12 results found
    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. Cell Biology

    MICOS coordinates with respiratory complexes and lipids to establish mitochondrial inner membrane architecture

    Jonathan R Friedman et al.
    A conserved mitochondrial inner membrane complex called MICOS works together with the mitochondrial lipid cardiolipin and respiratory complexes to direct the position, copy number and stabilization of cristae structures for efficient respiration.
    1. Biochemistry and Chemical Biology
    2. Cell Biology

    Protein translocation channel of mitochondrial inner membrane and matrix-exposed import motor communicate via two-domain coupling protein

    Rupa Banerjee et al.
    Two domains of the peripheral membrane protein Tim44 interact with two different sectors of a translocase to coordinate the translocation of proteins across the inner mitochondrial membrane.
    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. Genetics and Genomics

    Homozygous YME1L1 mutation causes mitochondriopathy with optic atrophy and mitochondrial network fragmentation

    Bianca Hartmann et al.
    Mutations affecting a nuclear encoded metalloprotease cause of a new form of mitochondriopathy, highlighting the importance of this protease for mitochondrial function in humans.
    1. Biochemistry and Chemical Biology
    2. Cell Biology

    An evidence based hypothesis on the existence of two pathways of mitochondrial crista formation

    Max E Harner et al.
    Lamellar and tubular cristae in mitochondria form by two different molecular pathways that form both involve F1FO-ATP synthase dimers and the MICOS complex, but formation of lamellar cristae also relies on Mgm1/Opa1.
    1. Neuroscience

    Peroxisomal dysfunctions cause lysosomal storage and axonal Kv1 channel redistribution in peripheral neuropathy

    Sandra Kleinecke et al.
    Dysfunctions of myelin peroxisomes cause a lysosomal storage-like disorder associated with alterations in glial and axonal membranes, which is the likely cause of nerve impairment in peroxisomal disorders.
    1. Structural Biology and Molecular Biophysics

    Two forms of Opa1 cooperate to complete fusion of the mitochondrial inner-membrane

    Yifan Ge et al.
    An in vitro reconstitution assay reveals stoichiometric levels of the short form of Opa1 work together with the long form of Opa1 to mediate efficient and fast membrane pore opening.
    1. Structural Biology and Molecular Biophysics

    Cryo-EM structures of S-OPA1 reveal its interactions with membrane and changes upon nucleotide binding

    Danyang Zhang et al.
    GTP binding induces rearrangement of S-OPA1 assembly on membrane and thus triggers membrane remodeling with reduced curvature.

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