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Molecular Genetics and Metabolism
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October 8, 2021
Variants in the MIPEP gene presenting with complex neurological phenotype without cardiomyopathy, impair OXPHOS protein maturation and lead to a reduced OXPHOS abundance in patient cells
Juliette Pulman, Benedetta Ruzzenente, Martin Horak, et al.
Human Molecular Genetics
|
December 20, 2018
Mutations in the MRPS28 gene encoding the small mitoribosomal subunit protein bS1m in a patient with intrauterine growth retardation, craniofacial dysmorphism and multisystemic involvement
Juliette Pulman, Benedetta Ruzzenente, Lucas Bianchi, et al.
Nature Communications
|
November 18, 2017
LRPPRC-mediated folding of the mitochondrial transcriptome
Stefan J Siira, Henrik Spåhr, Anne-Marie J Shearwood, et al.
Developmental Medicine and Child Neurology
|
January 29, 2021
Cerebral blood flow and acute episodes of Leigh syndrome in neurometabolic disorders
Klervie Loiselet, Benedetta Ruzzenente, Charles-Joris Roux, et al.
Nature
|
October 10, 2014
POLRMT does not transcribe nuclear genes
Inge Kühl, Christian Kukat, Benedetta Ruzzenente, et al.
Human Molecular Genetics
|
February 9, 2013
TWINKLE is an essential mitochondrial helicase required for synthesis of nascent D-loop strands and complete mtDNA replication
Dusanka Milenkovic, Stanka Matic, Inge Kühl, et al.
Plos Genetics
|
February 12, 2014
NSUN4 is a dual function mitochondrial protein required for both methylation of 12S rRNA and coordination of mitoribosomal assembly
Metodi Dimitrov Metodiev, Henrik Spåhr, Paola Loguercio Polosa, et al.
Plos Genetics
|
October 25, 2011
The bicoid stability factor controls polyadenylation and expression of specific mitochondrial mRNAs in Drosophila melanogaster
Ana Bratic, Anna Wredenberg, Sebastian Grönke, et al.
Molecular Genetics and Metabolism Reports
|
July 2, 2026
Functional characterization of a biallelic <i>MIPEP</i> variant associated with global developmental delay, infantile epileptic spasms syndrome, and hypotonia
Benedetta Ruzzenente, Pierre-Hadrien Becker, Elissa Afram, et al.
Life (Basel, Switzerland)
|
February 25, 2023
Novel ELAC2 Mutations in Individuals Presenting with Variably Severe Neurological Disease in the Presence or Absence of Cardiomyopathy
Cérane Cafournet, Sofia Zanin, Anne Guimier, et al.
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Search research articles
Search
Showing results (11-20 of 36) with videos related to
Sort By:
Page
of 4
Molecular Genetics and Metabolism
|
October 8, 2021
Variants in the MIPEP gene presenting with complex neurological phenotype without cardiomyopathy, impair OXPHOS protein maturation and lead to a reduced OXPHOS abundance in patient cells
Juliette Pulman, Benedetta Ruzzenente, Martin Horak, et al.
Human Molecular Genetics
|
December 20, 2018
Mutations in the MRPS28 gene encoding the small mitoribosomal subunit protein bS1m in a patient with intrauterine growth retardation, craniofacial dysmorphism and multisystemic involvement
Juliette Pulman, Benedetta Ruzzenente, Lucas Bianchi, et al.
Nature Communications
|
November 18, 2017
LRPPRC-mediated folding of the mitochondrial transcriptome
Stefan J Siira, Henrik Spåhr, Anne-Marie J Shearwood, et al.
Developmental Medicine and Child Neurology
|
January 29, 2021
Cerebral blood flow and acute episodes of Leigh syndrome in neurometabolic disorders
Klervie Loiselet, Benedetta Ruzzenente, Charles-Joris Roux, et al.
Nature
|
October 10, 2014
POLRMT does not transcribe nuclear genes
Inge Kühl, Christian Kukat, Benedetta Ruzzenente, et al.
Human Molecular Genetics
|
February 9, 2013
TWINKLE is an essential mitochondrial helicase required for synthesis of nascent D-loop strands and complete mtDNA replication
Dusanka Milenkovic, Stanka Matic, Inge Kühl, et al.
Plos Genetics
|
February 12, 2014
NSUN4 is a dual function mitochondrial protein required for both methylation of 12S rRNA and coordination of mitoribosomal assembly
Metodi Dimitrov Metodiev, Henrik Spåhr, Paola Loguercio Polosa, et al.
Plos Genetics
|
October 25, 2011
The bicoid stability factor controls polyadenylation and expression of specific mitochondrial mRNAs in Drosophila melanogaster
Ana Bratic, Anna Wredenberg, Sebastian Grönke, et al.
Molecular Genetics and Metabolism Reports
|
July 2, 2026
Functional characterization of a biallelic <i>MIPEP</i> variant associated with global developmental delay, infantile epileptic spasms syndrome, and hypotonia
Benedetta Ruzzenente, Pierre-Hadrien Becker, Elissa Afram, et al.
Life (Basel, Switzerland)
|
February 25, 2023
Novel ELAC2 Mutations in Individuals Presenting with Variably Severe Neurological Disease in the Presence or Absence of Cardiomyopathy
Cérane Cafournet, Sofia Zanin, Anne Guimier, et al.
Page
of 4