Reciprocal regulation of mitochondrial fission and fusion
R Sabouny, TE Shutt - Trends in biochemical sciences, 2020 - cell.com
The dynamic processes of mitochondrial fission and fusion are tightly regulated, determine
mitochondrial shape, and influence mitochondrial functions. For example, fission and fusion …
mitochondrial shape, and influence mitochondrial functions. For example, fission and fusion …
[HTML][HTML] Characterization of the C584R variant in the mtDNA depletion syndrome gene FBXL4, reveals a novel role for FBXL4 as a regulator of mitochondrial fusion
R Sabouny, R Wong, L Lee-Glover… - … et Biophysica Acta (BBA …, 2019 - Elsevier
Mutations in FBXL4 (F-Box and Leucine rich repeat protein 4), a nuclear-encoded mitochondrial
protein with an unknown function, cause mitochondrial DNA depletion syndrome. We …
protein with an unknown function, cause mitochondrial DNA depletion syndrome. We …
PISD is a mitochondrial disease gene causing skeletal dysplasia, cataracts, and white matter changes
…, CM Goedhart, PN Sam, R Sabouny… - Life science …, 2019 - life-science-alliance.org
Exome sequencing of two sisters with congenital cataracts, short stature, and white matter
changes identified compound heterozygous variants in the PISD gene, encoding the …
changes identified compound heterozygous variants in the PISD gene, encoding the …
The role of mitochondrial dynamics in mtDNA maintenance
R Sabouny, TE Shutt - Journal of cell science, 2021 - journals.biologists.com
The dynamic nature of mitochondria, which can fuse, divide and move throughout the cell,
allows these critical organelles to adapt their function in response to cellular demands, and is …
allows these critical organelles to adapt their function in response to cellular demands, and is …
[HTML][HTML] A new automated tool to quantify nucleoid distribution within mitochondrial networks
HS Ilamathi, M Ouellet, R Sabouny… - Scientific reports, 2021 - nature.com
Mitochondrial DNA (mtDNA) maintenance is essential to sustain a functionally healthy
population of mitochondria within cells. Proper mtDNA replication and distribution within …
population of mitochondria within cells. Proper mtDNA replication and distribution within …
The Keap1–Nrf2 stress response pathway promotes mitochondrial hyperfusion through degradation of the mitochondrial fission protein Drp1
R Sabouny, E Fraunberger, M Geoffrion… - Antioxidants & redox …, 2017 - liebertpub.com
Aims: Mitochondrial function is coupled to metabolic and survival pathways through both
direct signaling cascades and dynamic changes in mitochondrial morphology. For example, a …
direct signaling cascades and dynamic changes in mitochondrial morphology. For example, a …
[HTML][HTML] Aberrant mitochondrial morphology and function in the BTBR mouse model of autism is improved by two weeks of ketogenic diet
Y Ahn, R Sabouny, BR Villa, NC Yee… - International journal of …, 2020 - mdpi.com
Autism spectrum disorder (ASD) is a highly prevalent neurodevelopmental disorder that
exhibits a common set of behavioral and cognitive impairments. Although the etiology of ASD …
exhibits a common set of behavioral and cognitive impairments. Although the etiology of ASD …
[HTML][HTML] Perturbed mitochondrial dynamics is a novel feature of colitis that can be targeted to lessen disease
NL Mancini, L Goudie, W Xu, R Sabouny… - Cellular and molecular …, 2020 - Elsevier
Background & Aims Mitochondria exist in a constantly remodelling network, and excessive
fragmentation can be pathophysiological. Mitochondrial dysfunction can accompany enteric …
fragmentation can be pathophysiological. Mitochondrial dysfunction can accompany enteric …
[HTML][HTML] Mesenchymal stem cells shift mitochondrial dynamics and enhance oxidative phosphorylation in recipient cells
Mesenchymal stem cells (MSCs) are the most commonly used cells in tissue engineering
and regenerative medicine. MSCs can promote host tissue repair through several different …
and regenerative medicine. MSCs can promote host tissue repair through several different …
Reversible mitochondrial fragmentation in iPSC-derived cardiomyocytes from children with DCMA, a mitochondrial cardiomyopathy
L Rohani, P Machiraju, R Sabouny, G Meng… - Canadian Journal of …, 2020 - Elsevier
Background Dilated cardiomyopathy with ataxia syndrome (DCMA) is an understudied
autosomal recessive disease caused by loss-of-function mutations in the poorly characterized …
autosomal recessive disease caused by loss-of-function mutations in the poorly characterized …