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Mr Julien Baruteau
Institure for Women's Health
86-96 Chenies Mews
  • Senior Clinical Research Associate
  • ICH Genetics & Genomic Medicine Prog
  • UCL GOS Institute of Child Health
  • Faculty of Pop Health Sciences

I am a Research Training Fellow in the Gene Transfer Technology Group at University College London since 2013 and a Honorary Clinical Fellow in Metabolic Medicine, Great Ormond Street Hospital in London. Prior to this post, I trained in Toulouse, France where I graduated as Paediatrician in 2008. I specialised in Inherited Metabolic Diseases in Paris and Toulouse, France where I worked as a Locum Consultant during four years. I gained experience in Liver Cell Therapy in Metabolic Diseases in Pr Sokal's laboratory, Catholic University of Louvain, Brussels, in Belgium in 2011. Since 2012 I have joined the Metabolic team in Great Ormond Street Hospital as a Clinical Fellow. In 2013, I performed a national survey in the UK about a urea cycle defect called argininosuccinic aciduria, a rare inherited metabolic disorder. In order to improve patients' outcome, Action Medical Research supports my efforts to develop a gene therapy approach for this disease.

Research Themes
Research Summary

Arginino-succinate lyase (Asl) is a key enzyme for both urea and citrulline-NO cycles. Patients with ASL deficiency develop a multisystemic disease, arginosuccinic aciduria (ASA), affecting predominantly the liver and the brain. ASA (OMIM 207900) is an autosomal recessive argininosuccinate lyase deficiency with an incidence of 1:70,000 in the UK, which makes this disease the second most common urea cycle defect. ASA patients have a more complex clinical phenotype compared to other urea cycle defects with a higher incidence of neurocognitive symptoms, cirrhosis and systemic hypertension. Unlike other types of urea cycle disorders (UCD) the severity of the brain disease in ASA cannot be explained by hyperammonaemia and is thought to be caused by a neuronal deficiency of nitric oxide (NO). The aim of my project is to select the best approach for gene therapy in ASA that can be progressed to a human trial within 5 years. Gene therapy using early neonatal injections for ASA is a promising approach with several potential advantages including 1) a potentially low immune response, 2) the avoidance of neurological damage, 3) smaller doses of vectors required to achieve sufficient level of transduction.

Teaching Summary
My teaching activities are supervising and tutoring students training in the lab.
Academic Background
2012 DM Doctor of Medicine – Cell Biology Universite Paul Sabatier (Toulouse III)
2008 DM Doctor of Medicine – Paediatrics Universite Paul Sabatier (Toulouse III)
2004 BMBS Bachelor of Medicine & Bachelor of Surgery – Medicine Universite de Bourgogne
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