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Ingrid Tein

Title:
Director, Neurometabolic Clinic, Investigational Unit and Research Laboratory
Designations:
B.Sc., MD, FRCP (C)
Phone:
416-813-5041
Email:
Ingrid.tein@sickkids.ca
Alternate Contact Name:
Wendy Bennett
Alternate Phone:
416-813-5668
Alternate Email:
Wendy.bennett@sickkids.ca
U of T Positions:
Associate Professor of Paediatrics, Laboratory Medicine and Pathobiology

Biography

In 1991, Ingrid Tein founded and continues to direct the Neurometabolic Clinic, Neuroinvestigational Unit and Neurometabolic Research Laboratory at The Hospital for Sick Children (SickKids) for the prioritized clinical, biochemical and molecular investigation of children for a spectrum of fatty acid oxidation (FAO) disorders.

This is a unique facility in Canada which is internationally recognized. Tein's team receives referrals from throughout Canada, U.S., Caribbean, Europe, Middle East, Africa and South America. They have investigated the mechanisms of disease pathogenesis at a biochemical and molecular level through the intensive study of specific genetic defects, in order to provide insight into the precise correlation between genotype and phenotype and to provide key information regarding the protein and molecular basis of both normal and abnormal human FAO.

Using a translational bench-to-bedside approach, Tein's team has identified novel phenotypes and genotypes (e.g. defects of SCAD, LCHAD/TFP, CPT II), developed new diagnostic screening tests and new treatment strategies aimed at bypassing or correcting the specific metabolic block (e.g. DHA reversal of neuropathy in LCHAD deficiency; rescue by antioxidants in SCADD cells), which in the aggregate have decreased long-term morbidity and mortality in affected individuals and have been implemented internationally. They also investigate children with mitochondrial, glycolytic/glycogenolytic, and vitamin/cofactor responsive disorders, etc. who may manifest with recurrent encephalopathy/seizures, movement disorders or exercise intolerance, motor weakness and/or recurrent myoglobinuria.

The team runs prospective cross-over targeted cofactor treatment trials in children with mitochondrial disorders with q3- monthly clinical, neurological, biochemical, QOL and ergometric assessments to determine treatment efficacy and have identified abnormalities of muscle metabolism by 31P-MRS cycle ergometry. 

Research

The primary focus of Tein's research program is to increase understanding of the developmental expression, roles and interaction of the carnitine/organic cation transporter family (OCTN1, OCTN2, OCTN3) in maintaining intracellular carnitine (Cn) homeostasis for efficient fatty acid oxidation (FAO) and ATP production.

The secondary focus of her program has been the prioritized clinical, biochemical and molecular investigation of children in her team's neurometabolic clinic for FAO or mitochondrial defects and targeted treatment development.

Presently, they're conducting a randomized, double-blind cross over study to evaluate the efficacy of CoQ10  on cognitive and motor function in Prader Willi Syndrome based upon our identification of muscle Complex 1 deficiency in a small PWS cohort, providing the groundwork for a new therapeutic target.

Education and experience

  • 1972–1976: Honours B.Sc., University of Toronto, Toronto, ON
  • 1976–1979: MD, University of Toronto, Toronto, ON
  • 1979–1980: Mixed internships, University of Toronto, Toronto, ON
  • 1980: Licensure of Medical Council of Canada
  • 1980–1982: Residency Paediatrics, SickKids, University of Toronto, Toronto, Ontario
  • 1982–1983: Residency Paediatrics, McMaster University Medical Centre, Hamilton, ON
  • 1984: FRCP (C), Canadian Board of Paediatrics, Canada
  • 1983–1986: Residency Paediatric Neurology, SickKids, University of Toronto, Toronto, ON
  • 1986: Ontario General Medical Licence
  • 1987–1989: Duncan L. Gordon Research Fellowship, SickKids, University of Toronto
  • 1987–1988: Clinique et Unite de Recherche Genetique Medical, Hopital Necker Enfant Malades, Inserm U 75, Paris, France
  • 1988–1990: Research Fellowship (Von Aitken Coma Foundation Trust and American MDA Research Fellowship), Dept. of Neurology, Columbia University, New York, NY, U.S 

