Yilin Kang, Michael James Baker ... Diana Stojanovski
A novel and metazoan-specific protein, Tim29, is identified as a subunit of the human TIM22 complex and shown to function in the assembly of hTim22 and facilitate contacts with the TOM complex.
Missense mutations in the nuclear-encoded adenine nucleotide translocase 1 (Ant1) cause the protein to clog the mitochondrial protein import pathway, to severely inhibit cell growth in yeast, and to cause neurodegeneration and myopathy in mice that phenocopy ANT1-induced human disease.
Yilin Kang, Alexander J Anderson ... Diana Stojanovski
A neuronal specific function of the mitochondrial chaperone, hTim8a in Complex IV biology provides insight into the pathomechanisms underlying the mitochondrial disease, Mohr-Tranebjaerg syndrome.
Rupa Banerjee, Christina Gladkova ... Dejana Mokranjac
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.
TIM23 core deficiency specifically impacts the oxidative phosphorylation and the mitochondrial ribosome complexes and, unexpectedly, leads to reduction of plasma membrane voltage-dependent potassium channels in neurons.
See-Yeun Ting, Nicholas L Yan ... Elizabeth A Craig
The motor that drives preproteins into the mitochondrial matrix is coupled to the translocase by Tim44, a two-domain scaffold protein with an intrinsically disordered "dynamic arm" and a structurally stable anchoring domain.
Corinne von Känel, Sergio A Muñoz-Gómez ... Andre Schneider
Mitochondrial inner membrane translocation of presequence-containing proteins by the single bifunctional TIM complex of T. brucei requires an non-canonical J domain-containing protein.
Ceereena Ubaida-Mohien, Sally Spendiff ... Russell T Hepple
Skeletal muscle from world-class octogenarian athletes, representing a population with very high physical function in advanced age, exhibits greater mass and strength that is associated with overrepresentation of proteins involved in mitochondrial biology and more oxidatively competent muscle fibers.
