A5042890482- Mitochondrial Function and Pathology
- RNA Research and Splicing
- Metabolism and Genetic Disorders
- Epigenetics and DNA Methylation
- Adipose Tissue and Metabolism
- Genetic and Kidney Cyst Diseases
- Birth, Development, and Health
- Immune Cell Function and Interaction
- Growth Hormone and Insulin-like Growth Factors
- Estrogen and related hormone effects
- RNA regulation and disease
- Coenzyme Q10 studies and effects
- interferon and immune responses
- Amyotrophic Lateral Sclerosis Research
- Bone health and osteoporosis research
- Connective Tissue Growth Factor Research
- Sexual Differentiation and Disorders
- ATP Synthase and ATPases Research
- Genetic Neurodegenerative Diseases
- Fibroblast Growth Factor Research
- Stress Responses and Cortisol
- Climate Change and Health Impacts
- RNA modifications and cancer
- Biomarkers in Disease Mechanisms
- Evolution and Genetic Dynamics
University of California, San Francisco
2019-2024
University of Helsinki
2016-2019
Astrocytes, often considered as secondary responders to neurodegeneration, are emerging primary drivers of brain disease. Here we show that mitochondrial DNA depletion in astrocytes affects their cilium, the signaling organelle a cell. The progressive oxidative phosphorylation deficiency induces FOXJ1 and RFX transcription factors, known master regulators motile ciliogenesis. Consequently, robust gene expression program involving cilia components multiciliated cell differentiation factors...
Abstract In lactating mothers, the high calcium (Ca 2+ ) demand for milk production triggers significant bone loss 1 . Although oestrogen normally counteracts excessive resorption by promoting formation, this sex steroid drops precipitously during postpartum period. Here we report that brain-derived cellular communication network factor 3 (CCN3) secreted from KISS1 neurons of arcuate nucleus (ARC fills void and functions as a potent osteoanabolic to build in females. We began showing our...
Research Article29 October 2018Open Access Transparent process Metabolomes of mitochondrial diseases and inclusion body myositis patients: treatment targets biomarkers Jana Buzkova Programs Unit, Molecular Neurology, Biomedicum-Helsinki, University Helsinki, Finland Search for more papers by this author Joni Nikkanen orcid.org/0000-0001-7099-4061 Sofia Ahola Anna H Hakonen Ksenia Sevastianova Department Medicine, Helsinki Hospital, Minerva Foundation Institute Medical Research, Topi Hovinen...
Adaptations to infectious and dietary pressures shape mammalian physiology disease risk. How such adaptations affect sex-biased diseases remains insufficiently studied. In this study, we show that sex-dependent hepatic gene programs confer a robust (~300%) survival advantage for male mice during lethal bacterial infection. The transcription factor B cell lymphoma 6 (BCL6), which masculinizes expression at puberty, is essential advantage. However, protection by BCL6 protein comes cost...
Mitochondrial DNA (mtDNA) depletion syndrome (MDS) is a group of severe, tissue-specific diseases childhood with unknown pathogenesis. Brain-specific MDS manifests as devastating spongiotic encephalopathy no curative therapy. Here, we report cell type–specific stress responses and effects rapamycin treatment ketogenic diet (KD) in mice mimicking human MDS, these interventions were reported to improve some mitochondrial disease signs or symptoms. These astrocyte-specific knockout Twnk gene...
Report6 November 2017Open Access Transparent process A complex genomic locus drives mtDNA replicase POLG expression to its disease-related nervous system regions Joni Nikkanen Research Programs Unit, Molecular Neurology, University of Helsinki, Finland Search for more papers by this author Juan Cruz Landoni Diego Balboa Biomedicum Stem Cell Center, Maarja Haugas Department Biosciences, Juha Partanen Anders Paetau HUSLAB and Pathology, Helsinki Hospital, Pirjo Isohanni Pediatric Children's...
Abstract Astrocytes, often considered as secondary responders to neurodegenerative processes, are emerging primary drivers of brain disease. The underlying pathogenic mechanisms are, however, insufficiently understood. Here we show that pathogenesis mitochondrial spongiotic encephalopathy, a severe manifestation diseases, involves abnormal maintenance the astrocytic cilium, major signaling organelle cell. We progressive respiratory chain deficiency in astrocytes activates FOXJ1 and RFX...
In lactating mothers, the high calcium (Ca
ABSTRACT Current concepts in evolutionary medicine propose that trade-offs and mismatches with a shifting environment increase disease risk. While biological sex also impacts prevalence, contributions of environmental pressures to sex-biased diseases remain unexplored. Here, we show sex-dependent hepatic programs confer robust (~300%) survival advantage for male mice during lethal bacterial infection. The transcription factor BCL6, which masculinizes gene expression at puberty, is essential...
Abstract Central estrogen signaling in the medial basal hypothalamus (MBH) coordinates energy expenditure, food intake, and reproduction, as well skeletal homeostasis. Prior work from our lab found that via Estrogen Receptor alpha (ERa) arcuate (ARC) nucleus normally restrains bone formation female mice only. We asked if high mass Esr1Nkx2-1Cre mouse model, which ablates ERa MBH, is maintained or deteriorates with chronic overnutrition, this known to increase frailty humans. To carry out...