Achievements

  • Jan. 1991–Dec. 1993: S. Lunenfeld Foundation Scholarship, SickKids
  • July 1992–June 1997: Medical Research Council of Canada Scholarship
  • July 1992–June 1993: William A. Hawke Award, Clinical Excellence in Teaching, Division of Neurology, The Hospital for Sick Children, Toronto, Ontario
  • June 1993: Selected by Medical Research Council of Canada to represent Canada at Lindau meeting of Nobel Laureates, Lindau, Germany
  • Sept. 1994–Aug. 1995: Appointment to Dean's Research Advisory Committee, University of Toronto 
  • Sept. 1998: John Stobo Prichard Award Young Investigator Award given every four years by International Child Neurology Association (ICNA) for significant contribution to systematic clinical and/or basic research . Presented at 8th ICNA Congress, Ljubljana, Slovenia. Sept. 13-18, 1998
  • May 2002: Young Investigator Scholarship Paediatric Neurotransmitter Symposium, NIH, Washington,  DC. May 17-19, 2002
  • Sept. 2002–June, 2022: Elected Member, International Child Neurology Association Executive Board (elected 5 consecutive 4-year terms) 
  • Jan. 2004–Dec. 2004: Detweiler Travelling Fellowship, Awarded by Royal College of Physicians and Surgeons of Canada for support of sabbatical in Pediatric Metabolic Exercise Physiology 
  • Nov. 2007: Teaching Award, Hong Kong Society of Child Neurology and Developmental Pediatrics, University of Hong Kong
  • July 2007–June 2008: Pediatric Subspecialty Teaching Award. Dept. Pediatrics, Hospital for Sick Children (2nd place), University of Toronto
  • July, 2008–June 2009: William A. Hawke Award, Clinical Excellence in Teaching, Division of Neurology, The Hospital for Sick Children, University of Toronto, Ontario
  • Jun. 2010: Invited top grant recipient. Mitochondrial Medicine 2010 Symposium. United Mitochondrial Diseases Foundation Annual Congress, Scottsdale, Arizona. June 16-19, 2010
  • Nov. 2011–June, 2014: Chair, Research Task Force, International Child Neurology Association
  • March 2012: John H. Menkes Award, Endowed Memorial Lectureship in Pediatric Neurology, Division of Pediatric Neurology, UCLA, California, USA. Given in honour of John Menkes and in recognition of significant contributions to neurometabolic diseases in Child Neurology.
  • May 2012–May, 2014: President-Elect, International Child Neurology Association
  • May 2013: Colleen Giblin Memorial Endowed Lectureship in Pediatric Neurology, Dept. of Neurology, Columbia University, New York, NY, USA. 
    Given in recognition of significant research contributions to neurometabolic diseases in Child Neurology
  • July 2012–June 2013: William A. Hawke Award, Clinical Excellence in Teaching, Division of Neurology, The Hospital for Sick Children, University of Toronto, Ontario
  • May 2014–Nov. 2018: President, The International Child Neurology Association  International organization of > 2700 child neurologists to promote excellence in clinical care, education and research internationally and to promote collaborations between resource limited and resource rich regions optimizing the neurological care and development of children globally . 
  • May 2014–May 2022: Member, World Brain Alliance – collaboration of executives of international professional Neurological, Neurosurgical, Psychiatric and Rehabilitation Organizations to raise awareness and reduce the global burden of brain and mental health diseases
  • Jan. 2014–Jan. 2017: Co-Chair, NINDS International Mitochondrial Disease Common Data Element Working Group for Toolkit Devt.
  • 2018: Co-Scientific Director of Canadian Mitochondrial Network and Director of Clinical and Therapeutic Discovery Pillar 
  • Sept. 18: Member, External Advisory Committee, North American Mitochondrial Disease Consortium (NAMDC)
  • 2020: Biography included as International Founder in book “ Child Neurology. Its Origins, Founders, Growth and Evolution 2e”.  1960- 2000 “

Publications

  1. Verbeeten KC, Lamhonwah AM, Bulman D, Faghfoury H, Chakraborty P, Tein I *, Geraghty MT*. Carnitine uptake defect due to a 5'UTR mutation in a pedigree with false positives and false negatives on Newborn screening.  Mol Genet Metab. 2020 Mar;129(3):213-218. doi: 10.1016/j.ymgme.2019.12.006. Epub 2019 Dec 10. PMID: 31864849  (* Co-SRAs)
  2. Rodan LH, Wells GD, Banks L, Thompson S, Schneiderman JE, Tein I. L-Arginine Affects Aerobic Capacity and Muscle Metabolism in MELAS (Mitochondrial Encephalomyopathy, Lactic Acidosis and Stroke-Like Episodes) Syndrome. PLoS One. 2015 May 20;10(5):e0127066. doi: 10.1371/journal.pone.0127066. eCollection 2015. PMID: 25993630
  3. Rodan LH, Poublanc J, Fisher JA, Sobczyk O, Wong T, Hlasny E, Mikulis D, Tein I. Cerebral hyperperfusion and decreased cerebrovascular reactivity correlate with neurologic disease severity in MELAS. Mitochondrion. 2015 May;22:66-74. doi: 10.1016/j.mito.2015.03.002. Epub 2015 Mar 21.PMID: 25801712 
  4. Zolkipli Z, Pedersen CB, Lamhonwah AM, Gregersen N, Tein I. Vulnerability to oxidative stress in vitro in pathophysiology of mitochondrial short-chain acyl-CoA dehydrogenase deficiency: response to antioxidants. PLoS One. 2011 Apr 1;6(4):e17534. doi: 10.1371/journal.pone.0017534.PMID: 21483766 
  5. Lamhonwah AM, Skaug J, Scherer SW, Tein I. A third human carnitine/organic cation transporter (OCTN3) as a candidate for the 5q31 Crohn's disease locus (IBD5). Biochem Biophys Res Commun. 2003 Jan 31;301(1):98-101. doi: 10.1016/s0006-291x(02)02946-7.PMID: 12535646 

