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The Bacterial ClpXP-ClpB Family Is Enriched with RNA-Binding Protein Complexes.
Auburger G, Key J, Gispert S. Auburger G, et al. Cells. 2022 Aug 2;11(15):2370. doi: 10.3390/cells11152370. Cells. 2022. PMID: 35954215 Free PMC article. Review.
Trapping, proteome, and complexome data retrieved consistent coaccumulation of CLPXP with GFM1 and TUFM orthologs. CLPX shows broad interaction selectivity encompassing mitochondrial translation elongation, RNA granules, and nucleoids. CLPB preferentially attaches t …
Trapping, proteome, and complexome data retrieved consistent coaccumulation of CLPXP with GFM1 and TUFM orthologs. CLPX shows broad i …
UPR(mt) activation improves pathological alterations in cellular models of mitochondrial diseases.
Suárez-Rivero JM, Pastor-Maldonado CJ, Povea-Cabello S, Álvarez-Córdoba M, Villalón-García I, Talaverón-Rey M, Suárez-Carrillo A, Munuera-Cabeza M, Reche-López D, Cilleros-Holgado P, Piñero-Perez R, Sánchez-Alcázar JA. Suárez-Rivero JM, et al. Orphanet J Rare Dis. 2022 May 17;17(1):204. doi: 10.1186/s13023-022-02331-8. Orphanet J Rare Dis. 2022. PMID: 35581596 Free PMC article.
BACKGROUND: Mitochondrial diseases represent one of the most common groups of genetic diseases. ...CONCLUSIONS: Overall, we provide compelling evidence to propose the activation of intrinsic cellular compensatory mechanisms as promising therapeutic strategy f …
BACKGROUND: Mitochondrial diseases represent one of the most common groups of genetic diseases. ...CONCLUSIONS: Overall …
The first case of combined oxidative phosphorylation deficiency-1 due to a GFM1 mutation in the Serbian population: a case report and literature review.
Aleksic D, Jankovic MG, Todorovic S, Kovacevic M, Borkovic M. Aleksic D, et al. Turk J Pediatr. 2023;65(6):1018-1024. doi: 10.24953/turkjped.2022.1082. Turk J Pediatr. 2023. PMID: 38204316 Free article. Review.
BACKGROUND: Combined oxidative phosphorylation deficiency-1 (COXPD1) resulting from a mutation in the G elongation factor mitochondrial 1 (GFM1) gene is an autosomal recessive multisystem disorder arising from a defect in the mitochond …
BACKGROUND: Combined oxidative phosphorylation deficiency-1 (COXPD1) resulting from a mutation in the G elongati …
Clinical, neuroimaging and biochemical findings in patients and patient fibroblasts expressing ten novel GFM1 mutations.
Barcia G, Rio M, Assouline Z, Zangarelli C, Gueguen N, Dumas VD, Marcorelles P, Schiff M, Slama A, Barth M, Hully M, de Lonlay P, Munnich A, Desguerre I, Bonnefont JP, Steffann J, Procaccio V, Boddaert N, Rötig A, Metodiev MD, Ruzzenente B. Barcia G, et al. Hum Mutat. 2020 Feb;41(2):397-402. doi: 10.1002/humu.23937. Epub 2019 Nov 11. Hum Mutat. 2020. PMID: 31680380
Brain magnetic resonance imaging showed the involvement of basal ganglia, brainstem, and periventricular white matter. Mutant EFG1 and OXPHOS proteins were decreased in patient's fibroblasts consistent with impaired mitochondrial translation. Thus, we expand the gen …
Brain magnetic resonance imaging showed the involvement of basal ganglia, brainstem, and periventricular white matter. Mutant EFG1 an …
Inactivity of Peptidase ClpP Causes Primary Accumulation of Mitochondrial Disaggregase ClpX with Its Interacting Nucleoid Proteins, and of mtDNA.
Key J, Torres-Odio S, Bach NC, Gispert S, Koepf G, Reichlmeir M, West AP, Prokisch H, Freisinger P, Newman WG, Shalev S, Sieber SA, Wittig I, Auburger G. Key J, et al. Cells. 2021 Nov 29;10(12):3354. doi: 10.3390/cells10123354. Cells. 2021. PMID: 34943861 Free PMC article.
ClpX co-accumulated in mitochondria with the nucleoid component POLDIP2, the mitochondrial poly(A) mRNA granule element LRPPRC, and tRNA processing factor GFM1 (in mouse, also GRSF1). Only in mouse did accumulated ClpX, GFM1, and GRSF1 appear in nuclear fract …
ClpX co-accumulated in mitochondria with the nucleoid component POLDIP2, the mitochondrial poly(A) mRNA granule element LRPPRC, and t …
Polyamide Microsized Particulate Polyplex Carriers for the 2'-OMethylRNA EFG1 Antisense Oligonucleotide.
