Biochemistry and Pharmacology - Research Publications

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Author: Anderson, Alexander × Author: Stroud, David × End date: 2019 × Start date: 2010 × Type: Journal Article ×

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    Mitochondria-hubs for regulating cellular biochemistry: emerging concepts and networks
    Anderson, AJ ; Jackson, TD ; Stroud, DA ; Stojanovski, D (ROYAL SOC, 2019-08)
    Mitochondria are iconic structures in biochemistry and cell biology, traditionally referred to as the powerhouse of the cell due to a central role in energy production. However, modern-day mitochondria are recognized as key players in eukaryotic cell biology and are known to regulate crucial cellular processes, including calcium signalling, cell metabolism and cell death, to name a few. In this review, we will discuss foundational knowledge in mitochondrial biology and provide snapshots of recent advances that showcase how mitochondrial function regulates other cellular responses.
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    Function of hTim8a in complex IV assembly in neuronal cells provides insight into pathomechanism underlying Mohr-Tranebjaerg syndrome
    Kang, Y ; Anderson, AJ ; Jackson, TD ; Palmer, CS ; De Souza, DP ; Fujihara, KM ; Stait, T ; Frazier, AE ; Clemons, NJ ; Tull, D ; Thorburn, DR ; McConville, MJ ; Ryan, MT ; Stroud, DA ; Stojanovski, D (ELIFE SCIENCES PUBLICATIONS LTD, 2019-11-04)
    Human Tim8a and Tim8b are members of an intermembrane space chaperone network, known as the small TIM family. Mutations in TIMM8A cause a neurodegenerative disease, Mohr-Tranebjærg syndrome (MTS), which is characterised by sensorineural hearing loss, dystonia and blindness. Nothing is known about the function of hTim8a in neuronal cells or how mutation of this protein leads to a neurodegenerative disease. We show that hTim8a is required for the assembly of Complex IV in neurons, which is mediated through a transient interaction with Complex IV assembly factors, in particular the copper chaperone COX17. Complex IV assembly defects resulting from loss of hTim8a leads to oxidative stress and changes to key apoptotic regulators, including cytochrome c, which primes cells for death. Alleviation of oxidative stress with Vitamin E treatment rescues cells from apoptotic vulnerability. We hypothesise that enhanced sensitivity of neuronal cells to apoptosis is the underlying mechanism of MTS.