Abstract: A cell is said to be a unit of life since its function, make the organisms to stay alive. Cellular functions are regulated and performed by various cell organelles. One such important cell organelle is mitochondria, which is said to be power house of the cell, because mitochondria generate energy for cellular metabolism. Mitochondria, generates energy by various biochemical reactions which result in the synthesis of energy in the form of ATP (adenosine tri-phosphate). Many different enzymes, co-factors, substrate involves in the ATP synthesis are encoded by mitochondrial DNA (mtDNA). When these mtDNA gets mutated, it leads to mitochondrial diseases. Mitochondrial diseases cause biochemical malfunction of a cell. Since mitochondria are inherited from mother to offspring, mitochondrial diseases also get inherited from a carrier mother. This condition is known as maternal inheritance during fertilization. These mitochondrial diseases has no cure but can be diagnosed by prenatal screening as well as by comparing mtDNA sequence of a patient with Cambridge reference sample of mtDNA, which is the first mtDNA sequence analysed in Europe. This comparison pays way for phyogenetic analysis. Mitochondrial diseases result in mortality.
Keywords: mtDNA, ATP synthesis, maternal inheritance