Ingrid Tein

Dr Ingrid Tein is Director of the Neurometabolic Clinic and Research Laboratory and Staff Neurologist in the Division of Neurology; Associate Professor of Pediatrics, Laboratory Medicine and Pathobiology; and Senior Associate Scientist in the Genetics and Genome Biology Program of the Research Institute, The Hospital for Sick Children.  

Research Synopsis

The Neurometabolic Clinic (Division of Neurology, The Hospital for Sick Children) and Neurometabolic Research Laboratory (Peter Gilgan  Centre for Research and Learning) investigate human fatty acid oxidation (OCTN2, CPTII, SCAD, SCHAD, VLCAD, TFP/LCHAD deficiencies) and mitochondrial disorders (e.g. MELAS and Leigh Syndrome). 

We investigate these disorders from bench to bedside through:

• the characterization of the clinical phenotypes
• physiological measurement of aerobic function (total body aerobic oxidative capacity using maximal graded cycle ergometry in the Physiology Research Unit at SickKids and muscle oxidative capacity using 31P-MRS of exercising muscle in the MR-compatible cycle ergometer, SickKIds MRI centre)
• biochemical phenotyping
• in vitro cell culture systems
• proteomics and molecular biology techniques

In order to elucidate the underlying mechanisms of disease pathophysiology for the development of treatment strategies which are then translated back to the patients and evaluated in prospective, controlled, cross-over treatment trials with clinical, biochemical, neurophysiologic, ergometric and quality of life measures.  

Recent Publications

Clinical and radiologic reversal of stroke-like episodes in MELAS with high-dose L-arginine. Siddiq I, Widjaja E, Tein I. Neurology. 2015 Jul 14;85(2):197-8. doi: 10.1212/WNL.0000000000001726. Epub 2015 Jun 12. No abstract available.
PMID: 26070336 Free PMC Article

L-Arginine Affects Aerobic Capacity and Muscle Metabolism in MELAS (Mitochondrial Encephalomyopathy, Lactic Acidosis and Stroke-Like Episodes) Syndrome. Rodan LH, Wells GD, Banks L, Thompson S, Schneiderman JE, Tein I.
PLoS One. 2015 May 20;10(5):e0127066. doi: 10.1371/journal.pone.0127066. eCollection 2015. PMID: 25993630 Free PMC Article.

Cerebral hyperperfusion and decreased cerebrovascular reactivity correlate with neurologic disease severity in MELAS. Rodan LH, Poublanc J, Fisher JA, Sobczyk O, Wong T, Hlasny E, Mikulis D, Tein I. Mitochondrion. 2015 May;22:66-74. doi: 10.1016/j.mito.2015.03.002. Epub 2015 Mar 21. PMID: 25801712

The mdx mouse as a model for carnitine deficiency in the pathogenesis of Duchenne muscular dystrophy. Zolkipli Z, Mai L, Lamhonwah AM, Tein I. Muscle Nerve. 2012 Nov;46(5):767-72. doi: 10.1002/mus.23368. PMID: 23055315

Abnormal fatty acid metabolism in spinal muscular atrophy may predispose to perioperative risks. Zolkipli Z, Sherlock M, Biggar WD, Taylor G, Hutchison JS, Peliowski A, Alman BA, Ling SC, Tein I. Eur J Paediatr Neurol. 2012. Sep;16(5):549-53. doi: 10.1016/j.ejpn.2012.01.004. Epub 2012 Jan 20. PMID: 22264649

Pyridoxine dependent epilepsy and antiquitin deficiency: clinical and molecular characteristics and recommendations for diagnosis, treatment and follow-up. Stockler S, Plecko B, Gospe SM Jr, Coulter-Mackie M, Connolly M, van Karnebeek C, Mercimek-Mahmutoglu S, Hartmann H, Scharer G, Struijs E, Tein I, Jakobs C, Clayton P, Van Hove JL. Mol Genet Metab. 2011 Sep-Oct;104(1-2):48-60. doi: 10.1016/j.ymgme.2011.05.014. Epub 2011 May 24. Review. PMID: 21704546

Vulnerability to oxidative stress in vitro in pathophysiology of mitochondrial short-chain acyl-CoA dehydrogenase deficiency: response to antioxidants. Zolkipli Z, Pedersen CB, Lamhonwah AM, Gregersen N, Tein I. PLoS One. 2011 Apr 1;6(4):e17534. doi: 10.1371/journal.pone.0017534. PMID: 21483766 Free PMC Article.

Upregulation of mammary gland OCTNs maintains carnitine homeostasis in suckling infants. Lamhonwah AM, Mai L, Chung C, Lamhonwah D, Ackerley C, Tein I. Biochem Biophys Res Commun. 2011 Jan 28;404(4):1010-5. doi: 0.1016/j.bbrc.2010.12.100. Epub 2010 Dec 25. PMID: 21187065

Skeletal muscle metabolism in cystic fibrosis and primary ciliary dyskinesia. Wells GD, Wilkes DL, Schneiderman JE, Rayner T, Elmi M, Selvadurai H, Dell SD, Noseworthy MD, Ratjen F, Tein I, Coates AL. Pediatr Res. 2011 Jan;69(1):40-5. doi: 10.1203/PDR.0b013e3181fff35f. PMID: 20938370

Cystic fibrosis transmembrane conductance regulator in human muscle: Dysfunction causes abnormal metabolic recovery in exercise. Lamhonwah AM, Bear CE, Huan LJ, Kim Chiaw P, Ackerley CA, Tein I. Ann Neurol. 2010 Jun;67(6):802-8. doi: 10.1002/ana.21982. PMID: 20517942

Antioxidant dysfunction: potential risk for neurotoxicity in ethylmalonic aciduria. Pedersen CB, Zolkipli Z, Vang S, Palmfeldt J, Kjeldsen M, Stenbroen V, Schmidt SP, Wanders RJ, Ruiter JP, Wibrand F, Tein I, Gregersen N. J Inherit Metab Dis. 2010 Jun;33(3):211-22. doi: 10.1007/s10545-010-9086-6. Epub 2010 May 5. PMID: 20443061

Organic cation/carnitine transporter family expression patterns in adult murine heart. Lamhonwah AM, Wong J, Tam C, Mai L, Tein I. Pathol Res Pract. 2009;205(6):395-402. doi: 10.1016/j.prp.2008.12.010. Epub 2009 Feb 23. PMID: 19233567

Appointments

Director, Neurometabolic Clinic and Research Laboratory and Staff Neurologist, Division of Neurology
Senior Associate Scientist, Genetics and Genome Biology Program, Research Institute at The Hospital for Sick Children, Toronto