Properties of Human Mitochondrial Ribosomes

@article{Obrien2003PropertiesOH,
  title={Properties of Human Mitochondrial Ribosomes},
  author={Thomas W O'brien},
  journal={IUBMB Life},
  year={2003},
  volume={55}
}
Mammalian mitochondrial ribosomes (55S) differ unexpectedly from bacterial (70S) and cytoplasmic ribosomes (80S), as well as other kinds of mitochondrial ribosomes. Typical of mammalian mitochondrial ribosomes, the bovine mitochondrial ribosome has been developed as a model system for the study of human mitochondrial ribosomes, to address several questions related to the structure, function, biosynthesis and evolution of these interesting ribosomes. Bovine mitochondrial ribosomal proteins (MRPs… 
Structure of the large ribosomal subunit from human mitochondria
TLDR
The structure of the human mitochondrial ribosome reveals how it is adapted to synthesize mitochondrial membrane proteins and unveils an adaptation of the exit tunnel for hydrophobic nascent peptides and extensive remodeling of the central protuberance.
The discovery of mitochondrial ribosomes
TLDR
All of the MRPs are candidates for mitochondrial disease and they actually contain smaller RNAs than bacterial ribosomes, which is due to the presence of several extra proteins which are unique to mitochondrial ribosome.
Mitochondrial ribosome assembly in health and disease
TLDR
This article focuses on summarizing the current available information on mitoribosome assembly pathway, factors that form the mitoribergsome assembly machinery, and the effect of defective mitorIBosomeAssembly assembly on human health.
Regulatory Roles of Mitochondrial Ribosome in Lung Diseases and Single Cell Biology.
TLDR
The structure, elements, functions, and regulatory roles in associated diseases, including mitochondrial ribosomal RNA (mt-rRNA) variations can affect the mitochondria in developing disease are summarized.
Import of ribosomal proteins into yeast mitochondria.
TLDR
This review article summarizes the current knowledge on the transport of proteins into the mitochondrial matrix, and thereby specifically focus on proteins of the mitochondrial ribosome.
Trypanosoma brucei Mitochondrial Ribosomes
Although eukaryotic mitochondrial (mt) ribosomes evolved from a putative prokaryotic ancestor their compositions vary considerably among organisms. We determined the protein composition of tandem
Reconstructing the evolution of the mitochondrial ribosomal proteome
TLDR
The evolutionary history of the mitoribosomal proteome is reconstructed, revealing an ancestral ribosome of alpha-proteobacterial descent that more than doubled its protein content in most eukaryotic lineages.
Ribosome Biogenesis and Cancer: Overview on Ribosomal Proteins
TLDR
The main steps of ribosome biogenesis alterations occurring during tumorigenesis are summarized, highlighting the changes in the expression level of cytosolic ribosomal proteins (CRPs) and mitochondrial ribosom proteins (MRPs) in different types of tumors.
...
...

References

SHOWING 1-10 OF 49 REFERENCES
Mitochondrial ribosomal proteins (MRPs) of yeast.
TLDR
Surprisingly, only a minority of the MRPs that have been characterized show significant sequence similarities to known ribosomal proteins from other sources, thus limiting the deduction of their functions by simple comparison of amino acid sequences.
Structure and Function of Mammalian Mitochondrial Ribosomes
TLDR
The 55S ribosomes from mammalian mitochondria have an intrinsic GTP binding site in the small subunit, an unprecedented occurrence in translational systems (Denslow et al., 1991).
Evolution of proteins in mammalian cytoplasmic and mitochondrial ribosomes
TLDR
It is significant that mammalian mitochondrial r- Proteins are evolving more rapidly than cytoplasmic r-proteins in the same cell, since both sets of r-Protein are encoded by nuclear genes.
Mammalian Mitochondrial Ribosomal Proteins
TLDR
To identify mammalian mitochondrial ribosomal proteins and their corresponding genes, mature rat MRPs were purified and 12 different N-terminal amino acid sequences were determined and screened for corresponding DNA sequences to identify the genes or to establish consensus cDNAs.
Structural Compensation for the Deficit of rRNA with Proteins in the Mammalian Mitochondrial Ribosome
TLDR
The results showed that the proteins with binding sites on rRNA shortened or lost in the mitoribosome were enlarged when compared with the E. coli counterparts; this suggests the structural compensation of the rRNA deficit by the enlarged proteins in themitorIBosome.
Ribosomes from rat liver mitochondira. II. Partial characterization.
Mammalian mitochondrial ribosomal proteins (4). Amino acid sequencing, characterization, and identification of corresponding gene sequences.
TLDR
The Mammalian Mitochondrial Ribosomal Consortium is using a direct approach of purifying individual mammalian (bovine) MRPs, determining their N-terminal and/or internal peptide sequences using different protein sequencing techniques, and using the resulting sequence information for screening expressed sequence tags and genomic data bases to determine human, mouse, and rat homologues of the bovine proteins.
Proteomic Analysis of the Mammalian Mitochondrial Ribosome
TLDR
A mitochondrial homologue of S12 is clearly identified, which is a key regulatory protein of translation fidelity and a candidate for the autosomal dominant deafness gene, DFNA4, indicating a new function for the mitoribosome in programmed cell death.
...
...