Mitochondria: from cell death executioners to regulators of cell differentiation

Auditory neuropathy — neural and synaptic mechanisms

“…Dominant optic atrophy is one of the most frequent genetic optic atrophies, and OPA1 mutations are causal in the majority of the cases 76 . The OPA1 protein is a mitochondria targeted dynaminrelated GTPase 77,78 that is critical for mitochondrial shaping 79 . The majority of the ~200 known human OPA1 mutations (www.mitodyn.org; the MITOchondrial DYNamics variation portal) cause haploinsufficiency via truncation; these mutations are thought to cause nonsyndromic auto somal dominant optic atrophy (DOA) 80 .…”

Mitochondrial dynamics in neuronal injury, development and plasticity

“…The enzyme responsible for fission of the outer mitochondrial membrane (OMM) is dynamin-related protein-1 (Drp1), whereas mitochondrial fusion requires coordination of three enzymes. Mitofusin 1 and 2 (Mfn1 and 2) promote fusion of the OMM, whereas optic atrophy 1 (Opa1) promotes fusion of the inner mitochondrial membrane (IMM) (Otera et al, 2013;Kasahara and Scorrano, 2014) (Fig. 1).…”

Genomic heterogeneity meets cellular energetics: crosstalk between the mitochondria and the cell cycle

“…In addition to their role as the bioenergetics center of the cell, mitochondria are central to a myriad of cellular functions including iron [37] and calcium homeostasis [38] , metabolism of amino acids, lipids, nucleotides and carbohydrates, apoptosis, and a variety of signaling pathways [38][39][40][41][42] . Dysfunctions in many of these mitochondrial processes have been associated with cancer development [40,42] and chemoresistance [43] .…”