Genomics

Dataset Information

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Mitochondrial and nuclear genomic response to loss of LRPPRC expression


ABSTRACT: Rapid advances in genotyping and sequencing technology have dramatically accelerated the discovery of genes underlying human disease. Elucidating the function of such genes and understanding their role in pathogenesis, however, remains challenging. Here, we introduce a genomic strategy to functionally characterize such genes, and apply it to LRPPRC (leucine-rich PPR-motif containing), a poorly studied gene that is mutated in Leigh Syndrome, French Canadian type (LSFC). We utilize RNAi to engineer an allelic series of cellular models in which LRPPRC has been stably silenced to different levels of knockdown efficiency. Using expression profiling, we discovered a specific role for LRPPRC in the expression of all mitochondrial DNA (mtDNA)-encoded mRNAs, but not the rRNAs, without affecting nuclear genes encoding mitochondrial proteins.

ORGANISM(S): Homo sapiens

PROVIDER: GSE20847 | GEO | 2010/03/13

SECONDARY ACCESSION(S): PRJNA124753

REPOSITORIES: GEO

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