TY - JOUR
T1 - Unique architectural features of mammalian mitochondrial protein synthesis
AU - Rackham, Oliver
AU - Saurer, Martin
AU - Ban, Nenad
AU - Filipovska, Aleksandra
PY - 2025/1
Y1 - 2025/1
N2 - Mitochondria rely on coordinated expression of their own mitochondrial DNA (mtDNA) with that of the nuclear genome for their biogenesis. The bacterial ancestry of mitochondria has given rise to unique and idiosyncratic features of the mtDNA and its expression machinery that can be specific to different organisms. In animals, the mitochondrial protein synthesis machinery has acquired many new components and mechanisms over evolution. These include several new ribosomal proteins, new stop codons and ways to recognise them, and new mechanisms to deliver nascent proteins into the mitochondrial inner membrane. Here we describe the mitochondrial protein synthesis machinery in mammals and its unique mechanisms of action elucidated to date and highlight the technologies poised to reveal the next generation of discoveries in mitochondrial translation.
AB - Mitochondria rely on coordinated expression of their own mitochondrial DNA (mtDNA) with that of the nuclear genome for their biogenesis. The bacterial ancestry of mitochondria has given rise to unique and idiosyncratic features of the mtDNA and its expression machinery that can be specific to different organisms. In animals, the mitochondrial protein synthesis machinery has acquired many new components and mechanisms over evolution. These include several new ribosomal proteins, new stop codons and ways to recognise them, and new mechanisms to deliver nascent proteins into the mitochondrial inner membrane. Here we describe the mitochondrial protein synthesis machinery in mammals and its unique mechanisms of action elucidated to date and highlight the technologies poised to reveal the next generation of discoveries in mitochondrial translation.
KW - mitochondria
KW - mitochondrial disease
KW - ribosomes
KW - RNA
KW - translation
UR - http://www.scopus.com/inward/record.url?scp=85195294839&partnerID=8YFLogxK
U2 - 10.1016/j.tcb.2024.05.001
DO - 10.1016/j.tcb.2024.05.001
M3 - Review article
C2 - 38853081
AN - SCOPUS:85195294839
SN - 0962-8924
VL - 35
SP - 11
EP - 23
JO - Trends in Cell Biology
JF - Trends in Cell Biology
IS - 1
ER -