The number of tRNA isodecoders has increased dramatically in mammals, but the specific molecular and physiological reasons for this expansion remain elusive. To address this fundamental question we used CRISPR editing to knockout the seven-membered phenylalanine tRNA gene family in mice, both individually and combinatorially. ...

• Telethon Kids Institute
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• Reports and Findings
• 2023
• April 2023

The mitochondrial genome encodes core subunits of the respiratory chain that drives oxidative phosphorylation and is, therefore, essential for energy conversion. Advances in high-throughput sequencing technologies and cryoelectron microscopy have shed light on the structure and organization of the mitochondrial genome and revealed unique mechanisms of mitochondrial gene regulation. ...

• Telethon Kids Institute
• Our research
• Reports and Findings
• 2022
• April 2022

The evolutionary acquisition of mitochondria has given rise to the diversity of eukaryotic life. Mitochondria have retained their ancestral α-proteobacterial traits through the maintenance of double membranes and their own circular genome. Their genome varies in size from very large in plants to the smallest in animals and their parasites. The mitochondrial genome encodes essential genes for protein synthesis and has to coordinate its expression with the nuclear genome from which it sources most of the proteins required for mitochondrial biogenesis and function. ...

• Telethon Kids Institute
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• Reports and Findings
• 2021
• July 2021

We conclude that the novel chocolate-based formulation of midazolam provides improved tolerability while remaining efficacious ...

• Telethon Kids Institute
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• 2018
• December 2018

Research priority setting activities in paediatric chronic disease cover many discipline areas and have elicited a broad range of topics ...

• Telethon Kids Institute
• Our research
• Reports and Findings
• 2018
• October 2018

A simple, clinically relevant model to reliably discriminate patients at high and lower risk of death in patients with malignant pleural mesothelioma ...

• Telethon Kids Institute
• Our research
• Reports and Findings
• 2016
• April 2016

Higher levels of paternal dietary vitamin B12 were appeared to be associated with an increased risk of childhood ALL, with those in the highest tertile of... ...

• Telethon Kids Institute
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• Reports and Findings
• 2014
• October 2014

Maternal occupational pesticide exposure during pregnancy and/or paternal occupational pesticide exposure around conception have been suggested to increase... ...

• Telethon Kids Institute
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• Reports and Findings
• 2014
• March 2014

The evidence that accelerated fetal growth is associated with a modest increased risk of childhood ALL is strong and consistent with known biological... ...

• Telethon Kids Institute
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• Reports and Findings
• 2013
• June 2013

It is unknown whether parental occupational exposure to chemicals before during and after pregnancy increases the risk of acute lymphoblastic... ...

• Telethon Kids Institute
• Our research
• Reports and Findings
• 2011
• November 2011

Raelene Endersby R BSc (Hons) PhD Brainchild Fellow; Co-Head, Brain Tumour Research raelene.endersby@telethonkids.org.au Co-Head, Brain Tumour Research Raelene was awarded her PhD in 2003 from the Harry Perkins Institute for Medical Research (under ...

• Telethon Kids Institute
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Mitochondrial ribosomes are specialized for the synthesis of membrane proteins responsible for oxidative phosphorylation. Mammalian mitoribosomes have diverged considerably from the ancestral bacterial ribosomes and feature dramatically reduced ribosomal RNAs. The structural basis of the mammalian mitochondrial ribosome assembly is currently not well understood. Here we present eight distinct assembly intermediates of the human large mitoribosomal subunit involving seven assembly factors. ...

• Telethon Kids Institute
• Our research
• Reports and Findings
• 2021
• June 2021