- Joe Gould’s Secret, by Joseph Mitchell, The New Yorker, September 26, 1964.
2. Researchers at the Howard Hughes Medical Institute at the University of Utah have discovered two genes that cause long QT syndrome, an inherited disorder that causes sudden death in young, otherwise healthy people.
In two papers published in the March 10 issue of Cell , HHMI researchers report that mutations in genes encoding proteins that comprise part of the heart's electrical system cause increased risk of sudden death from an abnormal, chaotic heart rhythm. The findings are the first to uncover the molecular basis for cardiac arrhythmias, abnormal heart beats which can disrupt blood flow to the brain and other vital organs.
Long QT syndrome causes episodes of arrhythmia, and although this specific inherited syndrome is not a common disorder, cardiac arrhythmias kill about 300,000 Americans each year, said principal investigator Mark T. Keating of HHMI at the University of Utah.
Keating’s group identified mutations in two of the heart's ion channels, which are proteins embedded in the surface of heart cells. Under normal circumstances, the channels open and close rhythmically. This process ensures an orderly flow of the chemicals necessary to sustain the heartbeat. “Both mutant genes cause an increase in the excitability of heart tissue, a dangerous situation that can prime the pump for the development of life-threatening arrhythmias,” Keating said.
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