Mitochondrial protein import dysfunction: mitochondrial disease, neurodegenerative disease and cancer
The majority of proteins localised to mitochondria are encoded by the nuclear genome, with
approximately 1500 proteins imported into mammalian mitochondria. Dysfunction in this …
approximately 1500 proteins imported into mammalian mitochondria. Dysfunction in this …
Ndufs4 knockout mouse models of Leigh syndrome: pathophysiology and intervention
MAE Van De Wal, MJW Adjobo-Hermans, J Keijer… - Brain, 2022 - academic.oup.com
Mitochondria are small cellular constituents that generate cellular energy (ATP) by oxidative
phosphorylation (OXPHOS). Dysfunction of these organelles is linked to a heterogeneous …
phosphorylation (OXPHOS). Dysfunction of these organelles is linked to a heterogeneous …
The mitochondrial outer membrane protein Tom70-mediator in protein traffic, membrane contact sites and innate immunity
S Kreimendahl, J Rassow - International journal of molecular sciences, 2020 - mdpi.com
Tom70 is a versatile adaptor protein of 70 kDa anchored in the outer membrane of
mitochondria in metazoa, fungi and amoeba. The tertiary structure was resolved for the …
mitochondria in metazoa, fungi and amoeba. The tertiary structure was resolved for the …
Blackout in the powerhouse: clinical phenotypes associated with defects in the assembly of OXPHOS complexes and the mitoribosome
Mitochondria produce the bulk of the energy used by almost all eukaryotic cells through
oxidative phosphorylation (OXPHOS) which occurs on the four complexes of the respiratory …
oxidative phosphorylation (OXPHOS) which occurs on the four complexes of the respiratory …
The mitochondrial intermembrane space: the most constricted mitochondrial sub-compartment with the largest variety of protein import pathways
The mitochondrial intermembrane space (IMS) is the most constricted sub-mitochondrial
compartment, housing only about 5% of the mitochondrial proteome, and yet is endowed …
compartment, housing only about 5% of the mitochondrial proteome, and yet is endowed …
Role of the mitochondrial protein import machinery and protein processing in heart disease
Mitochondria are essential organelles for cellular energy production, metabolic
homeostasis, calcium homeostasis, cell proliferation, and apoptosis. About 99% of …
homeostasis, calcium homeostasis, cell proliferation, and apoptosis. About 99% of …
Autosomal recessive progeroid syndrome due to homozygosity for a TOMM7 variant
Multiple genetic loci have been reported for progeroid syndromes. However, the molecular
defects in some extremely rare forms of progeria have yet to be elucidated. Here, we report a …
defects in some extremely rare forms of progeria have yet to be elucidated. Here, we report a …
Interplay between mitochondrial protein import and respiratory complexes assembly in neuronal health and degeneration
The fact that> 99% of mitochondrial proteins are encoded by the nuclear genome and
synthesised in the cytosol renders the process of mitochondrial protein import fundamental …
synthesised in the cytosol renders the process of mitochondrial protein import fundamental …
Biological colloids: Unique properties of membraneless organelles in the cell
A Bratek-Skicki, M Van Nerom, D Maes… - Advances in Colloid and …, 2022 - Elsevier
Biomolecular condensates are membraneless, intracellular organelles that form via liquid-
liquid phase separation (LLPS) and have the ability to concentrate a wide range of …
liquid phase separation (LLPS) and have the ability to concentrate a wide range of …
Split Intein-Mediated Protein Ligation for detecting protein-protein interactions and their inhibition
Z Yao, F Aboualizadeh, J Kroll, I Akula, J Snider… - Nature …, 2020 - nature.com
Here, to overcome many limitations accompanying current available methods to detect
protein-protein interactions (PPIs), we develop a live cell method called Split Intein-Mediated …
protein-protein interactions (PPIs), we develop a live cell method called Split Intein-Mediated …