Cargando…

FBXL4 deficiency increases mitochondrial removal by autophagy

Pathogenic variants in FBXL4 cause a severe encephalopathic syndrome associated with mtDNA depletion and deficient oxidative phosphorylation. To gain further insight into the enigmatic pathophysiology caused by FBXL4 deficiency, we generated homozygous Fbxl4 knockout mice and found that they display...

Descripción completa

Detalles Bibliográficos
Autores principales:	Alsina, David, Lytovchenko, Oleksandr, Schab, Aleksandra, Atanassov, Ilian, Schober, Florian A, Jiang, Min, Koolmeister, Camilla, Wedell, Anna, Taylor, Robert W, Wredenberg, Anna, Larsson, Nils‐Göran
Formato:	Online Artículo Texto
Lenguaje:	English
Publicado:	John Wiley and Sons Inc. 2020
Materias:	Articles
Acceso en línea:	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7338799/ https://www.ncbi.nlm.nih.gov/pubmed/32525278 http://dx.doi.org/10.15252/emmm.201911659

Ejemplares similares

Stable Isotope Labeling of Amino Acids in Flies (SILAF) Reveals Differential Phosphorylation of Mitochondrial Proteins Upon Loss of OXPHOS Subunits
por: Schober, Florian A., et al.
Publicado: (2021)

TEFM regulates both transcription elongation and RNA processing in mitochondria
por: Jiang, Shan, et al.
Publicado: (2019)

High levels of TFAM repress mammalian mitochondrial DNA transcription in vivo
por: Bonekamp, Nina A, et al.
Publicado: (2021)

Transcriptomic and proteomic landscape of mitochondrial dysfunction reveals secondary coenzyme Q deficiency in mammals
por: Kühl, Inge, et al.
Publicado: (2017)

The one-carbon pool controls mitochondrial energy metabolism via complex I and iron-sulfur clusters
por: Schober, Florian A., et al.
Publicado: (2021)

A role for BCL2L13 and autophagy in germline purifying selection of mtDNA
por: Kremer, Laura S., et al.
Publicado: (2023)

Defects of mitochondrial RNA turnover lead to the accumulation of double-stranded RNA in vivo
por: Pajak, Aleksandra, et al.
Publicado: (2019)

Case Report: A Novel Mutation in the Mitochondrial MT-ND5 Gene Is Associated With Leber Hereditary Optic Neuropathy (LHON)
por: Engvall, Martin, et al.
Publicado: (2021)

SLC12A2 mutations cause NKCC1 deficiency with encephalopathy and impaired secretory epithelia
por: Stödberg, Tommy, et al.
Publicado: (2020)

Protocol for the derivation, culturing, and differentiation of human iPS-cell-derived neuroepithelial stem cells to study neural differentiation in vitro
por: Calvo-Garrido, Javier, et al.
Publicado: (2021)

SQSTM1/p62-Directed Metabolic Reprogramming Is Essential for Normal Neurodifferentiation
por: Calvo-Garrido, Javier, et al.
Publicado: (2019)

SUV3 helicase is required for correct processing of mitochondrial transcripts
por: Clemente, Paula, et al.
Publicado: (2015)

C6orf203 is an RNA-binding protein involved in mitochondrial protein synthesis
por: Gopalakrishna, Shreekara, et al.
Publicado: (2019)

Metabolic resistance to the inhibition of mitochondrial transcription revealed by CRISPR‐Cas9 screen
por: Mennuni, Mara, et al.
Publicado: (2021)

Strong Purifying Selection in Transmission of Mammalian Mitochondrial DNA
por: Stewart, James Bruce, et al.
Publicado: (2008)

FBXL4 ubiquitin ligase deficiency promotes mitophagy by elevating NIX levels
por: Elcocks, Hannah, et al.
Publicado: (2023)

Human GTPBP5 is involved in the late stage of mitoribosome large subunit assembly
por: Cipullo, Miriam, et al.
Publicado: (2020)

