Michael T. Ryan
Monash University
Publications 196
Background Atypical haemolytic uraemic syndrome (aHUS) is a rare, potentially life-threatening condition caused by dysregulation of the complement pathway. Eculizumab is currently the only approved treatment for this disorder.
2 CitationsSource
#1Luke E. Formosa (Monash University)H-Index: 7
#2Linden Muellner-Wong (Monash University)H-Index: 1
Last.Michael T. Ryan (Monash University)H-Index: 60
view all 8 authors...
Mitochondrial Complex I harbors 7 mitochondrial and 38 nuclear-encoded subunits. Its biogenesis requires the assembly and integration of distinct intermediate modules, mediated by numerous assembly factors. The Mitochondrial Complex I Intermediate Assembly (MCIA) complex, containing assembly factors NDUFAF1, ECSIT, ACAD9, and TMEM126B, is required for building the intermediate ND2-module. The role of the MCIA complex and the involvement of other proteins in the biogenesis of this module is uncle...
#1Shadi Maghool (La Trobe University)
#2N. Dinesha G. Cooray (La Trobe University)
Last.Megan J. Maher (University of Melbourne)H-Index: 20
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Assembly factors play key roles in the biogenesis of many multi-subunit protein complexes regulating their stability, activity, and the incorporation of essential cofactors. The human assembly factor Coa6 participates in the biogenesis of the CuA site in complex IV (cytochrome c oxidase, COX). Patients with mutations in Coa6 suffer from mitochondrial disease due to complex IV deficiency. Here, we present the crystal structures of human Coa6 and the pathogenic W59CCoa6-mutant protein. These struc...
#1Daniella H. Hock (University of Melbourne)
#2Boris Reljic (University of Melbourne)H-Index: 6
Last.David A. Stroud (University of Melbourne)H-Index: 24
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Assembly factors play a critical role in the biogenesis of the mitochondrial respiratory chain, which includes complexes I-IV. Complexes I, III and IV are found in a high molecular weight structure known as the respiratory chain supercomplex or respirasome. A number of proteins are frequently suggested to be required for supercomplex assembly, including the homolog of hypoxia inducible gene 1 domain family member HIGD2A which has been shown to stabilize the respirasome in yeast. We show that los...
#1Yilin Kang (University of Melbourne)H-Index: 5
#2Alexander J. Anderson (University of Melbourne)
Last.Diana Stojanovski (University of Melbourne)H-Index: 27
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Human Tim8a is a member of an intermembrane space chaperone network, known as the small TIM family, which transport hydrophobic membrane proteins through this compartment. Mutations in TIMM8A cause a neurodegenerative disease, Mohr-Tranebjaerg syndrome (MTS), which is characterised by sensorineural hearing loss, dystonia and blindness. Nothing is known about the function of hTim8a in neuronal cells and consequently how lack of hTim8a leads to a neurodegenerative disease. We identified a novel ce...
#1Shen Y. Heazlewood (Monash University)H-Index: 8
#2Tanveer Ahmad (Monash University)
Last.Susan K. Nilsson (Monash University)H-Index: 33
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Traditionally, megakaryocytes (MK) are known for platelet generation; with their role as regulators of hematopoietic stem cells (HSC) only recently being appreciated. While it is generally accepted high ploidy MK produce platelets, we have now identified that the vast majority of platelets are generated from a novel sub-population of MK, large cytoplasmic MK (LCM). When prospectively isolated from mouse marrow and transplanted, LCM readily form platelets in vivo. We have further characterized LC...