A5048859295- Mitochondrial Function and Pathology
- Fibroblast Growth Factor Research
- Metabolism and Genetic Disorders
- Genetics and Neurodevelopmental Disorders
- Metabolism, Diabetes, and Cancer
- Sirtuins and Resveratrol in Medicine
- Epigenetics and DNA Methylation
- Erythrocyte Function and Pathophysiology
- PI3K/AKT/mTOR signaling in cancer
- Research in Social Sciences
- Autophagy in Disease and Therapy
- GDF15 and Related Biomarkers
- Adipose Tissue and Metabolism
- Adenosine and Purinergic Signaling
- DNA Repair Mechanisms
- Cardiovascular Disease and Adiposity
- ATP Synthase and ATPases Research
- Lipid metabolism and biosynthesis
- RNA modifications and cancer
- Inflammatory Myopathies and Dermatomyositis
- Connective tissue disorders research
- Tryptophan and brain disorders
University of Helsinki
2012-2019
MRC Mitochondrial Biology Unit
2016
Medical Research Council
2016
Abstract Nutrient availability is the major regulator of life and reproduction, a complex cellular signaling network has evolved to adapt organisms fasting. These sensor pathways monitor energy metabolism, especially mitochondrial ATP production NAD + / NADH ratio, as signals for nutritional state. We hypothesized that these would be modified by respiratory chain disease, because inefficient utilization production. Oral administration nicotinamide riboside ( NR ), vitamin B3 precursor, was...
To validate new mitochondrial myopathy serum biomarkers for diagnostic use.We analyzed FGF21 (S-FGF21) and GDF15 from patients with (1) diseases (2) nonmitochondrial disorders partially overlapping disorder phenotypes. We (3) did a meta-analysis of S-FGF21 in disease (4) S-Fgf21 skeletal muscle Fgf21 expression 6 mouse models different muscle-manifesting dysfunctions.We report that consistently increases primary myopathy, especially translation defects or DNA (mtDNA) deletions (675 347...
Deficiency of USF1 protects against obesity, insulin resistance, and cardiovascular disease in mice humans, induces brown adipose tissue to burn triglycerides glucose.
Haematopoietic progenitor cells show special sensitivity to mitochondrial DNA (mtDNA) mutagenesis, which suggests that increased mtDNA mutagenesis could underlie anemias. Here we elevated in mice with a proof-reading deficient polymerase (PolG) leads incomplete clearance, asynchronized iron loading erythroid precursors, and total free cellular content. The resulting Fenton chemistry oxidative damage premature destruction of erythrocytes by splenic macrophages. Our data indicate mitochondria...
Nicotinamide adenine dinucleotide (NAD) and glutathione are vital molecules that control redox-state, enzyme functions metabolic flux in hundreds of cellular reactions. High NAD+ level occurs fasting has been associated with health outcomes model systems, while low NAD+/NADH ratio specific diseases. Still, the normal, booster-treated or disease-related levels NADs glutathiones not well known humans. Here, we present a standardized technology for high-throughput quantitative measurement NAD+,...