List of works - Zofia M Chrzanowska-Lightowlers

Mitochondrial dysfunction in a cell culture model of familial amyotrophic lateral sclerosis

scientific article published on July 2002

A functional peptidyl-tRNA hydrolase, ICT1, has been recruited into the human mitochondrial ribosome

scientific article

GRSF1 regulates RNA processing in mitochondrial RNA granules.

scientific article published on March 2013

Human mitochondrial mRNAs--like members of all families, similar but different

scientific article published on 6 March 2010

Mutations in C12orf65 in patients with encephalomyopathy and a mitochondrial translation defect

scientific article

mtRF1a is a human mitochondrial translation release factor decoding the major termination codons UAA and UAG

scientific article

Autophagy impairment with lysosomal and mitochondrial dysfunction is an important characteristic of oxidative stress-induced senescence

scientific article published on 28 October 2016

Nuclear DNA-encoded tRNAs targeted into mitochondria can rescue a mitochondrial DNA mutation associated with the MERRF syndrome in cultured human cells.

scientific article

The human mitochondrial ribosome recycling factor is essential for cell viability

scientific article published on 9 September 2008

An mtDNA mutation in the initiation codon of the cytochrome C oxidase subunit II gene results in lower levels of the protein and a mitochondrial encephalomyopathy

scientific article

Overexpression of human mitochondrial valyl tRNA synthetase can partially restore levels of cognate mt-tRNAVal carrying the pathogenic C25U mutation

scientific article published on 8 April 2008

Nuclear factors involved in mitochondrial translation cause a subgroup of combined respiratory chain deficiency

scientific article published on 17 December 2010

Annexin A2 binds to the localization signal in the 3′ untranslated region of c-myc mRNA

article

The p.M292T NDUFS2 mutation causes complex I-deficient Leigh syndrome in multiple families

scientific article published on September 6, 2010

Molecular basis of infantile reversible cytochrome c oxidase deficiency myopathy

scientific article published on 31 August 2009

Store-Operated Ca2+ Entry Controls Induction of Lipolysis and the Transcriptional Reprogramming to Lipid Metabolism

scientific article

Defective mitochondrial mRNA maturation is associated with spastic ataxia

scientific article

Mutation of the human mitochondrial phenylalanine-tRNA synthetase causes infantile-onset epilepsy and cytochrome c oxidase deficiency

scientific article published on 24 October 2013

A human mitochondrial poly(A) polymerase mutation reveals the complexities of post-transcriptional mitochondrial gene expression.

scientific article

Mutations in mitochondrial ribosomal protein MRPL12 leads to growth retardation, neurological deterioration and mitochondrial translation deficiency

scientific article published on 18 April 2013

Inhibition of mitochondrial protein synthesis promotes increased stability of nuclear-encoded respiratory gene transcripts

article

LRPPRC mutations cause early-onset multisystem mitochondrial disease outside of the French-Canadian population.

scientific article published on 27 October 2015

Mitochondrial protein synthesis: figuring the fundamentals, complexities and complications, of mammalian mitochondrial translation.

scientific article

Human mitochondrial leucyl tRNA synthetase can suppress non cognate pathogenic mt-tRNA mutations

scientific article published on 10 January 2014

Targeting of the cytosolic poly(A) binding protein PABPC1 to mitochondria causes mitochondrial translation inhibition

scientific article

A microtiter plate assay for cytochrome c oxidase in permeabilized whole cells

scientific article published on October 1993

The mRNA-binding protein COLBP is glutamate dehydrogenase

article

REXO2 is an oligoribonuclease active in human mitochondria.

scientific article

The process of mammalian mitochondrial protein synthesis.

scientific article

Serum-deprivation stimulates cap-binding by PARN at the expense of eIF4E, consistent with the observed decrease in mRNA stability

scientific article

PPR (pentatricopeptide repeat) proteins in mammals: important aids to mitochondrial gene expression

article

Human pentatricopeptide proteins: only a few and what do they do?

scientific article published on 23 April 2013

Polyadenylation and degradation of mRNA in mammalian mitochondria: a missing link?

scientific article

Intracellular mitochondrial triplasmy in a patient with two heteroplasmic base changes

scientific article

Differences in RNA processing underlie the tissue specific phenotype of ISCU myopathy

scientific article

MTO1 mediates tissue specificity of OXPHOS defects via tRNA modification and translation optimization, which can be bypassed by dietary intervention

scientific article

Termination of Protein Synthesis in Mammalian Mitochondria

scientific article published on August 26, 2011

Human mitochondrial ribosomes can switch their structural RNA composition.

scientific article published on 11 October 2016

Functional polypeptides can be synthesized from human mitochondrial transcripts lacking termination codons

scientific article

The Pseudouridine Synthase RPUSD4 Is an Essential Component of Mitochondrial RNA Granules

scientific article published on 12 January 2017

175th ENMC International Workshop: Mitochondrial protein synthesis in health and disease, 25–27th June 2010, Naarden, The Netherlands

article

Structure based hypothesis of a mitochondrial ribosome rescue mechanism

scientific article

The Mouse Tumor Cell Lines EL4 and RMA Display Mosaic Expression of NK-Related and Certain Other Surface Molecules and Appear to Have a Common Origin

scientific article published on 01 May 2000

TGF-beta1 and IFN-gamma cross-regulate antigen presentation to CD4 T cells by macrophages

scientific article published on July 2002

Production of transmitochondrial cybrids containing naturally occurring pathogenic mtDNA variants.

