Abstract：
:Members of the pentatricopeptide repeat domain (PPR) protein family bind RNA and are important for post-transcriptional control of organelle gene expression in unicellular eukaryotes, metazoans and plants. They also have a role in human pathology, as mutations in the leucine-rich PPR-containing (LRPPRC) gene cause severe neurodegeneration. We have previously shown that the mammalian LRPPRC protein and its Drosophila melanogaster homolog DmLRPPRC1 (also known as bicoid stability factor) are necessary for mitochondrial translation by controlling stability and polyadenylation of mRNAs. We here report characterization of DmLRPPRC2, a second fruit fly homolog of LRPPRC, and show that it has a predominant mitochondrial localization and interacts with a stem-loop interacting RNA binding protein (DmSLIRP2). Ubiquitous downregulation of DmLrpprc2 expression causes respiratory chain dysfunction, developmental delay and shortened lifespan. Unexpectedly, decreased DmLRPPRC2 expression does not globally affect steady-state levels or polyadenylation of mitochondrial transcripts. However, some mitochondrial transcripts abnormally associate with the mitochondrial ribosomes and some products are dramatically overproduced and other ones decreased, which, in turn, results in severe deficiency of respiratory chain complexes. The function of DmLRPPRC2 thus seems to be to ensure that mitochondrial transcripts are presented to the mitochondrial ribosomes in an orderly fashion to avoid poorly coordinated translation.
journal_name
Nucleic Acids Resjournal_title
Nucleic acids researchauthors
Baggio F,Bratic A,Mourier A,Kauppila TE,Tain LS,Kukat C,Habermann B,Partridge L,Larsson NGdoi
10.1093/nar/gku1132subject
Has Abstractpub_date
2014-12-16 00:00:00pages
13920-38issue
22eissn
0305-1048issn
1362-4962pii
gku1132journal_volume
42pub_type
杂志文章abstract：:The current methods for quantifying genome-wide 5-methylcytosine (5mC) oxides are still scarce, mostly restricted with two limitations: assay sensitivity is seriously compromised with cost, assay time and sample input; epigenetic information is irreproducible during polymerase chain reaction (PCR) amplification withou...
journal_title：Nucleic acids research
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abstract：:Optical DNA mapping (ODM) allows visualization of long-range sequence information along single DNA molecules. The data can for example be used for detecting long range structural variations, for aiding DNA sequence assembly of complex genomes and for mapping epigenetic marks and DNA damage across the genome. ODM tradi...
journal_title：Nucleic acids research
pub_type： 杂志文章
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journal_title：Nucleic acids research
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journal_title：Nucleic acids research
pub_type： 杂志文章
doi：10.1093/nar/20.20.5311
更新日期：1992-10-25 00:00:00
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journal_title：Nucleic acids research
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journal_title：Nucleic acids research
pub_type： 杂志文章
doi：10.1093/nar/gkg291
更新日期：2003-04-01 00:00:00
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journal_title：Nucleic acids research
pub_type： 杂志文章
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更新日期：1974-11-01 00:00:00
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journal_title：Nucleic acids research
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更新日期：1995-08-25 00:00:00
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更新日期：2006-08-11 00:00:00
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journal_title：Nucleic acids research
pub_type： 杂志文章
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更新日期：2007-01-01 00:00:00
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更新日期：1983-07-11 00:00:00
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pub_type： 杂志文章
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更新日期：1995-10-25 00:00:00
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journal_title：Nucleic acids research
pub_type： 杂志文章
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更新日期：1993-02-11 00:00:00
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pub_type： 杂志文章
doi：10.1093/nar/9.6.1499
更新日期：1981-03-25 00:00:00
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更新日期：1993-10-11 00:00:00
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journal_title：Nucleic acids research
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更新日期：2016-11-02 00:00:00
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journal_title：Nucleic acids research
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更新日期：1998-04-01 00:00:00
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journal_title：Nucleic acids research
pub_type： 杂志文章
doi：10.1093/nar/8.1.67
更新日期：1980-01-11 00:00:00