Past (selected)

Medical Research Council of Canada Operating Grant (1992–1995) 
"Characterization of the plasmalemmal carnitine transporter." 
Principal Investigator : I. Tein  
 
Physicians' Services Incorporated Foundation Grant, Ontario (1992–1998) 
"Study of the pathophysiology of recurrent childhood myoglobinuria 
and lipid storage myopathies secondary to genetic defects of fatty acid 
oxidation and development of new treatment strategies."  
Principal Investigator: I. Tein 
 
Heart and Stroke Foundation of Ontario Grant (1996–2002) 
"Molecular Characterization of the Plasmalemmal Carnitine Transporter " 
Principal Investigator: I. Tein  
 
Physicians’ Services Incorporated Foundation Grant (2001–2003) 
 “The Role of Defective Carnitine Transport in Male Infertility “ 
Principal Investigator: I. Tein  
 
Physicians’ Services Incorporated Foundation Grant (2003–2006) 
“The Role of Carnitine Transporters in Sperm Motility and Maturation and Male Fertility. “Principal Investigator: I. Tein 
 
Mead-Johnson Grant (2003–2009) 
“Role of the Carnitine/Organic Cation Transporters in the Mammary Gland and Implications for the Suckling Infant “ Principal Investigator: I. Tein 
 
Heart and Stroke Foundation of Ontario Grant (2002–2007) 
“Carnitine-Responsive Cardiomyopathy & the Different-Affinity Carnitine Transporters “ Principal Investigator: I. Tein 
 
Muscular Dystrophy Grant– Hospital for Sick Children Foundation (2007–2008) 
“The Role of Muscle Carnitine Deficiency and the Carnitine/Organic Cation Transporter Family in Duchenne Muscular Dystrophy “  
Principal Investigator: I. Tein 
 
Guillain Barre Syndrome Grant – The Hospital for Sick Children Foundation (2007–2008) 
“Shared Epitopes of Functional Variants of the Organic Cation/Carnitine Transporter family (OCTN1 and OCTN2) and Campylobacter jejuni may underlie susceptibility to Crohn’s Disease and Guillain-Barre Syndrome”  
Principal Investigator: I. Tein 
 
Canadian Institutes of Health Research (2008–2013) 
CIHR Team in Childhood Obesity Research  
Principal Investigator: Brian McCrindle 
Co-PI’s: H. Anderson, J. Hamilton, P. Parkin, P. Pencharz 
Co-applicant: I. Tein 
 
Mead-Johnson Grant (2010–2012) 
“Role of organic cation/carnitine (OCTN/Cn) transporter family in developing murine heart “  
Principal Investigator: I. Tein 
 
United Mitochondrial Diseases Foundation (2010–2013) 
“Efficacy of L-arginine therapy on vasodilatation and mitochondria in MELAS syndrome” 
Principal Investigator: I. Tein 
 
Rare Diseases Foundation Grant (2012–2013) 
“Characterization of the carnitine transporter gene OCTN3 in patients with peroxisomal dysfunction using Next-Generation Sequencing.” 
Principal Investigators: I. Tein, A. Lamhonwah 
 
Rare Diseases Foundation Grant (2012–2013) 
“Dysregulation of fatty acid oxidation by defective SMN1 gene: Implications for therapy.” 
Principal Investigator: I. Tein 
 
The Myositis Association (2015–2018) 
“Creatine Supplementation in Childhood Myositis “ 
PI: Brian Feldman 
Co-investigators: I. Tein, A, Schulze, G. Wells,  S. Stephens 

Current

Foundation for Prader-Willi Research (2019–2021) 
“Mitochondrial Complex I dysfunction in Prader Willi Syndrome: A New therapeutic target.”  
Principal Investigator: I. Tein 
 
Physicians’ Services Incorporated Foundation of Ontario (2015–2020) 
“Identification of Carnitine-responsive cardiomyopathy and myopathy in adult patients with dilated and hypertrophic cardiomyopathy and limb girdle weakness.”   
Principal Investigator: Hannaneh Faghfoury,  Co-PI: Ingrid Tein 

“The effect of exercise, creatine and coenzyme Q10 supplementation on muscle  
function in children with myositis.” 
Principal Investigator: B. Feldman 
Co-Investigators: I. Tein, S. Stephens, G. Wells, A. Schulze 

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