eISSN: 1509-572x
ISSN: 1641-4640
Folia Neuropathologica
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SCImago Journal & Country Rank
3/2018
vol. 56
 
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abstract:
Original paper

The level of microRNA 21 is upregulated by rapamycin in serum of tuberous sclerosis complex patients and subependymal giant cell astrocytoma (SEGA)-derived cell cultures

Bozena Kuzniewska, Krzysztof Sadowski, Katarzyna Urbanska, Malgorzata Urbanska, Katarzyna Kotulska, Ewa Liszewska, Wieslawa Grajkowska, Sergiusz Jóźwiak, Magdalena Dziembowska

Folia Neuropathol 2018; 56 (3): 167-174
Online publish date: 2018/09/28
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Tuberous sclerosis complex (TSC) represents a genetic condition, in which the clinical manifestations are caused by the disinhibition of the mammalian target of rapamycin (mTOR) pathway due to mutations in the TSC1 (hamartin) or TSC2 (tuberin) genes. The deregulated mTOR activity leads to multi-site tumors, including subependymal giant cell astrocytoma (SEGA). SEGA is a brain tumor that affects around 15% of TSC patients. The aim of the study was to evaluate miR-21 expression in the serum of two groups of TSC patients: with or without SEGA tumors. We found no differences in the level of miR-21 depending on the presence of SEGA. Next, we studied the influence of prolonged rapamycin administration on miR-21 level in the blood serum of TSC patients (6-12 months of rapamycin) and in primary cultures of SEGA-derived cells treated with rapamycin in vitro. Here we show that rapamycin treatment leads to the upregulation of miR-21 in both patients’ serum and in primary SEGA tumor cells in the culture indicating the regulatory relationship between rapamycin treatment and miR-21 expression.
keywords:

tuberous sclerosis, SEGA, miR-21, rapamycin

references:
Bissler JJ, McCormack FX, Young LR, Elwing JM, Chuck G, Leonard JM, Schmithorst VJ, Laor T, Brody AS, Bean J, Salisbury S, Franz DN. Sirolimus for angiomyolipoma in tuberous sclerosis complex or lymphangioleiomyomatosis. N Engl J Med 2008; 358: 140-151.
Franz DN, Belousova E, Sparagana S, Bebin EM, Frost MD, Kuperman R, Witt O, Kohrman MH, Flamini JR, Wu JY, Curatolo P, de Vries PJ, Berkowitz N, Niolat J, Jozwiak S. Long-term use of everolimus in patients with tuberous sclerosis complex: final results from the EXIST-1 study. PLoS One 2016; 11: e0158476.
Franz DN, Leonard J, Tudor C, Chuck G, Care M, Sethuraman G, Dinopoulos A, Thomas G, Crone KR. Rapamycin causes regression of astrocytomas in tuberous sclerosis complex. Ann Neurol 2006; 59: 490-498.
Henske EP, Jozwiak S, Kingswood JC, Sampson JR, Thiele EA. Tuberous sclerosis complex. Nat Rev Dis Primers 2016; 2: 16035.
Jozwiak S, Mandera M, Mlynarski W. Natural history and current treatment options for subependymal giant cell astrocytoma in tuberous sclerosis complex. Semin Pediatr Neurol 2015; 22: 274-281.
Jozwiak S, Nabbout R, Curatolo P, participants of the TSC Consensus Meeting for SEGA and Epilepsy Management. Management of subependymal giant cell astrocytoma (SEGA) associated with tuberous sclerosis complex (TSC): Clinical recommendations. Eur J Paediatr Neurol 2013; 17: 348-352.
Kotulska K, Borkowska J, Roszkowski M, Mandera M, Daszkiewicz P, Drabik K, Jurkiewicz E, Larysz-Brysz M, Nowak K, Grajkowska W, Domanska-Pakiela D, Jozwiak S. Surgical treatment of subependymal giant cell astrocytoma in tuberous sclerosis complex patients. Pediatr Neurol 2014; 50: 307-312.
Kumarswamy R, Volkmann I, Thum T. Regulation and function of miRNA-21 in health and disease. RNA Biol 2011; 8: 706-713.
Lam HC, Liu HJ, Baglini CV, Filippakis H, Alesi N, Nijmeh J, Du H, Lope AL, Cottrill KA, Handen A, Asara JM, Kwiatkowski DJ, Ben-Sahra I, Oldham WM, Chan SY, Henske EP. Rapamycin-induced miR-21 promotes mitochondrial homeostasis and adaptation in mTORC1 activated cells. Oncotarget 2017; 8: 64714-64727.
Li J, Kim SG, Blenis J. Rapamycin: one drug, many effects. Cell Metab 2014; 19: 373-379.
Northrup H, Krueger DA, International Tuberous Sclerosis Complex Consensus. Tuberous sclerosis complex diagnostic criteria update: recommendations of the 2012 Iinternational Tuberous Sclerosis Complex Consensus Conference. Pediatr Neurol 2013; 49: 243-254.
Roth J, Roach ES, Bartels U, Jozwiak S, Koenig MK, Weiner HL, Franz DN, Wang HZ. Subependymal giant cell astrocytoma: diagnosis, screening, and treatment. Recommendations from the International Tuberous Sclerosis Complex Consensus Conference 2012. Pediatr Neurol 2013; 49: 439-444.
Sadowski K, Kotulska K, Jozwiak S. Management of side effects of mTOR inhibitors in tuberous sclerosis patients. Pharmacol Rep 2016; 68: 536-542.
Sadowski K, Kotulska K, Schwartz RA, Jozwiak S. Systemic effects of treatment with mTOR inhibitors in tuberous sclerosis complex: a comprehensive review. J Eur Acad Dermatol Venereol 2016; 30: 586-594.
Trelinska J, Dachowska I, Baranska D, Stawiski K, Kotulska K, Fendler W, Jozwiak S, Mlynarski W. Maintenance therapy with everolimus for subependymal giant cell astrocytoma in patients with tuberous sclerosis (the EMINENTS study). Pediatr Blood Cancer 2017; 64.
Trelinska J, Dachowska I, Kotulska K, Baranska D, Fendler W, Jozwiak S, Mlynarski W. Factors affecting response to everolimus therapy for subependymal giant cell astrocytomas associated with tuberous sclerosis. Pediatr Blood Cancer 2015; 62: 616-621.
Trindade AJ, Medvetz DA, Neuman NA, Myachina F, Yu J, Priolo C, Henske EP. MicroRNA-21 is induced by rapamycin in a model of tuberous sclerosis (TSC) and lymphangioleiomyomatosis (LAM). PLoS One 2013; 8: e60014.
van Scheppingen J, Iyer AM, Prabowo AS, Muhlebner A, Anink JJ, Scholl T, Feucht M, Jansen FE, Spliet WG, Krsek P, Zamecnik J, Buccoliero AM, Giordano F, Genitori L, Kotulska K, Jozwiak S, Jaworski J, Liszewska E, van Vliet EA, Aronica E. Expression of microRNAs miR21, miR146a, and miR155 in tuberous sclerosis complex cortical tubers and their regulation in human astrocytes and SEGA-derived cell cultures. Glia 2016; 64: 1066-1082.
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