Mitochondrial translation in animal cells involves the synthesis of proteins specifically within mitochondria. Mitochondria are considered semiautonomous organelles as they contain their own circular DNA (mtDNA) encoding a limited number of essential genes that primarily encode components of the oxidative phosphorylation system, critical for ATP production. The process of mitochondrial translation differs significantly however from those of nuclear genes, involving distinct molecular machinery and mechanisms. Firstly, the mitochondrial genetic code is different from the universal genetic code used in cytoplasmic/nuclear translation. Some amino acid codons are reassigned in mitochondria, leading to differences in protein synthesis. For instance, UGA, which serves as a stop codon in the cytoplasm, is often used as a tryptophan codon in mitochondria. Additionally, mitochondria encode a more limited set of tRNAs and lack certain amino acids, making them dependent on the import of nuclear-encoded tRNAs and amino acids. Mitochondrial ribosomes (also known as mitoribosomes), are responsible for translating mtDNA-encoded genes into mitochondrial proteins. Mitoribosomes are structurally distinct from cytoplasmic ribosomes with a unique protein composition. In animal cells, mitoribosomes consist of a small 28S subunit and a large 39S subunit, compared to the 40S and 60S subunits of cytoplasmic ribosomes. These differences can be exploited in research to selectively inhibit mitochondrial translation for experimental purposes. Mitochondrial transcription results in polycistronic RNA molecules, with multiple genes transcribed into a single transcript. Polycistronic transcripts are then processed into individual mRNAs that encode specific mitochondrial proteins. The transcription and processing of mitochondrial RNA are again performed by specialized mitochondrial RNA polymerases and processing enzymes. Although mtDNA encodes a small number of proteins, most mitochondrial proteins are encoded by nuclear genes and synthesized in the cytoplasm. These nuclear-encoded proteins are then transported into the mitochondria post-translationally. The import process is mediated by specific targeting sequences, such as mitochondrial targeting sequences (MTS) or presequences, which direct the proteins to the appropriate mitochondrial compartments. In some cases, mitochondrial proteins encoded by nuclear genes are imported into the mitochondria whilst they are still being synthesized on cytoplasmic ribosomes. This so-called co-translational import process is facilitated by chaperones and import receptors within the mitochondrial outer membrane. Nascent polypeptide chains are recognized by these receptors and translocated into the mitochondria in a membrane potential-dependent manner. Once mitochondrial proteins are imported, they are assembled into functional complexes within the mitochondria. These protein complexes play crucial roles in oxidative phosphorylation, the electron transport chain, and other mitochondrial metabolic functions. Defects in the assembly of these complexes can therefore lead to mitochondrial dysfunction and are associated with various mitochondrial diseases in man. We provide a wide product range of research reagents for investigating mitochondrial translation, including MRPL18 antibodies. Explore our full mitochondrial translation product range below and discover more, for less.