Mitochondrial protein-import machinery: correlating structure with function

MJ Baker, AE Frazier, JM Gulbis, MT Ryan - Trends in cell biology, 2007 - cell.com
Most mitochondrial proteins are synthesized in the cytosol, translocated into the organelle
and directed along specific sorting pathways. Over the past 20 years,> 30 proteins have …

Cytosolic proteostasis through importing of misfolded proteins into mitochondria

L Ruan, C Zhou, E Jin, A Kucharavy, Y Zhang, Z Wen… - Nature, 2017 - nature.com
Loss of proteostasis underlies ageing and neurodegeneration characterized by the
accumulation of protein aggregates and mitochondrial dysfunction,,,,. Although many …

Role of PINK1 binding to the TOM complex and alternate intracellular membranes in recruitment and activation of the E3 ligase Parkin

M Lazarou, SM Jin, LA Kane, RJ Youle - Developmental cell, 2012 - cell.com
Mutations in the mitochondrial kinase PINK1 and the cytosolic E3 ligase Parkin can cause
Parkinson's disease. Damaged mitochondria accumulate PINK1 on the outer membrane …

[HTML][HTML] Dual role of mitofilin in mitochondrial membrane organization and protein biogenesis

K von der Malsburg, JM Müller, M Bohnert… - Developmental cell, 2011 - cell.com
The mitochondrial inner membrane consists of two domains, inner boundary membrane and
cristae membrane that are connected by crista junctions. Mitofilin/Fcj1 was reported to be …

[HTML][HTML] Phosphatidylethanolamine and cardiolipin differentially affect the stability of mitochondrial respiratory chain supercomplexes

L Böttinger, SE Horvath, T Kleinschroth, C Hunte… - Journal of molecular …, 2012 - Elsevier
The mitochondrial inner membrane contains two non-bilayer‐forming phospholipids,
phosphatidylethanolamine (PE) and cardiolipin (CL). Lack of CL leads to destabilization of …

Membrane protein insertion through a mitochondrial β-barrel gate

AIC Höhr, C Lindau, C Wirth, J Qiu, DA Stroud, S Kutik… - Science, 2018 - science.org
INTRODUCTION The outer membranes of Gram-negative bacteria, mitochondria, and
chloroplasts characteristically contain β-barrel membrane proteins. These proteins contain …

PPTC7 antagonizes mitophagy by promoting BNIP3 and NIX degradation via SCFFBXL4

GT Nguyen-Dien, B Townsend, PG Kulkarni… - EMBO …, 2024 - embopress.org
Mitophagy must be carefully regulated to ensure that cells maintain appropriate numbers of
functional mitochondria. The SCFFBXL4 ubiquitin ligase complex suppresses mitophagy by …

[HTML][HTML] Regulation of mitochondrial protein import by cytosolic kinases

O Schmidt, AB Harbauer, S Rao, B Eyrich, RP Zahedi… - Cell, 2011 - cell.com
Mitochondria import a large number of nuclear-encoded proteins via membrane-bound
transport machineries; however, little is known about regulation of the preprotein …

Human telomerase acts as a hTR-independent reverse transcriptase in mitochondria

NK Sharma, A Reyes, P Green, MJ Caron… - Nucleic acids …, 2012 - academic.oup.com
Human telomerase reverse transcriptase (hTERT) is localized to mitochondria, as well as
the nucleus, but details about its biology and function in the organelle remain largely …

OM14 is a mitochondrial receptor for cytosolic ribosomes that supports co-translational import into mitochondria

C Lesnik, Y Cohen, A Atir-Lande, M Schuldiner… - Nature …, 2014 - nature.com
It is well established that import of proteins into mitochondria can occur after their complete
synthesis by cytosolic ribosomes. Recently, an additional model was revived, proposing that …