{"database":"ENA","file_versions":[],"scores":null,"additional":{"omics_type":["Genomics"],"center_name":["01.423, Biochemistry II, Goettingen Universitatsmedizin"],"full_dataset_link":["https://www.ebi.ac.uk/ena/browser/view/PRJNA126769"],"scientific_name":["Mus musculus"],"long_description":["Progressive mitochondrial respiratory chain (RC) deficiency is associated with a wide spectrum of adult-onset degenerative diseases, as well as with normal aging. We have previously generated the Deletor mice to model late-onset progressive RC defects. Here we report novel tissue-specific pathways contributing to mitochondrial disease pathogenesis, identified by gene expression analysis. We found that RC-deficient muscle fibers secrete the fasting-induced hormone, fibroblast growth factor 21 (FGF21). This response leads to fatty acid recruitment from adipocytes and resistance to high-fat-diet induced obesity in mice, but does not affect glucose or insulin metabolism. FGF21 is also induced in the muscle of mitochondrial myopathy patients and in other RC-deficient mice. These data show that skeletal muscle is an endocrine organ, which signals its energy deficiency through FGF21. Furthermore, RC deficiency in single muscle fibers initiates a global starvation response. These data have important implications for conditions with primary or secondary RC deficiency. Overall design: Mice overexpressing mutant Twinkle (C10ORF2) protein are the first animal model for Progressive External Ophtalmoplegia (PEO). Using PEO-model and wt-mice, cerebellum and hippocampus were analyzed for gene expression profile."],"repository":["ENA"],"additional_accession":[]},"is_claimable":false,"name":"Mus musculus","description":"cerebellum and hippocampus - deletor x wt","dates":{"last_updated":"2025-09-24","first_public":"2024-04-04"},"accession":"PRJNA126769","cross_references":{"GEO":["GSE21961"],"taxon":["10090"]}}