**Nuclear Encoded Mitochondrial Proteins in Metabolite Transport and Oxidation Pathway Connecting Metabolism of Nutrients Transport and Oxidation Pathway Connecting Metabolism of Nutrients**

**Nuclear Encoded Mitochondrial Proteins in Metabolite** 

DOI: 10.5772/intechopen.72937

Janka Vašková, Jozef Firment and Ladislav Vaško Janka Vašková, Jozef Firment and Ladislav Vaško Additional information is available at the end of the chapter

Additional information is available at the end of the chapter

http://dx.doi.org/10.5772/intechopen.72937

#### **Abstract**

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250 Mitochondrial Diseases

[Chinese Journal of Medical Genetics]. 2017;**34**:26-29

American Journal of Human Genetics. 2011;**89**:656-667

In the mitochondria, there are ongoing processes essential to the survival of cells associated with the production of energy ending in the oxidative phosphorylation and the formation of ATP, constituting a form of energy for majority of metabolic processes. Except for nutrient oxidation in the citric acid cycle interfacing with the process of oxidative phosphorylation, mitochondria are linked to a number of metabolic pathways ongoing directly in mitochondria or indirectly in cell compartments by serving substrates. Mitochondrial activities maintenance requires continual draw of intermediates from cytosol through the double mitochondrial membrane as well as transport in the reverse direction. Interconnection and regulation of all the processes are mediated by transporters and carriers, activities of which are affected by cell and body requirements. In the chapter, the main transport systems localized in membranes of mitochondria, their regulation, affection, and disorders in the background of mitochondria aberrant functions are described. Voltage-dependent anion channels, translocase of mitochondrial outer membrane, deoxynucleotide carrier, ADP/ ATP nucleotide translocase, and phosphate carrier in mitochondrial inner membrane are among them. In more detail, the pyruvate carrier and its abnormal activity, but also others as di- and tri-carboxylate, glutamate, and ornithine carriers, are characterized. The uncoupling protein, as solute carrier family members, involvement is also mentioned.

**Keywords:** carrier, mitochondria, mitochondrial inner membrane, mitochondrial outer membrane, transporter