Araújo D, Braz J, Dencheva NV, Carvalho I, Henriques M, Denchev ZZ, Malfois M, Silva S. Araújo D, et al. ACS Appl Bio Mater. 2021 May 17;4(5):4607-4617. doi: 10.1021/acsabm.1c00334. Epub 2021 Apr 30. ACS Appl Bio Mater. 2021. PMID: 35006798
Anti-EFG1 2'-OMethylRNA is an antisense oligonucleotide (ASO) that has the ability to recognize and block the EFG1 gene and to control Candida albicans filamentation. ...Thus, the goal of this work was to develop polyplexes based on porous poly(gamma-butyrolactam) ( …
Anti-EFG1 2'-OMethylRNA is an antisense oligonucleotide (ASO) that has the ability to recognize and block the EFG1 gene and to …
Activation of a cryptic splice site in the mitochondrial elongation factor GFM1 causes combined OXPHOS deficiency.
Simon MT, Ng BG, Friederich MW, Wang RY, Boyer M, Kircher M, Collard R, Buckingham KJ, Chang R, Shendure J, Nickerson DA, Bamshad MJ; University of Washington Center for Mendelian Genomics; Van Hove JLK, Freeze HH, Abdenur JE. Simon MT, et al. Mitochondrion. 2017 May;34:84-90. doi: 10.1016/j.mito.2017.02.004. Epub 2017 Feb 12. Mitochondrion. 2017. PMID: 28216230 Free PMC article.
While exome sequencing was negative for CDG related candidate genes, the testing revealed compound heterozygous mutations in the mitochondrial elongation factor G gene (GFM1). One of the mutations had been reported previously while the second, novel variant was foun …
While exome sequencing was negative for CDG related candidate genes, the testing revealed compound heterozygous mutations in the mitochon …
Whole exome sequencing identifies a novel compound heterozygous GFM1 variant underlying developmental delay, dystonia, polymicrogyria, and severe intellectual disability in a Pakhtun family.
Khan AU, Khan I, Khan MI, Latif M, Siddiqui MI, Khan SU, Htar TT, Wahid G, Ullah I, Bibi F, Khan A, Naseer MI, Seo GH, Jelani M. Khan AU, et al. Am J Med Genet A. 2022 Sep;188(9):2693-2700. doi: 10.1002/ajmg.a.62856. Epub 2022 Jun 15. Am J Med Genet A. 2022. PMID: 35703069
Mitochondrial protein synthesis requires three elongation factors including EF-Tu (TUFM; OMIM 602389), EF-Ts (TSFM; OMIM 604723), and EF-G1 (GFM1; OMIM 606639). Pathogenic variants in any of these three members result in defective mitochondrial translation wh …
Mitochondrial protein synthesis requires three elongation factors including EF-Tu (TUFM; OMIM 602389), EF-Ts (TSFM; OMIM 604723), and …
Carbon ion combined with tigecycline inhibits lung cancer cell proliferation by inducing mitochondrial dysfunction.
Yan J, Xie Y, Wang F, Chen Y, Zhang J, Dou Z, Gan L, Li H, Si J, Sun C, Di C, Zhang H. Yan J, et al. Life Sci. 2020 Dec 15;263:118586. doi: 10.1016/j.lfs.2020.118586. Epub 2020 Oct 13. Life Sci. 2020. PMID: 33065148
Additionally, combination treatment induced the most severe mitochondrial dysfunction by sharply reducing ATP, MMP and increasing Ca(2+) level of mitochondria. Up-regulation and down-regulation of mitochondrial translation proteins (EF-Tu, GFM1 and MRP …
Additionally, combination treatment induced the most severe mitochondrial dysfunction by sharply reducing ATP, MMP and increas …
A novel composition of two heterozygous GFM1 mutations in a Chinese child with epilepsy and mental retardation.
You C, Xu N, Qiu S, Li Y, Xu L, Li X, Yang L. You C, et al. Brain Behav. 2020 Oct;10(10):e01791. doi: 10.1002/brb3.1791. Epub 2020 Aug 9. Brain Behav. 2020. PMID: 32776492 Free PMC article.
INTRODUCTION: G elongation factor mitochondrial 1 (GFM1) encodes one of the mitochondrial translation elongation factors. GFM1 variants were reported to be associated with neurological diseases and liver diseases in a few cases. ...
INTRODUCTION: G elongation factor mitochondrial 1 (GFM1) encodes one of the mitochondrial translation elongation factor …
21 results