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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

1986Residency, Vanderbilt University
1987Fellowship, University of Pennsylvania
1988Postdoctoral Fellowship, Institut Suisse de Recherches Experimentales sur le Cancer, Lausanne
1992Postdoctoral Fellowship, University of Pennsylvania

Education/Academic qualification

MD, University of Rochester

… → 1982

Research interests

  • Arrhythmias
  • Cellular Electrophysiology
  • Epilepsy
  • Genetics
  • Genomics
  • Ion Channels
  • Neurogenetics
  • Pharmacology

Fingerprint Dive into the research topics where Alfred L George Jr is active. These topic labels come from the works of this person. Together they form a unique fingerprint.

  • 12 Similar Profiles
Sodium Channels Medicine & Life Sciences
Mutation Medicine & Life Sciences
Long QT Syndrome Medicine & Life Sciences
Cardiac Arrhythmias Medicine & Life Sciences
Genes Medicine & Life Sciences
Skeletal Muscle Medicine & Life Sciences
Chloride Channels Medicine & Life Sciences
Myotonic Disorders Medicine & Life Sciences

Network Recent external collaboration on country level. Dive into details by clicking on the dots.

Grants 2014 2023

Induced Pluripotent Stem Cells
Antisense Oligonucleotides
Neurons
In Vitro Techniques

Project 2

Burridge, P., George Jr, A. L. & Potet, F.

American Heart Association

7/1/196/30/23

Project: Research project

Epilepsy
Brain Diseases
Mutation
Drug Approval
Pharmacology
Channelopathies
Epilepsy
Research
Ion Channels
Neurons

Research Output 1978 2019

2 Citations (Scopus)

Calmodulin mutations and life-threatening cardiac arrhythmias: Insights from the International Calmodulinopathy Registry

Crotti, L., Spazzolini, C., Tester, D. J., Ghidoni, A., Baruteau, A. E., Beckmann, B. M., Behr, E. R., Bennett, J. S., Bezzina, C. R., Bhuiyan, Z. A., Celiker, A., Cerrone, M., Dagradi, F., De Ferrari, G. M., Etheridge, S. P., Fatah, M., Garcia-Pavia, P., Al-Ghamdi, S., Hamilton, R. M., Al-Hassnan, Z. N. & 21 others, Horie, M., Jimenez-Jaimez, J., Kanter, R. J., Kaski, J. P., Kotta, M. C., Lahrouchi, N., Makita, N., Norrish, G., Odland, H. H., Ohno, S., Papagiannis, J., Parati, G., Sekarski, N., Tveten, K., Vatta, M., Webster, G., Wilde, A. A. M., Wojciak, J., George, A. L., Ackerman, M. J. & Schwartz, P. J., Sep 14 2019, In : European heart journal. 40, 35, p. 2964-2975A

Research output: Contribution to journalArticle

Calmodulin
Registries
Cardiac Arrhythmias
Mutation
Phenotype
1 Citation (Scopus)

Cardiac Evaluation of Children With a Family History of Sudden Death

Webster, R. G., Olson, R., Schoppen, Z. J., Giancola, N., Balmert, L. C., Cherny, S. & George Jr, A. L., Aug 13 2019, In : Journal of the American College of Cardiology. 74, 6, p. 759-770 12 p.

Research output: Contribution to journalArticle

Sudden Death
Heart Diseases
Odds Ratio
Pediatrics
Tertiary Care Centers
Open Access
Dystonia
Brain Diseases
Electrophysiology
Movement Disorders
Cricetulus

Genetic Mosaicism in Calmodulinopathy

Wren, L. M., Jiménez-Jáimez, J., Al-Ghamdi, S., Al-Aama, J. Y., Bdeir, A., Al-Hassnan, Z. N., Kuan, J. L., Foo, R. Y., Potet, F., Johnson, C. N., Aziz, M. C., Carvill, G. L., Kaski, J. P., Crotti, L., Perin, F., Monserrat, L., Burridge, P., Schwartz, P. J., Chazin, W. J., Bhuiyan, Z. A. & 1 others, George Jr, A. L., Sep 1 2019, In : Circulation. Genomic and precision medicine. 12, 9, p. 375-385 11 p.

Research output: Contribution to journalArticle

Open Access
Mosaicism
Calmodulin
Mutation
Fetal Death
Cardiac Arrhythmias
1 Citation (Scopus)
Myoclonic Epilepsy
GABA-A Receptors
Seizures
Inhibitory Postsynaptic Potentials
Hypnotics and Sedatives