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Human RCC1L is involved in the maintenance of mitochondrial nucleoids and mtDNA
by
Matsumoto, Emi
, Higashiyama, Tetsuya
, Sasaki, Narie
, Sasaki, Taeko
in
631/337
/ 631/80
/ Alternative splicing
/ Cell Cycle Proteins - genetics
/ Cell Cycle Proteins - metabolism
/ Chloramphenicol
/ DNA, Mitochondrial - genetics
/ DNA, Mitochondrial - metabolism
/ Humanities and Social Sciences
/ Humans
/ Isoforms
/ Mitochondria - genetics
/ Mitochondria - metabolism
/ Mitochondrial DNA
/ Mitochondrial nucleoid
/ Mitochondrial Proteins - genetics
/ Mitochondrial Proteins - metabolism
/ multidisciplinary
/ Nucleoids
/ Phenotypes
/ Protein Isoforms - genetics
/ Protein Isoforms - metabolism
/ Pyruvic acid
/ RCC1L
/ Science
/ Science (multidisciplinary)
/ Translation
/ Uridine
2025
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Human RCC1L is involved in the maintenance of mitochondrial nucleoids and mtDNA
by
Matsumoto, Emi
, Higashiyama, Tetsuya
, Sasaki, Narie
, Sasaki, Taeko
in
631/337
/ 631/80
/ Alternative splicing
/ Cell Cycle Proteins - genetics
/ Cell Cycle Proteins - metabolism
/ Chloramphenicol
/ DNA, Mitochondrial - genetics
/ DNA, Mitochondrial - metabolism
/ Humanities and Social Sciences
/ Humans
/ Isoforms
/ Mitochondria - genetics
/ Mitochondria - metabolism
/ Mitochondrial DNA
/ Mitochondrial nucleoid
/ Mitochondrial Proteins - genetics
/ Mitochondrial Proteins - metabolism
/ multidisciplinary
/ Nucleoids
/ Phenotypes
/ Protein Isoforms - genetics
/ Protein Isoforms - metabolism
/ Pyruvic acid
/ RCC1L
/ Science
/ Science (multidisciplinary)
/ Translation
/ Uridine
2025
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Human RCC1L is involved in the maintenance of mitochondrial nucleoids and mtDNA
by
Matsumoto, Emi
, Higashiyama, Tetsuya
, Sasaki, Narie
, Sasaki, Taeko
in
631/337
/ 631/80
/ Alternative splicing
/ Cell Cycle Proteins - genetics
/ Cell Cycle Proteins - metabolism
/ Chloramphenicol
/ DNA, Mitochondrial - genetics
/ DNA, Mitochondrial - metabolism
/ Humanities and Social Sciences
/ Humans
/ Isoforms
/ Mitochondria - genetics
/ Mitochondria - metabolism
/ Mitochondrial DNA
/ Mitochondrial nucleoid
/ Mitochondrial Proteins - genetics
/ Mitochondrial Proteins - metabolism
/ multidisciplinary
/ Nucleoids
/ Phenotypes
/ Protein Isoforms - genetics
/ Protein Isoforms - metabolism
/ Pyruvic acid
/ RCC1L
/ Science
/ Science (multidisciplinary)
/ Translation
/ Uridine
2025
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Human RCC1L is involved in the maintenance of mitochondrial nucleoids and mtDNA
Journal Article
Human RCC1L is involved in the maintenance of mitochondrial nucleoids and mtDNA
2025
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Overview
Mitochondrial DNA (mtDNA) is organized with proteins into mitochondrial nucleoid (mt-nucleoid). The mt-nucleoid is a unit for the maintenance and function of mtDNA. The regulator of chromosome condensation 1-like protein (RCC1L) performs various functions in mitochondria, including translation, but its involvement in regulating mt-nucleoid maintenance is unknown. Herein, we found that human RCC1L was required to maintain mt-nucleoids and mtDNA. Human RCC1L has three splicing isoforms: RCC1L
V1
, RCC1L
V2
, and RCC1L
V3
. Knockout (KO) cells lacking all RCC1L isoforms, which were lethal without pyruvate and uridine, exhibited a decrease in mt-nucleoids and mtDNA, along with swollen and fragmented mitochondria. Among the three RCC1L isoforms, only RCC1L
V1
recovered all phenotypes observed in RCC1L KO cells. As the treatment of wild-type cells with chloramphenicol, a mitochondrial translation inhibitor, did not lead to the decrease in mt-nucleoids accompanied by mtDNA depletion, the decrease in mt-nucleoids and mtDNA in RCC1L KO cells was not solely attributed to impaired mitochondrial translation. Using conditional RCC1L KO cells, we observed a rapid decrease in mt-nucleoids and mtDNA during a specific period following RCC1L loss. Our findings indicate that RCC1L regulates the maintenance of mt-nucleoids and mtDNA besides its role in mitochondrial translational regulation.
Publisher
Nature Publishing Group UK,Nature Publishing Group,Nature Portfolio
Subject
/ 631/80
/ Cell Cycle Proteins - genetics
/ Cell Cycle Proteins - metabolism
/ DNA, Mitochondrial - genetics
/ DNA, Mitochondrial - metabolism
/ Humanities and Social Sciences
/ Humans
/ Isoforms
/ Mitochondrial Proteins - genetics
/ Mitochondrial Proteins - metabolism
/ Protein Isoforms - metabolism
/ RCC1L
/ Science
/ Uridine
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