244 results found
    1. Cell Biology
    2. Human Biology and Medicine

    Mitochondrial biogenesis is transcriptionally repressed in lysosomal lipid storage diseases

    King Faisal Yambire et al.
    Transcription factors KLF2 and ETV1 repress the transcriptional program of mitochondrial biogenesis, resulting in impaired mitochondrial function in lysosomal storage diseases.
    1. Biochemistry and Chemical Biology

    The mitochondrial acyl carrier protein (ACP) coordinates mitochondrial fatty acid synthesis with iron sulfur cluster biogenesis

    Jonathan G Van Vranken et al.
    The essential function of ACP is not related to mitochondrial fatty acid and lipoic acid synthesis, but rather an evolutionarily-conserved role in iron sulfur cluster biogenesis.
    1. Developmental Biology

    Electron transport chain biogenesis activated by a JNK-insulin-Myc relay primes mitochondrial inheritance in Drosophila

    Zong-Heng Wang et al.
    An insulin-Myc feed-forward loop triggered by transient JNK boosts transcription of genes essential for mitochondrial respiration and biogenesis during early oogenesis to support massive mtDNA replication and inheritance in Drosophila.
    1. Biochemistry and Chemical Biology

    Independent evolution of functionally exchangeable mitochondrial outer membrane import complexes

    Daniela G Vitali et al.
    The convergent evolution of yeast Mim1/2 and trypanosomal pATOM36 enable the first demonstration of reciprocal functional rescue of two evolutionary unrelated mitochondrial biogenesis complexes belonging to two different eukaryotic supergroups.
    1. Genetics and Genomics

    Natural variation in sugar tolerance associates with changes in signaling and mitochondrial ribosome biogenesis

    Richard G Melvin et al.
    The optimal macronutrient composition, macronutrient space, varies strongly between closely related species and is determined by genetic changes in regulatory genes that globally reprogram gene expression profiles of metabolic pathways.
    1. Cell Biology

    Mitochondrial Rab GAPs govern autophagosome biogenesis during mitophagy

    Koji Yamano et al.
    Two GAP proteins bound to mitochondria regulate the enyzme Rab7, and thereby the expansion of the isolation membrane during mitophagy, downstream of PINK1 and Parkin, two proteins that are mutated in familial Parkinson's disease.
    1. Biochemistry and Chemical Biology
    2. Cell Biology

    The deca-GX3 proteins Yae1-Lto1 function as adaptors recruiting the ABC protein Rli1 for iron-sulfur cluster insertion

    Viktoria Désirée Paul et al.
    Two newly identified assembly factors for the ribosome-associated iron-sulfur protein Rli1 reveal a general mechanism for how the cytosolic iron-sulfur protein assembly (CIA) machinery recruits apoproteins.
    1. Biochemistry and Chemical Biology

    Role of Nfu1 and Bol3 in iron-sulfur cluster transfer to mitochondrial clients

    Andrew Melber et al.
    Mitochondrial proteins Nfu1 and BolA3 mediate the transfer of preformed [4Fe-4S] clusters within the mitochondria to recipient proteins to shield clusters from oxidative damage during transfer.
    1. Biochemistry and Chemical Biology
    2. Structural Biology and Molecular Biophysics

    Cation selectivity of the presequence translocase channel Tim23 is crucial for efficient protein import

    Niels Denkert et al.
    Channel characteristics of the presequence translocation pore have a direct impact on protein import into the mitochondrial matrix.

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