EL MITOCONDRI

Els mitocondris són orgànuls presents en totes les cèŀlules nucleades eucariotes que
transformen energia per impulsar reaccions ceŀlulars, i per això la integritat
mitocondrial és essencial per a la vida, i el trastorn de la seva funció pot veure’s traduït
en malaltia. Tot i que l’origen de la cèŀlula eucariota és, encara, motiu de controvèrsia,
nombrosos estudis filogenètics donen suport a la teoria que aquesta és el producte d’una
sèrie de processos endosimbiòtics produïts a través d’evolució horitzontal, per la fusió,
o fusions, entre una cèŀlula arqueobacteriana i una cèŀlula eubacteriana (Sagan, 1967;
Margulis, 1981; Cavalier-Smith, 1987; Margulis, 1993). En general, es considera que el
mitocondri actual té un origen monofilètic, que prové d’un ancestre eubacterià de vida
lliure (protomitocondri) que va ser captat per una cèŀlula hoste; tot i així, l’origen
d’aquesta última encara està en ple debat (Gray et al., 1999; Davidov i Jurkevitch,
2009).

Mitochondrial Gene Translation and Related Diseases

Gene translation is a universal process that occurs in all
eukaryotic and prokaryotic organisms and it consists of the
consecutive decoding of the consecutive decoding of the
information contained in mRNA molecules (messenger
ribonucleic acid) in the form of a polypeptide chain, in
accordance with/following carefully the rules laid down by the
genetic code. The standard genetic code, with some exceptions, is
used by all living beings, and consists of the interrelationship of
the different triplets of mRNA nucleotides, termed codons, with
the 20 amino acids used in protein synthesis.
Gene translation requires the participation of many proteins and
RNA molecules working in a coordinated manner. The mRNA
codons do not directly recognise the specified amino acids, but
they do it through some adaptor molecules relating thoroughly
each mRNA triplet to the corresponding amino acid. The tRNA
(transfer RNA) are those adaptor molecular. In the cell there is at
least one tRNA for each amino acid, but in many cases, several
isoacceptors are able to recognise different codons having the
same amino acid uniquely or redundantly. The tRNA are
specifically aminoacylated with the assigned amino acid by the
aminoacyl tRNA synthetase (aaRS). This family of enzymes
consists of at least twenty protein, one for each amino acid of the
standard genetic code (Ribas de Pouplana and Schimmel, 2001).
Gene translation, both prokaryotic and eukaryotic, can be divided
into two man stages: aminoacylation of tRNA and ribosomal
translation. The first stage is based on the specific recognition and
activation of the amino acid made by the aaRS, and on the
addition of the amino acid to the 3’-terminal of the tRNA that’s
going to join. Once the tRNA is aminoacylated, it is directed to
the ribosome by the initiation or elongation factors, a complex
consisting of proteins related to the rRNA molecules (ribosomal
RNA), where the mechanical interaction between tRNA
aminoacylates and mRNA triplets occurs, and that constitutes the
centerpiece of protein synthesis.

Mitochondria

Mitochondria are organelles present in all nucleated eukaryotic

cells transforming energy to trigger cellular reactions. Because of
that, mitochondrial integrity is essential for life, and the disorder
of its function can be translated into disease. Although the origin
of the eukaryotic cell is still controversial, numerous phylogenetic
studies support the theory that it is the product of a series of
endosymbiotic processes produced through horizontal evolution,
by fusion or fusions between cell of archaebacteria and a cell of
eubacteria (Sagan, 1967; Margulis, 1981; Cavalier-Smith, 1987;
Margulis, 1993). In general, the current mitochondria is
considered to have a monophyletic origin, which comes from an
ancestor of a free-living eubacteria (proto-mitochondrion) that
was engulfed by a host cell. Even so, the origin of this last one is
still under discussion (Gray et al., 1999; Davidov and Jurkevitch,
2009).
https://www.ncbi.nlm.nih.gov/books/NBK9849/