Most of our knowledge regarding the process of protein import into mitochondria has come from research employing fungal systems. This review outlines recent advances in our understanding of this process in mammalian cells. In particular, we focus on the characterisation of cytosolic molecular chaperones that are involved in binding to mitochondrial-targeted preproteins, as well as the identification of both conserved and novel subunits of the import machineries of the outer and inner mitochondrial membranes. We also discuss diseases associated with defects in import and assembly of mitochondrial proteins and what is currently known about the regulation of import in mammals.
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http://dx.doi.org/10.1016/s0167-4889(02)00268-9 | DOI Listing |
Cells
January 2025
Biosciences, Faculty of Health and Life Sciences, University of Exeter, Exeter EX4 4QD, UK.
Peroxisomes are ubiquitous, dynamic, oxidative organelles with key functions in cellular lipid metabolism and redox homeostasis. They have been linked to healthy ageing, neurodegeneration, cancer, the combat of pathogens and viruses, and infection and immune responses. Their biogenesis relies on several peroxins (encoded by genes), which mediate matrix protein import, membrane assembly, and peroxisome multiplication.
View Article and Find Full Text PDFAnal Chem
January 2025
Research Center of Analytical Instrumentation, School of Mechanical Engineering, Sichuan University, Chengdu 610065, People's Republic of China.
The development of an atmospheric pressure interface (API) with a high ion transfer efficiency and wide mass range is advantageous for the performance improvement of mass spectrometry (MS) instruments. In this work, a novel ion guide, namely, the double-helix electrode ion funnel (DHE-IF), has been developed to enhance the ion transmission over a wide mass range in the rough vacuum region. The DHE-IF consists of two funnel-shaped helix electrodes.
View Article and Find Full Text PDFInt J Mol Sci
December 2024
Departamento de Biologia, Faculdade de Filosofia, Ciências e Letras de Ribeirão Preto, Universidade de São Paulo, Ribeirão Preto 14040-901, SP, Brazil.
In the flower development study, we identified SCI1 (Stigma/style Cell-cycle Inhibitor 1), a regulator of cell proliferation. SCI1 interacts with NtCDKG;2 ( Cyclin-Dependent Kinase G;2), a homolog of human CDK11, which is responsible for RanGTP-dependent microtubule stabilization, regulating spindle assembly rate. In a Y2H screening of a cDNA library using NtCDKG;2 as bait, a RanBP1 (Ran-Binding Protein 1) was revealed as its interaction partner.
View Article and Find Full Text PDFBiochemistry
January 2025
Molecular Biophysics Laboratory, Department of Biological Sciences, Indian Institute of Science Education and Research, Bhopal 462066, India.
The mitochondrial outer membrane (OMM) β-barrel proteins link the mitochondrion with the cytosol, endoplasmic reticulum, and other cellular membranes, establishing cellular homeostasis. Their active insertion and assembly in the outer mitochondrial membrane is achieved in an energy-independent yet highly effective manner by the Sorting and Assembly Machinery (SAM) of the OMM. The core SAM constituent is the 16-stranded transmembrane β-barrel Sam50.
View Article and Find Full Text PDFEMBO Rep
January 2025
LMU Munich, Biozentrum-Cell Biology, 82152, Planegg-Martinsried, Germany.
Import and assembly of mitochondrial proteins into multimeric complexes are essential for cellular function. Yet, many steps of these processes and the proteins involved remain unknown. Here, we identify a novel pathway for disulfide bond formation and assembly of mitochondrial inner membrane (IM) proteins.
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