A5021358192- Mitochondrial Function and Pathology
- Metabolism and Genetic Disorders
- ATP Synthase and ATPases Research
- Adipose Tissue and Metabolism
- Pluripotent Stem Cells Research
- RNA and protein synthesis mechanisms
- DNA Repair Mechanisms
- RNA modifications and cancer
- Peroxisome Proliferator-Activated Receptors
- Epigenetics and DNA Methylation
- Genetics and Neurodevelopmental Disorders
- Cancer, Hypoxia, and Metabolism
- Molecular Biology Techniques and Applications
- Bacteriophages and microbial interactions
- CRISPR and Genetic Engineering
- Biochemical Acid Research Studies
- Neonatal and fetal brain pathology
- Coenzyme Q10 studies and effects
- Glioma Diagnosis and Treatment
- Genetic Syndromes and Imprinting
- Alzheimer's disease research and treatments
- Ubiquitin and proteasome pathways
- RNA Research and Splicing
- Genetic Neurodegenerative Diseases
- Metalloenzymes and iron-sulfur proteins
Deakin University
2019-2025
West Virginia University
2025
Valley Hospital Medical Center
2024
Corning (United States)
2024
Hudson Institute of Medical Research
2014-2022
Monash University
2013-2022
Monash Institute of Medical Research
2011-2015
La Trobe University
2006-2012
The University of Melbourne
2000-2012
Centre for Eye Research Australia
2012
Complex I of the respiratory chain is composed at least 45 subunits that assemble together mitochondrial inner membrane. Defects in human complex result energy generation disorders and are also implicated Parkinson's disease altered apoptotic signaling. The assembly this poorly understood complicated by its large size regulation two genomes, with seven encoded DNA (mtDNA) remainder nuclear genes. Here we analyzed a number mtDNA- nuclear-gene-encoded into I. We found mtDNA-encoded first...
Mitochondrial DNA (mtDNA) encodes key proteins of the electron transfer chain (ETC), which produces ATP through oxidative phosphorylation (OXPHOS) and is essential for cells to perform specialised functions. Tumor-initiating use aerobic glycolysis, a combination glycolysis low levels OXPHOS, promote rapid cell proliferation tumor growth. Glioblastoma multiforme (GBM) an aggressively malignant brain mitochondria have been proposed play vital role in GBM tumorigenesis. Using next generation...
DNA methylation is an essential mechanism controlling gene expression during differentiation and development. We investigated the epigenetic regulation of nuclear-encoded, mitochondrial (mtDNA) polymerase γ catalytic subunit ( PolgA ) by examining status a CpG island within exon 2 PolgA. Bisulphite sequencing identified low levels (<10%) mouse oocytes, blastocysts embryonic stem cells (ESCs), while somatic tissues contained significantly higher (>40%). In contrast, induced pluripotent (iPS)...
As stem cells undergo differentiation, mitochondrial DNA (mtDNA) copy number is strictly regulated in order that specialized can generate appropriate levels of adenosine triphosphate (ATP) through oxidative phosphorylation (OXPHOS) to undertake their specific functions. It not understood whether tumor-initiating regulate mtDNA a similar manner or essential for tumorigenesis. We show human neural (hNSCs) increased content during differentiation process was mediated by synergistic relationship...
The TIM22 complex mediates the import of hydrophobic carrier proteins into mitochondrial inner membrane. While machinery has been well characterised in yeast, human remains poorly characterised. Here, we identify Tim29 (C19orf52) as a novel, metazoan-specific subunit complex. protein is integrated membrane with it's C-terminus exposed to intermembrane space. required for stability and functions assembly hTim22. Furthermore, contacts Translocase Outer Mitochondrial Membrane, TOM complex,...
Complex IV is the terminal enzyme of mitochondrial respiratory chain. In humans, biogenesis complex involves coordinated assembly 13 subunits encoded by both and nuclear genomes. The early stages involving DNA-encoded CO1 CO2 have been well studied. However, latter stages, during which many are incorporated, less understood. Using in vitro import assays, we found that Cox6a, Cox6b Cox7a assembled into pre-existing IV, while Cox4-1 Cox6c subcomplexes may represent rate-limiting intermediates....
Autosomal recessive ataxias are a clinically diverse group of syndromes that in some cases caused by mutations genes with roles the DNA damage response, transcriptional regulation or mitochondrial function. One these ataxias, known as Recessive Cerebellar Ataxia Type-2 (ARCA-2, also SCAR9/COQ10D4; OMIM: #612016), arises due to ADCK3 gene. The product this gene (ADCK3) is an atypical kinase thought play regulatory role coenzyme Q10 (CoQ10) biosynthesis. Although much work has been performed...
Human mitochondrial complex I is the largest enzyme of respiratory chain and composed 44 different subunits. Complex subunits are encoded by both nuclear (mt) DNA their assembly requires a number additional proteins. FAD-dependent oxidoreductase domain-containing protein 1 (FOXRED1) was recently identified as putative factor FOXRED1 mutations in patients cause deficiency; however, its role unknown. Here, we demonstrate that involved mid-late stages assembly. In patient with mutations, levels...
Abstract An increasing number of women fail to achieve pregnancy due either failed fertilization or embryo arrest during preimplantation development. This often results from decreased oocyte quality. Indeed, reduced mitochondrial DNA copy (mitochondrial deficiency) may disrupt quality in some women. To overcome deficiency, whilst maintaining genetic identity, we supplemented pig oocytes selected for cytoplasmic maturation and lower developmental competence, with autologous populations...
The large number of extant Muridae species provides the opportunity investigating functional limits nuclear/mitochondrial respiratory chain (RC) subunit interactions by introducing mitochondrial genomes from progressively more divergent into Mus musculus domesticus mtDNA-less (ρ0) cells. We created a panel such xenomitochondrial cybrids, using as donors cells six murid with divergence M. m. estimated at 2 to 12 Myr before present. Species used were spretus, caroli, dunni, pahari, Otomys...