[HTML][HTML] Importing mitochondrial proteins: machineries and mechanisms

A Chacinska, CM Koehler, D Milenkovic, T Lithgow… - Cell, 2009 - cell.com
Most mitochondrial proteins are synthesized on cytosolic ribosomes and must be imported
across one or both mitochondrial membranes. There is an amazingly versatile set of …

Mitochondrial protein import dysfunction: mitochondrial disease, neurodegenerative disease and cancer

CS Palmer, AJ Anderson, D Stojanovski - FEBS letters, 2021 - Wiley Online Library
The majority of proteins localised to mitochondria are encoded by the nuclear genome, with
approximately 1500 proteins imported into mammalian mitochondria. Dysfunction in this …

[HTML][HTML] Interaction between AIF and CHCHD4 regulates respiratory chain biogenesis

E Hangen, O Feraud, S Lachkar, H Mou, N Doti… - Molecular cell, 2015 - cell.com
Apoptosis-inducing factor (AIF) is a mitochondrial flavoprotein that, beyond its apoptotic
function, is required for the normal expression of major respiratory chain complexes. Here …

MIA40 is an oxidoreductase that catalyzes oxidative protein folding in mitochondria

L Banci, I Bertini, C Cefaro, S Ciofi-Baffoni… - Nature structural & …, 2009 - nature.com
MIA40 has a key role in oxidative protein folding in the mitochondrial intermembrane space.
We present the solution structure of human MIA40 and its mechanism as a catalyst of …

[HTML][HTML] A two-step mitochondrial import pathway couples the disulfide relay with matrix complex I biogenesis

E Peker, K Weiss, J Song, C Zarges… - The Journal of Cell …, 2023 - ncbi.nlm.nih.gov
Mitochondria critically rely on protein import and its tight regulation. Here, we found that the
complex I assembly factor NDUFAF8 follows a two-step import pathway linking IMS and …

[HTML][HTML] Redox-mediated regulation of mitochondrial biogenesis, dynamics, and respiratory chain assembly in yeast and human cells

S Geldon, E Fernández-Vizarra… - Frontiers in cell and …, 2021 - frontiersin.org
Mitochondria are double-membrane organelles that contain their own genome, the
mitochondrial DNA (mtDNA), and reminiscent of its endosymbiotic origin. Mitochondria are …

[HTML][HTML] The disulfide relay system of mitochondria is connected to the respiratory chain

K Bihlmaier, N Mesecke, N Terziyska… - The Journal of cell …, 2007 - ncbi.nlm.nih.gov
All proteins of the intermembrane space of mitochondria are encoded by nuclear genes and
synthesized in the cytosol. Many of these proteins lack presequences but are imported into …

[HTML][HTML] Mitochondrial proteins containing coiled-coil-helix-coiled-coil-helix (CHCH) domains in health and disease

N Modjtahedi, K Tokatlidis, P Dessen… - Trends in Biochemical …, 2016 - cell.com
Members of the coiled-coil-helix-coiled-coil-helix (CHCH) domain-containing protein family
that carry (CX 9 C) type motifs are imported into the mitochondrion with the help of the …

Mitochondrial protein transport in health and disease

Y Kang, LF Fielden, D Stojanovski - Seminars in cell & developmental …, 2018 - Elsevier
Mitochondria are fundamental structures that fulfil important and diverse functions within
cells, including cellular respiration and iron-sulfur cluster biogenesis. Mitochondrial function …

The ubiquitin-proteasome system regulates mitochondrial intermembrane space proteins

P Bragoszewski, A Gornicka… - … and cellular biology, 2013 - Am Soc Microbiol
Mitochondrial precursor proteins are synthesized in the cytosol and subsequently imported
into mitochondria. The import of mitochondrial intermembrane space proteins is coupled …