Notice bibliographique
Résumé
Dr. Robinson joined the University of Toronto in 1973 and founded the Congenital Lactic Acidosis Diagnostic and Research Laboratory at SickKids, which is responsible for the biochemical and genetic investigation of mitochondrial disorders with many national and international referrals each year. Dr. Robinson was appointed Professor in 1985, and served as Programme Head for Metabolism (1998-2006) and Acting Programme Head for Cancer and Blood (1999-2002) at SickKids. In 2001 he was awarded the prestigious CIHR Canada Research Chair in Metabolism and Nutrition. In 1998 he was the recipient of the international Barsky Lectureship in Mitochondrial Diseases and in 2011 was honoured with the distinguished Jacob's Ladder Norman Saunders International Research Award in Neurodegenerative Diseases. His work was continuously funded by the Medical Research Council of Canada/Canadian Institutes for Health Research, Heart and Stroke Foundation of Canada, Genome Canada, March of Dimes and UMDF. He published over 290 articles and reviews and spoke on Lactic Acidemia and mitochondrial diseases, giving over 80 invited national and international lectures. He is the author of the Lactic Acidemia chapter in “The Metabolic and Molecular Bases of Inherited Disease”. He accrued over 12 000 citations and had an exceptional h-index of 60. He also served on the editorial board for Pediatric Research (1999) and on peer review committees for the Canadian Genetic Diseases Network for Centres of Excellence (2002) and UMDF (2001). Dr. Robinson identified more than 10 novel diseases and provided the first clinical descriptions of many more. He did seminal work in the biochemical and molecular characterization of pyruvate dehydrogenase and pyruvate carboxylase deficiencies and various mitochondrial DNA defects and demonstrated underlying pathophysiologic mechanisms as well as correlated mutations with their clinical phenotypes. He showed that in certain mitochondrial respiratory chain defects presenting in childhood, it was the degree of enhancement of oxygen free radical production produced by the genetic lesion that largely correlated with the severity of the disease phenotype. He was part of the team that identified the defective LRPPRC gene affecting cytochrome oxidase as a cause of Leigh syndrome in French Canadian children from the Saguenay region of Quebec and showed how the LRPPRC functions as an mRNA stabilizing protein in mitochondria. His legacy of innovative work earned him profound respect from both his international scientific peers and appreciative families. He mentored numerous PhD and MSc students and postdoctoral fellows for whom he was an exceptional teacher. Known for his infectious laughter, unwavering support, and kindness, he became a lifelong friend to all his staff. His motto was “Once you join the Robinson lab, you are part of it for life!” He retired in March 2012 and spent the last years of his life with his wife Joyce, sons Glynn, Owain, and Alex, grandchildren, nieces, brother Roy, and sister Beryl. He will be deeply missed by all who were fortunate enough to have known him.
Récupéré en direct depuis OpenAlex et désinversé. Les résumés ne sont pas conservés dans cette base de données : les index inversés représentent 8,6 Go des 9,3 Go de texte de la base, et le serveur dispose de 13 Go libres.
Comment cette classification a été obtenuedéplier
Prédiction machine sur la base complète
Imitation des enseignantsNi prévalence calibrée, ni vérité terrain. Validation humaine à venir. Le volet Gemma est une étiquette directe du modèle pour chaque travail de la base, lue sur la notice réduite au titre. Le volet Codex est un classifieur appris des 10 348 étiquettes directes de Codex et calibré sur les taux pondérés de l'échantillon; les champs sans appui suffisant ne portent aucun appel Codex. Le mode candidate est l'union des deux volets; le consensus est leur intersection. Ces sorties portent le statut machine_predicted_unvalidated et ne sont pas des étiquettes humaines.
Scores du classifieur distillé par catégorie (deux têtes)
| Catégorie | Codex | Gemma |
|---|---|---|
| Métarecherche | 0,002 | 0,019 |
| Méta-épidémiologie (sens strict) | 0,001 | 0,001 |
| Méta-épidémiologie (sens large) | 0,001 | 0,001 |
| Bibliométrie | 0,001 | 0,001 |
| Études des sciences et des technologies | 0,002 | 0,001 |
| Communication savante | 0,003 | 0,003 |
| Science ouverte | 0,001 | 0,002 |
| Intégrité de la recherche | 0,004 | 0,008 |
| Charge utile insuffisante (le modèle a refusé de juger) | 0,200 | 0,179 |
Scores machine (provisoires)
Les deux têtes enseignantes du modèle étudiant, lues sur ce travail. Un score ordonne la base pour la relecture; il n'affirme jamais une catégorie, et le statut de validation accompagne chaque rangée tel quel.
Scores de référence d'un modèle non mature (critères de maturité non atteints, 7 itérations). Un score ordonne; il n'affirme jamais une catégorie.
score_only:v0-immature-baseline · tel quel depuis la passe de notation : score_only signifie que le nombre peut ordonner les travaux, et qu'aucune étiquette de catégorie n'en découleClassification
machine, non validéePrédiction automatique; un appel candidat d’une seule source (Gemma direct ou Codex distillé), pas un consensus.
Le détail, modèle par modèle et score par score, se trouve en fin de page sous « Comment cette classification a été obtenue ».