Paul M. Smith

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

Hans J C T Wessels, M. Huynen, R. Lightowlers, 2010, The EMBO journal.

The role of the mitochondrial ribosome in human disease: searching for mutations in 12S mitochondrial rRNA with high disruptive potential

Laura C. Greaves, Robert W. Taylor, R. Rodenburg, 2013, Human molecular genetics.

Molecular basis of infantile reversible cytochrome c oxidase deficiency myopathy

Robert W. Taylor, S. Dimauro, S. Marie, 2009, Brain : a journal of neurology.

ZMA, Taylor RW, Vila-Sanjurjo A. The presence of highly disruptive 16S rRNA mutations in clinical samples indicates a wider role for mutations of the mitochondrial ribosome in human disease. Mitochondrion 2015, 25, 17-27

Laura C. Greaves, Robert W. Taylor, R. Lightowlers, 2015 .

Strategies for treating disorders of the mitochondrial genome.

Robert W. Taylor, D. Turnbull, R. Lightowlers, 2004, Biochimica et biophysica acta.

Investigation of iron-sulfur protein maturation in eukaryotes.

J. Balk, A. Biederbick, R. Lill, 2007, Methods in molecular biology.

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

S. Seneca, Z. Chrzanowska-Lightowlers, R. Lightowlers, 2004, The Biochemical journal.

The crystal structure of MT0146/CbiT suggests that the putative precorrin-8w decarboxylase is a methyltransferase.

John F Hunt, G. DeTitta, J. Hunt, 2002, Structure.

The Identification of Multidrug-Resistant Microorganisms including Bergeyella zoohelcum Acquired from the Skin/Prosthetic Interface of Amputees and Their Susceptibility to Medihoney™ and Garlic Extract (Allicin)

Paul M. Smith, J. Blaxland, N. Rushmere, 2022, Microorganisms.

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

Robert W. Taylor, J. Kemp, P. Chinnery, 2010, Brain : a journal of neurology.

Heterologous Inferential Analysis (HIA) and Other Emerging Concepts: In Understanding Mitochondrial Variation In Pathogenesis: There is no More Low-Hanging Fruit.

J. Elson, Paul M. Smith, A. Vila-Sanjurjo, 2021, Methods in molecular biology.

Heterologous inferential analysis (HIA) as a method to understand the role of mitochondrial rRNA mutations in pathogenesis.

J. Elson, Paul M. Smith, A. Vila-Sanjurjo, 2015, Methods in molecular biology.

Altered 2-thiouridylation impairs mitochondrial translation in reversible infantile respiratory chain deficiency

P. Chinnery, R. Horvath, Paul M. Smith, 2013, Human molecular genetics.

Incidence and presentation of coeliac disease in South Glamorgan

Paul M. Smith, H. Jenkins, G. Swift, 2000, European journal of gastroenterology & hepatology.

The presence of highly disruptive 16S rRNA mutations in clinical samples indicates a wider role for mutations of the mitochondrial ribosome in human disease

Laura C. Greaves, Robert W. Taylor, R. Lightowlers, 2015, Mitochondrion.

The presence of highly disruptive 16 S rRNA mutations in clinical samples indicates a wider role for mutations of the mitochondrial ribosome in human disease Running Title : Role of the mitoribosomal large subunit rRNA in human disease

Laura C. Greaves, Robert W. Taylor, R. Lightowlers, 2015 .

Specific targeting of a DNA-alkylating reagent to mitochondria. Synthesis and characterization of [4-((11aS)-7-methoxy-1,2,3,11a-tetrahydro-5H-pyrrolo[2,1-c][1,4]benzodiazepin-5-on-8-oxy)butyl]-triphenylphosphonium iodide.

R. Lightowlers, Robin A. J. Smith, M. Murphy, 2003, European journal of biochemistry.

Bridging PNAs can bind preferentially to a deleted mitochondrial DNA template but replication by mitochondrial DNA polymerase gamma in vitro is not impaired.

Robert W. Taylor, D. Turnbull, R. Lightowlers, 2003, Biochimica et biophysica acta.

Acute liver failure with subsequent cirrhosis as the primary manifestation of TRMU mutations

H. Lochmüller, R. Horvath, B. Czermin, 2011, Journal of Inherited Metabolic Disease.

Structural analysis of mitochondrial rRNA gene variants identified in patients with deafness

J. Elson, Paul M. Smith, A. Vila-Sanjurjo, 2023, Frontiers in Physiology.

Altering the balance between healthy and mutated mitochondrial DNA

R. Lightowlers, Paul M. Smith, 2011, Journal of Inherited Metabolic Disease.

Our current understanding of the toxicity of altered mito-ribosomal fidelity during mitochondrial protein synthesis: What can it tell us about human disease?

J. F. Atkins, J. Elson, Paul M. Smith, 2023, Frontiers in Physiology.

This Work Is Licensed under a Creative Commons Attribution 3.0 Unported License Date Deposited: the Role of the Mitochondrial Ribosome in Human Disease: Searching for Mutations in 12s Mitochondrial Rrna with High Disruptive Potential

Laura C. Greaves, R. Rodenburg, R. Lightowlers, 2022 .

An antigenomic strategy for treating heteroplasmic mtDNA disorders.

D. Turnbull, R. Lightowlers, Paul M. Smith, 2001, Advanced drug delivery reviews.