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Proteomicsmir-1 coordinately regulates lysosomal v-ATPase and biogenesis to affect muscle contractility upon proteotoxic challenge during ageing
Muscle function relies on the precise architecture of dynamic contractile elements, which must be fine-tuned to maintain motility throughout life. Muscle is also highly plastic, and remodeled in response to stress, growth, neural and metabolic inputs. The evolutionarily conserved muscle microRNA, mi...
ORGANISM(S): Caenorhabditis elegans 
2021-07-14 | PXD023544 | Pride
Lfq Worm 
ProteomicsPreserved respiratory chain capacity and physiology in mice with profoundly reduced levels of mitochondrial respirasomes
The mammalian respiratory chain complexes I, III2 and IV (CI, CIII2 and CIV) are critical for cellular bioenergetics and form a stable assembly, the respirasome (CI-CIII2-CIV), that is biochemically and structurally well documented. The role of the respirasome in bioenergetics and regulation of meta...
ORGANISM(S): Mus musculus (Mouse) 
2024-03-19 | PXD023846 | Pride
Tmt Supercomplex Oxphos Mitochondria Complexome profiling 
ProteomicsTranscriptomic and proteomic landscape of mitochondrial dysfunction reveals secondary coenzyme Q deficiency in mammals
Abstract Dysfunction of the oxidative phosphorylation (OXPHOS) system is a major cause of human disease and the cellular consequences are highly complex. Here, we present comparative analyses of mitochondrial proteomes, cellular transcriptomes and targeted metabolomics of five knockout mouse strains...
ORGANISM(S): Mus musculus (Mouse) 
2022-08-09 | PXD035880 | Pride
Mitochondria Coenzyme q Lfq Mouse 
ProteomicsMechanism of mitoribosomal small subunit biogenesis and preinitiation
Mitoribosomes are essential for synthesis and maintenance of bioenergetic proteins. Here, we use cryoelectron microscopy (cryo-EM) to determine a series of the small mitoribosomal subunit (SSU) intermediates in complex with auxiliary factors, revealing a sequential assembly mechanism. The methyltran...
ORGANISM(S): Homo sapiens (Human) 
2022-07-11 | PXD031678 | Pride
Silac Sucrose gradient Translation initiation Mitoribosome 
ProteomicsNeuronal metabolic rewiring promotesresilience to neurodegeneration caused by mitochondrial dysfunction
Neurodegeneration in mitochondrial disorders is considered irreversible because of limited metabolic plasticity in neurons, yet the cell-autonomous implications of mitochondrial dysfunction for neuronal metabolism in vivo are poorly understood. Here, we profiled the cell-specific proteome of Purkinj...
ORGANISM(S): Mus musculus (Mouse) 
2020-08-03 | PXD019690 | Pride
Anaplerosis Labe-free Tmt Purkinje neurons Mitofusin 2 
ProteomicsNFYB-1 regulates mitochondrial function and longevity via lysosomal prosaposin
Mitochondrial activity is critical for cellular vitality and organismal longevity, yet underlying regulatory mechanisms spanning these different levels of organization remain elusive. From RNAi screens for mitochondrial biogenesis, we discovered NFYB-1, a subunit of the NF-Y transcriptional complex,...
ORGANISM(S): Caenorhabditis elegans 
2020-03-11 | PXD013233 | Pride
Nfyb-1 Mitochondria Proteomics Ceramide Metabolomics Cardiolipin Single shot Stress response Prosaposin Ageing Label-free Transcriptomics 
ProteomicsMitochondrial RNA polymerase overexpression enhances transcription initiation and exercise capacity without increasing mature mtRNA levels
POLRMT is the sole RNA polymerase in human mitochondria, where it generates primers for mitochondrial DNA (mtDNA) replication and transcribes the mtDNA to express genes encoding essential components of the oxidative phosphorylation (OXPHOS) system. Elevated POLRMT levels are found in several cancers...
ORGANISM(S): Mus musculus (Mouse) 
2025-10-10 | PXD068527 | Pride
Polrmt Mitochondria Label-free Dda 
ProteomicsProtein kinase A controls the hexosamine pathway by tuning feedback inhibition of GFAT-1
The hexosamine pathway (HP) is a key anabolic pathway whose product uridine 5’-diphospho-N-acetyl-D-glucosamine (UDP-GlcNAc) is an essential precursor for all glycosylation processes in mammals. It modulates the ER stress response, is implicated in cancer and diabetes, and HP activation extends life...
ORGANISM(S): Homo sapiens (Human) 
2020-07-23 | PXD020451 | Pride
Human Pka Phosphorylation 
ProteomicsProteolytic processing promotes the decatenation activity of human mitochondrial TOP3A; BioID
The TOP3A gene encodes protein products that are targeted to both the nucleus and to mitochondria. The nuclear isoform of TOP3A is required for the resolution of double Holliday junctions during homologous recombination, while the mitochondrial isoform of TOP3A separates hemicatenated daughter mitoc...
ORGANISM(S): Homo sapiens (Human) 
2025-09-30 | PXD063285 | Pride
Top3a Label-free Bioid 
ProteomicsHigh levels of TFAM repress in vivo transcription of mitochondrial DNA
Mammalian mitochondrial DNA (mtDNA) is coated with mitochondrial transcription factor A (TFAM) and compacted into nucleoids. TFAM is not only the main component of mitochondrial nucleoids but its levels can also control mtDNA copy number. Here we show that the TFAM-to-mtDNA ratio is critical for mai...
ORGANISM(S): Mus musculus (Mouse) 
2021-08-18 | PXD023050 | Pride
Transcription Tmt Dna Tfam Mitochondria 
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