Notch and the pre-TCR coordinate thymocyte proliferation by induction of the SCF subunits Fbxl1 and Fbxl12
por: Zhao, Bin, et al.
Publicado: (2019)

SLIRP stabilizes LRPPRC via an RRM–PPR protein interface
por: Spåhr, Henrik, et al.
Publicado: (2016)

Novel Mutation m.10372A>G in MT-ND3 Causing Sensorimotor Axonal Polyneuropathy
por: Bruhn, Helene, et al.
Publicado: (2021)

Mitochondrial Polyadenylation Is a One-Step Process Required for mRNA Integrity and tRNA Maturation
por: Bratic, Ana, et al.
Publicado: (2016)

F-box protein Fbxl18 mediates polyubiquitylation and proteasomal degradation of the pro-apoptotic SCF subunit Fbxl7
por: Liu, Y, et al.
Publicado: (2015)

Increased proteome coverage by combining PAGE and peptide isoelectric focusing: Comparative study of gel-based separation approaches
por: Atanassov, Ilian, et al.
Publicado: (2013)

Mitofusin 2 is required to maintain mitochondrial coenzyme Q levels
por: Mourier, Arnaud, et al.
Publicado: (2015)

Complementation between polymerase- and exonuclease-deficient mitochondrial DNA polymerase mutants in genomically engineered flies
por: Bratic, Ana, et al.
Publicado: (2015)

Epigenetic suppression of FBXL7 promotes metastasis
por: Moro, Loredana, et al.
Publicado: (2020)

Mammalian RNase H1 directs RNA primer formation for mtDNA replication initiation and is also necessary for mtDNA replication completion
por: Misic, Jelena, et al.
Publicado: (2022)

Identification, Classification and Characterization Analysis of FBXL Gene in Cotton
por: Pan, Jingwen, et al.
Publicado: (2022)

MitoRibo-Tag Mice Provide a Tool for In Vivo Studies of Mitoribosome Composition
por: Busch, Jakob D., et al.
Publicado: (2019)

FBXL20-mediated Vps34 ubiquitination as a p53 controlled checkpoint in regulating autophagy and receptor degradation
por: Xiao, Juan, et al.
Publicado: (2015)

The amino terminal extension of mammalian mitochondrial RNA polymerase ensures promoter specific transcription initiation
por: Posse, Viktor, et al.
Publicado: (2014)

Rescue of primary ubiquinone deficiency due to a novel COQ7 defect using 2,4–dihydroxybensoic acid
por: Freyer, Christoph, et al.
Publicado: (2015)

The FBXL family of F-box proteins: variations on a theme
por: Mason, Bethany, et al.
Publicado: (2020)

A simple, flexible and efficient PCR-fusion/Gateway cloning procedure for gene fusion, site-directed mutagenesis, short sequence insertion and domain deletions and swaps
por: Atanassov, Ivan I, et al.
Publicado: (2009)

CDK-Mediator and FBXL19 prime developmental genes for activation by promoting atypical regulatory interactions
por: Feldmann, Angelika, et al.
Publicado: (2020)

Bayesian prediction of RNA translation from ribosome profiling
por: Malone, Brandon, et al.
Publicado: (2017)

Mitochondrial DNA copy number in human disease: the more the better?
por: Filograna, Roberta, et al.
Publicado: (2020)

Mutations in the mitochondrial tryptophanyl‐tRNA synthetase cause growth retardation and progressive leukoencephalopathy
por: Maffezzini, Camilla, et al.
Publicado: (2019)

SCF(Fbxl3) Ubiquitin Ligase Targets Cryptochromes at Their Cofactor Pocket
por: Xing, Weiman, et al.
Publicado: (2013)

Identification of FBXL4 as a Metastasis Associated Gene in Prostate Cancer
por: Stankiewicz, Elzbieta, et al.
Publicado: (2017)

Cannot write session to /tmp/vufind_sessions/sess_7s257d1beem28kvpl50b8o4d8v