Recent Submissions

  • Pharmacological aspects of the inhibition of mammalian respiratory complex I 

    Serreli, Riccardo (2018-05-19)
    Mitochondrial complex I, a large respiratory enzyme located in the inner mitochondrial membrane, catalyses electron transfer from NADH to ubiquinone while concomitantly translocating protons across the membrane to sustain ...
  • Mitochondrial oxodicarboxylate carrier deficiency is associated with mitochondrial DNA depletion and spinal muscular atrophy-like disease 

    Boczonadi, Veronika; King, Martin; Smith, Anthony Colin; Olahova, Monika; Bansagi, Boglarka; Roos, Andreas; Eyassu, Filmon et al.
    Purpose: Members of the mitochondrial carrier family (SLC25) transport metabolites, nucleotides, co-factors and inorganic ions across the mitochondrial inner membrane. Methods: We identified a pathogenic variant in a novel ...
  • MitoCore: a curated constraint-based model for simulating human central metabolism. 

    Smith, Anthony Colin; Eyassu, Filmon; Mazat, Jean-Pierre; Robinson, Alan Jonathan (BioMed Central, 2017-11-25)
    Background: The complexity of metabolic networks can make the origin and impact of changes in central metabolism occurring during diseases difficult to understand. Computer simulations can help unravel this complexity, and ...
  • A novel de novo dominant mutation in ISCU associated with mitochondrial myopathy 

    Legati, Andrea; Reyes, Aurelio Fernando; Ceccatelli Berti, Camilla; Stehling, Oliver; Marchet, Silvia; Lamperti, Costanza; Ferrari, Alberto et al. (BMJ Publishing Group, 2017-12)
    BACKGROUND: Hereditary myopathy with lactic acidosis and myopathy with deficiency of succinate dehydrogenase and aconitase are variants of a recessive disorder characterised by childhood-onset early fatigue, dyspnoea and ...

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