scientific article published on 2 August 2006

Sodium channel mRNAs at the neuromuscular junction: distinct patterns of accumulation and effects of muscle activity

scientific article

SLIRP stabilizes LRPPRC via an RRM-PPR protein interface

scientific article published on 28 June 2016

Gene therapy for mitochondrial DNA defects: is it possible?

scientific article

A novel point mutation in the mitochondrial tRNA(Trp) gene produces a neurogastrointestinal syndrome

scientific article published in June 2004

A truncating PET100 variant causing fatal infantile lactic acidosis and isolated cytochrome c oxidase deficiency

scientific article

List of works by Zofia M Chrzanowska-Lightowlers

Mitochondrial dysfunction in a cell culture model of familial amyotrophic lateral sclerosis

A functional peptidyl-tRNA hydrolase, ICT1, has been recruited into the human mitochondrial ribosome

GRSF1 regulates RNA processing in mitochondrial RNA granules.

Human mitochondrial mRNAs--like members of all families, similar but different

Mutations in C12orf65 in patients with encephalomyopathy and a mitochondrial translation defect

mtRF1a is a human mitochondrial translation release factor decoding the major termination codons UAA and UAG

Autophagy impairment with lysosomal and mitochondrial dysfunction is an important characteristic of oxidative stress-induced senescence

Nuclear DNA-encoded tRNAs targeted into mitochondria can rescue a mitochondrial DNA mutation associated with the MERRF syndrome in cultured human cells.

The human mitochondrial ribosome recycling factor is essential for cell viability

An mtDNA mutation in the initiation codon of the cytochrome C oxidase subunit II gene results in lower levels of the protein and a mitochondrial encephalomyopathy

Overexpression of human mitochondrial valyl tRNA synthetase can partially restore levels of cognate mt-tRNAVal carrying the pathogenic C25U mutation

Nuclear factors involved in mitochondrial translation cause a subgroup of combined respiratory chain deficiency

Annexin A2 binds to the localization signal in the 3′ untranslated region of c-myc mRNA

The p.M292T NDUFS2 mutation causes complex I-deficient Leigh syndrome in multiple families

Molecular basis of infantile reversible cytochrome c oxidase deficiency myopathy

Store-Operated Ca2+ Entry Controls Induction of Lipolysis and the Transcriptional Reprogramming to Lipid Metabolism

Defective mitochondrial mRNA maturation is associated with spastic ataxia

Mutation of the human mitochondrial phenylalanine-tRNA synthetase causes infantile-onset epilepsy and cytochrome c oxidase deficiency

A human mitochondrial poly(A) polymerase mutation reveals the complexities of post-transcriptional mitochondrial gene expression.

Mutations in mitochondrial ribosomal protein MRPL12 leads to growth retardation, neurological deterioration and mitochondrial translation deficiency

Inhibition of mitochondrial protein synthesis promotes increased stability of nuclear-encoded respiratory gene transcripts

LRPPRC mutations cause early-onset multisystem mitochondrial disease outside of the French-Canadian population.

Mitochondrial protein synthesis: figuring the fundamentals, complexities and complications, of mammalian mitochondrial translation.

Human mitochondrial leucyl tRNA synthetase can suppress non cognate pathogenic mt-tRNA mutations

Targeting of the cytosolic poly(A) binding protein PABPC1 to mitochondria causes mitochondrial translation inhibition

A microtiter plate assay for cytochrome c oxidase in permeabilized whole cells

The mRNA-binding protein COLBP is glutamate dehydrogenase

REXO2 is an oligoribonuclease active in human mitochondria.

The process of mammalian mitochondrial protein synthesis.

Serum-deprivation stimulates cap-binding by PARN at the expense of eIF4E, consistent with the observed decrease in mRNA stability

PPR (pentatricopeptide repeat) proteins in mammals: important aids to mitochondrial gene expression

Human pentatricopeptide proteins: only a few and what do they do?

Polyadenylation and degradation of mRNA in mammalian mitochondria: a missing link?

Intracellular mitochondrial triplasmy in a patient with two heteroplasmic base changes

Differences in RNA processing underlie the tissue specific phenotype of ISCU myopathy

MTO1 mediates tissue specificity of OXPHOS defects via tRNA modification and translation optimization, which can be bypassed by dietary intervention

Termination of Protein Synthesis in Mammalian Mitochondria

Human mitochondrial ribosomes can switch their structural RNA composition.

Functional polypeptides can be synthesized from human mitochondrial transcripts lacking termination codons

The Pseudouridine Synthase RPUSD4 Is an Essential Component of Mitochondrial RNA Granules

175th ENMC International Workshop: Mitochondrial protein synthesis in health and disease, 25–27th June 2010, Naarden, The Netherlands

Structure based hypothesis of a mitochondrial ribosome rescue mechanism

The Mouse Tumor Cell Lines EL4 and RMA Display Mosaic Expression of NK-Related and Certain Other Surface Molecules and Appear to Have a Common Origin

TGF-beta1 and IFN-gamma cross-regulate antigen presentation to CD4 T cells by macrophages

Production of transmitochondrial cybrids containing naturally occurring pathogenic mtDNA variants.

Sodium channel mRNAs at the neuromuscular junction: distinct patterns of accumulation and effects of muscle activity

SLIRP stabilizes LRPPRC via an RRM-PPR protein interface

Gene therapy for mitochondrial DNA defects: is it possible?

A novel point mutation in the mitochondrial tRNA(Trp) gene produces a neurogastrointestinal syndrome

A truncating PET100 variant causing fatal infantile lactic acidosis and isolated cytochrome c oxidase deficiency