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The basic science aspect of motor neuron degeneration research at Columbia University is divided into animal studies and human studies. Animal Studies
The final path of motor neuron degeneration appears to include this active cell death process. The cause of ALS may be multi-factorial but the detailed understanding of cell degeneration will help to develop new treatment strategies directed at the level of cell death, counteracting the cell death process. In the animal model of ALS, basic research also involves revealing the mechanism of inflammation involving microglial (special inflammatory cells in nervous system). Microglial inflammation appears to induce chemicals which are toxic to motor neuron survival. Apoptosis and inflammation may be closely tied to cell death. Dr. Przedborski’s research is focused on teasing out these two processes. Based on this research the development of new therapeutic agents — initially tested in the animal model of ALS -- are hoped to be tested in humans. One theory of ALS is that motor neuron degeneration is caused by an energy crisis in motor nerve cells. Minute cell organelles called mitochondria (also known as the "powerhouse" of the cell) are a key source of cellular energy production. It has been demonstrated that mitochondria are potentially abnormal in ALS motor neurons. The Columbia University mitochondrial research team, headed by : Salvatore DiMauro, MD and Michio Hirano, MD has contributed immensely to the progress of mitochondria in many disease conditions. Presently, we are collaborating with Dr.’s Flint Beale and Giovanni Manfredi (NY Cornell) to further determine the mechanism of mitochondrial abnormality in ALS. Autopsy Studies Human studies of motor neuron degeneration are research through our extensive ALS tissue bank. The tissue bank located at Columbia University is an essential part of on-going research into the causes of ALS. Blood, brain and spinal cord tissue from ALS patients are collected through autopsy for use in ALS research. It is through autopsy and tissue analysis that much of the understanding of ALS has been learned and has resulted in the development of potential therapeutic new treatments for ALS. The autopsy/tissue bank program is headed up by Arthur P. Hays, MD in the department of neuropathology. Research of human tissue is focused on cytoskeletal and mitochondrial changes as well as biochemical analysis significant for various inclusion bodies in diseased motor neurons. Autopsy and tissue donation continue to be an essential component of basic science to finding a cure for ALS Gene Strudies Additionally, in humans, Ali Naini, PhD is attempting to find new ALS mutations, heads genetic studies in ALS. We have expanded our research in this area to include other ALS Centers. Presently, Dr. Naini conducts SOD testing and has identified additional mutations in ALS. An integral part of ALS genetic studies is DNA banking. This will allow for future analysis of blood samples from ALS patients (not limited to familial ALS), which is essential to gaining a greater understanding of the genetic mechanism of all ALS motor neuron degeneration.
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In animal models of ALS, 
