33 results found
    1. Cell Biology
    2. Neuroscience

    Burst mitofusin activation reverses neuromuscular dysfunction in murine CMT2A

    Antonietta Franco et al.
    A trial of mitofusin activation shows neuron regeneration and phenotype reversal in vitro and in vivo in experimental Charcot–Marie–Tooth disease type 2A caused by mitofusin 2 mutations.
    1. Structural Biology and Molecular Biophysics
    2. Cell Biology

    A mitofusin-dependent docking ring complex triggers mitochondrial fusion in vitro

    Tobias Brandt et al.
    A combination of an in vitro mitochondrial fusion assay with electron cryo-tomography (cryo-ET) reveals distinct steps and protein complexes during mitofusin-dependent fusion between adjacent mitochondrial outer membranes.
    1. Cell Biology

    Human biallelic MFN2 mutations induce mitochondrial dysfunction, upper body adipose hyperplasia, and suppression of leptin expression

    Nuno Rocha et al.
    Specific human mitofusin 2 mutations induce selective upper body obesity with suppressed leptin expression and severe adipose mitochondrial dysfunction.
    1. Neuroscience

    Valosin-containing protein (VCP/p97) inhibitors relieve Mitofusin-dependent mitochondrial defects due to VCP disease mutants

    Ting Zhang et al.
    A potential medication has been found for the brain and muscle degenerative diseases caused by mutant forms of Valosin-Containing Protein.
    1. Cell Biology
    2. Neuroscience

    MUL1 acts in parallel to the PINK1/parkin pathway in regulating mitofusin and compensates for loss of PINK1/parkin

    Jina Yun et al.
    By reducing mitochondrial fusion, MUL1 compensates for the mutations in PINK1 or parkin that underlie certain cases of Parkinson's disease.
    1. Biochemistry and Chemical Biology
    2. Cell Biology

    Aim24 and MICOS modulate respiratory function, tafazzin-related cardiolipin modification and mitochondrial architecture

    Max Emanuel Harner et al.
    Interactions between Aim24 and the mitochondrial outer and inner membrane contact site complex (MICOS) are essential for mitochondrial ultrastructure, lipid composition and oxidative phosphorylation.
    1. Developmental Biology
    2. Neuroscience

    Mitochondrial fusion but not fission regulates larval growth and synaptic development through steroid hormone production

    Hector Sandoval et al.
    The dual role of Drosophila Mitofusin in steroid hormone production and cholesterol ester storage, which is evolutionary conserved by the combined expression of the two mammalian Mitofusins, ensures proper synaptic development.
    1. Biochemistry and Chemical Biology
    2. Cell Biology

    Cdc48 regulates a deubiquitylase cascade critical for mitochondrial fusion

    Tânia Simões et al.
    A signaling pathway—comprising a linear sequence of the ubiquitin-selective chaperone Cdc48/p97 and the deubiquitylases Ubp12 and Ubp2—synergistically regulates mitochondrial fusion, thereby fine-tuning ubiquitylation of the mitofusin Fzo1.
    1. Medicine

    Late-life restoration of mitochondrial function reverses cardiac dysfunction in old mice

    Ying Ann Chiao et al.
    Mitochondrial-targeted SS-31 peptide ameliorates mitochondrial dysfunction and rescues pre-existing cardiac dysfunction in old mice, supporting the translational potential of mitochondrial protective interventions to treat age-related diseases.
    1. Cell Biology
    2. Developmental Biology

    Mitochondrial fusion is required for spermatogonial differentiation and meiosis

    Grigor Varuzhanyan et al.
    Mitochondrial fusion enables a metabolic transition during spermatogenesis.

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