Barth syndrome is caused by mutations in the TAFAZZIN gene, which encodes the cardiolipin remodeling enzyme, tafazzin. Barth patients are characterized as sarcopenic, a musculoskeletal condition associated with loss of lean mass and decline of functional physical capabilities. Along with other symptoms, Barth patients exhibit exercise intolerance associated with cardiac alterations and a greater tendency for muscle wasting. Our lab previously showed that loss of tafazzin causes decreased ATP synthesis and decreased flux of glucose to the TCA cycle. In muscles, ATP is required to manage cardiac and skeletal muscle contraction, calcium homeostasis, and muscle fiber regeneration. Thus, decreased ATP production and deficient energy metabolism have been linked to muscle fatigue/wasting and abnormal protein degradation. Degradation of amino acids through protein catabolism can increase energy production as a means to replenish TCA cycle intermediates. My focus is to determine if tafazzin deficiency leads to diminished ATP production via alterations in the metabolism of proteins and amino acids.
More about Ramim:
I am from Bangladesh. I completed my bachelor’s and master’s in microbiology from the University of Dhaka, Bangladesh. I am passionate about hiking, trekking, mountaineering, traveling, and photography. I love to visit and explore new places and meet new people to learn about them and their culture. When I am not in the lab, I like to work out in the gym or play outdoor sports like volleyball, cricket, tennis, badminton, and soccer. I also like to partake in extreme sports, including skydiving, skiing, etc.
