Molecular signature pathway of gene protein interaction in human mitochondrial DNA (mtDNA) metabolism linked disease

Mitochondrial DNA (mtDNA) contains approximately 37 numbers genes which encoded functional proteins that are essential for cellular energy production and regulation of biochemical process within human. Therefore, any kind of mtDNA abnormalities can initiate an energy calamity, ensuing in crucial mitochondrial diseases. It also depends on nuclear genes because its protein products properly help in mtDNA replication and maintenances of dNTP pools within cytosol and mitochondrial matrix part. Mitochondrial DNA dNTP synthesis comes from two broad categories: primarily mitochondrial salvage and cytosolic de novo pathways with the direct help towards some kind of factors and a variety of enzymes. However, defective mtDNA maintenance can lead to severe disease in humans which may have poor curative therapeutic accessible approaches. Existing research supports that a vast number of drugs and inhibitor components have played significant roles in regulating the mtDNA as well as dNTP pool alteration. The review will primarily focus on the protein regulatory pathways of mitochondrial deoxyribonucleotide (mtDNA) metabolism i.e. mtDNA synthesis, maintenances and its degradation with the connection of several mtDNA metabolism linked diseases.