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The ubiquitin-conjugating enzyme UBE2D maintains a youthful proteome and ensures protein quality control during aging by sustaining proteasome activity.
PLoS Biol
January 2025
Department of Developmental Neurobiology, St. Jude Children's Research Hospital, Memphis, Tennessee, United States of America.
Ubiquitin-conjugating enzymes (E2s) are key for protein turnover and quality control via ubiquitination. Some E2s also physically interact with the proteasome, but it remains undetermined which E2s maintain proteostasis during aging. Here, we find that E2s have diverse roles in handling a model aggregation-prone protein (huntingtin-polyQ) in the Drosophila retina: while some E2s mediate aggregate assembly, UBE2D/effete (eff) and other E2s are required for huntingtin-polyQ degradation.
View Article and Find Full Text PDFLong-chain acyl-CoA synthetase regulates systemic lipid homeostasis via glycosylation-dependent lipoprotein production.
Life Metab
April 2024
State Key Laboratory of Molecular Developmental Biology, Institute of Genetics and Developmental Biology, Chinese Academy of Sciences, Beijing 100101, China.
Interorgan lipid transport is crucial for organism development and the maintenance of physiological function. Here, we demonstrate that long-chain acyl-CoA synthetase (dAcsl), which catalyzes the conversion of fatty acids into acyl-coenzyme As (acyl-CoAs), plays a critical role in regulating systemic lipid homeostasis. dAcsl deficiency in the fat body led to the ectopic accumulation of neutral lipids in the gut, along with significantly reduced lipoprotein contents in both the fat body and hemolymph.
View Article and Find Full Text PDFPreventing excessive autophagy protects from the pathology of mtDNA mutations in Drosophila melanogaster.
Nat Commun
December 2024
Department of Medical Biochemistry and Biophysics, Karolinska Institutet, 171 65, Stockholm, Sweden.
Aberration of mitochondrial function is a shared feature of many human pathologies, characterised by changes in metabolic flux, cellular energetics, morphology, composition, and dynamics of the mitochondrial network. While some of these changes serve as compensatory mechanisms to maintain cellular homeostasis, their chronic activation can permanently affect cellular metabolism and signalling, ultimately impairing cell function. Here, we use a Drosophila melanogaster model expressing a proofreading-deficient mtDNA polymerase (POLγ) in a genetic screen to find genes that mitigate the harmful accumulation of mtDNA mutations.
View Article and Find Full Text PDFArticle Synopsis
- Dentatorubral-pallidoluysian atrophy (DRPLA) is a neurodegenerative disease characterized by symptoms like ataxia, dementia, and epilepsy, caused by an expansion of CAG repeats in the ATROPHIN 1 (ATN1) gene.
- Researchers developed Drosophila (fruit fly) models that express either normal ATN1 (Q7) or a pathogenic version with expanded repeats (Q88), revealing that the pathogenic variant significantly reduces fly motility, lifespan, and affects internal structures more severely than the normal version.
- RNA sequencing identified pathways related to protein quality control that are altered by pathogenic ATN1, and subsequent genetic experiments highlighted the
Heterozygous pathogenic variants in are associated with oligodontia-colorectal cancer syndrome (ODCRCS), a disorder characterized by oligodontia, colorectal cancer, and in some cases, sparse hair and eyebrows. We have identified four individuals with one of two , heterozygous variants (NM_004655.4:c.
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