartz, M. J., Shah, S. J., Vaughan, D. E. & Wasserstrom, J. A.
National Heart, Lung, and Blood Institute
9/1/23 → 8/31/28
Project: Research project
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Administrative Core - Main
George, A. L., Judge, S. M. & Quaggin, S. E.
National Institute of Diabetes and Digestive and Kidney Diseases
9/1/23 → 6/30/28
Project: Research project
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Determining Effects of BVH-7000 on Diverse KCNQ2 Pathogenic Variants
Biohaven Pharmaceuticals, Inc.
8/30/23 → 8/29/24
Project: Research project
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SyncroPatch 384 Automated Patch Clamp Instrument
George, A. L., Burridge, P., DeCaen, P. G., Horvath, C. M., Kearney, J. A., Kiskinis, E., McNally, E. M., Miller, R. J., Prakriya, M., Smith, R. S., Surmeier Jr, D. J. & Vanoye, C. G.
Office of the Director, National Institutes of Health
3/1/23 → 2/29/24
Project: Research project
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Decrypting Variants of Uncertain Significance in Long-QT Syndrome
George, A. L., George, A. L., Vanoye, C. G. & Vanoye, C. G.
National Heart, Lung, and Blood Institute
8/1/22 → 7/31/26
Project: Research project
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Epilepsy-associated SCN2A (NaV1.2) variants exhibit diverse and complex functional properties
Thompson, C. H., Potet, F., Abramova, T. V., DeKeyser, J. M., Ghabra, N. F., Vanoye, C. G., Millichap, J. J. & George, A. L., Oct 2 2023, In: Journal of General Physiology. 155, 10, e202313375.Research output: Contribution to journal › Article › peer-review
Open Access1 Scopus citations -
Outlining cardiac ion channel protein interactors and their signature in the human electrocardiogram
Maurya, S., Mills, R. W., Kahnert, K., Chiang, D. Y., Bertoli, G., Lundegaard, P. R., Duran, M. P. H., Zhang, M., Rothenberg, E., George, A. L., MacRae, C. A., Delmar, M. & Lundby, A., Jul 2023, In: Nature Cardiovascular Research. 2, 7, p. 673-692 20 p.Research output: Contribution to journal › Article › peer-review
Open Access -
Prophecy or empiricism? Clinical value of predicting versus determining genetic variant functions
Brunklaus, A., George, A. L., Lal, D., Heinzen, E. L. & Goldman, A. M., Nov 2023, In: Epilepsia. 64, 11, p. 2909-2913 5 p.Research output: Contribution to journal › Comment/debate › peer-review
Open Access -
RNA-based translation activators for targeted gene upregulation
Cao, Y., Liu, H., Lu, S. S., Jones, K. A., Govind, A. P., Jeyifous, O., Simmons, C. Q., Tabatabaei, N., Green, W. N., Holder, J. L., Tahmasebi, S., George, A. L. & Dickinson, B. C., Dec 2023, In: Nature communications. 14, 1, 6827.Research output: Contribution to journal › Article › peer-review
Open Access -
Scanning mutagenesis of the voltage-gated sodium channel NaV1.2 using base editing
Pablo, J. L. B., Cornett, S. L., Wang, L. A., Jo, S., Brünger, T., Budnik, N., Hegde, M., DeKeyser, J. M., Thompson, C. H., Doench, J. G., Lal, D., George, A. L. & Pan, J. Q., Jun 27 2023, In: Cell reports. 42, 6, 112563.Research output: Contribution to journal › Article › peer-review
Open Access
Datasets
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2NDJ : Structural Basis for KCNE3 and Estrogen Modulation of the KCNQ1 Channel
Kroncke, B. M. (Contributor), Van Horn, W. D. (Contributor), Smith, J. (Contributor), Kang, C. (Contributor), Welch, R. C. (Contributor), Song, Y. (Contributor), Nannemann, D. P. (Contributor), Sisco, N. J. (Contributor), George, A. L. (Contributor), Meiler, J. (Contributor), Vanoye, C. G. (Contributor) & Sanders, C. R. (Contributor), RCSB-PDB, 2016
DOI: 10.2210/pdb2ndj/pdb, https://www.rcsb.org/structure/2NDJ
Dataset