Nikos Protonotarios

Greek researcher and cardiologist

Dr. Nikos Protonotarios in 2012

Nikos Protonotarios (Naxos, 1 October 1956 – Athens, 25 September 2014) was a Greek researcher and cardiologist who made fundamental contributions to the field of arrhythmogenic myocardial diseases.[1][2]

## Biography[edit]

He was born in Apeiranthos, Naxos, Greece. In 1981, he graduated at the top of his class from the Medical School of the National and Kapodistrian University of Athens. In 1983, he returned to his hometown, Naxos, where he fulfilled his Rural Medical Service.[3]

During this period, along with his wife, Dr. Antigoni-Eleni Tsatsopoulou, he identified and studied the first cases of arrhythmogenic myocardial disease,[4] which he later made known to the global medical community as "Naxos Disease" through his historic work in the international cardiology journal, British Heart Journal, in 1986.[5]

## Contribution to cardiology[edit]

Protonotarios identified first and described the myocardial disease "Naxos Disease", as well as the gene responsible for it.[6][7] His work paved the way for the application of genetic science in the treatment of myocardial disease, marking the first time in the history of clinical cardiology that genetic science was applied to address a myocardial disease. His research on "Naxos Disease" not only led to the recognition of plakoglobin as a pathogenic gene for arrhythmogenic right ventricular cardiomyopathy (ARVC/D) but also opened the path for the discovery of other desmosomal genes related to the pathogenesis of the disease.[8][9]

His work on plakoglobin formed the basis for the development of an innovative diagnostic test for identifying patients with ARVC/D[10] , and later, it laid the groundwork for the discovery of a completely new drug, SB216763, which, in cellular models and genetically mutated experimental animals, eliminates all abnormalities associated with the disease.[11]

## References[edit]

1. ^ Li, Guo-Liang; Saguner, Ardan M.; Fontaine, Guy H. (2018-05-10). "Naxos disease: from the origin to today". Orphanet Journal of Rare Diseases. 13 (1): 74. doi:10.1186/s13023-018-0814-6. PMC 5946438. PMID 29747658.
2. ^ https://www.protothema.gr/greece/article/413453/efuge-o-kardiologos-ereunitis-nikos-protonotarios/ «Έφυγε» ο καρδιολόγος-ερευνητής Νίκος Πρωτονοτάριος - Ήταν ο Έλληνας επιστήμονας που ανακάλυψε το γονίδιο που προκαλεί αιφνίδιο θάνατο σε νέους και αθλητές - Πώς εντόπισε την αιτία της καρδιοπάθειας
3. ^ "Διεθνής βράβευση ιατρών από τη Νάξο!". Κυκλάδες Voice (in Greek). 2023-10-27. Retrieved 2023-11-01.
4. ^ "ΤΟ ΒΗΜΑ". ΤΟ ΒΗΜΑ (in Greek). 2008-11-24. Retrieved 2023-11-01.
5. ^ Protonotarios, N.; Tsatsopoulou, A.; Patsourakos, P.; Alexopoulos, D.; Gezerlis, P.; Simitsis, S.; Scampardonis, G. (1986). "Cardiac abnormalities in familial palmoplantar keratosis". British Heart Journal. 56 (4): 321–326. doi:10.1136/hrt.56.4.321. PMC 1236865. PMID 2945574.
6. ^ Protonotarios N, Tsatsopoulou A, Patsourakos P, Alexopoulos D, Gezerlis P, Simitsis S, Scampardonis G (1986). "Cardiac abnormalities in familial palmoplantar keratosis". Heart. 56 (4): 321–326. doi:10.1136/hrt.56.4.321. PMC 1236865. PMID 2945574.`{{cite journal}}`: CS1 maint: multiple names: authors list (link)
7. ^ Fontaine G, Protonotarios N, Tsatsopoulou A, Tsezana R, Fontaliran F, Frank R (1994). "Comparisons between Naxos disease and arrhythmogenic right ventricular dysplasia by electrocardiography and biopsy". Circulation. 90: 3233.`{{cite journal}}`: CS1 maint: multiple names: authors list (link)
8. ^ Coonar AS, Protonotarios N, Tsatsopoulou A; et al. (1998). "Gene for Arrhythmogenic Right Ventricular Cardiomyopathy With Diffuse Nonepidermolytic Palmoplantar Keratoderma and Woolly Hair (Naxos Disease) Maps to 17q21". Circulation. 97 (20): 2049–2058. doi:10.1161/01.CIR.97.20.2049. PMID 9610536.`{{cite journal}}`: CS1 maint: multiple names: authors list (link)
9. ^ McKoy G., Protonotarios N, Crosby A, Tsatsopoulou A, Anastasakis A, Coonar A, Norman M, Baboonian C, Jeffery S, McKenna WJ (2000). "Identification of a deletion in plakoglobin in arrhythmogenic right ventricular cardiomyopathy with palmoplantar keratoderma and woolly hair (Naxos disease)". The Lancet. 355 (9221): 2119–2124. doi:10.1016/S0140-6736(00)02379-5. PMID 10902626. S2CID 39821701.`{{cite journal}}`: CS1 maint: multiple names: authors list (link)
10. ^ Asimaki A, Tandri H, Huang H, Halushka MK, Gautam S, Basso C, Thiene G, Tsatsopoulou A, Protonotarios N, McKenna WJ, Calkins H, Saffitz JE (2009). "A New Diagnostic Test for Arrhythmogenic Right Ventricular Cardiomyopathy". New England Journal of Medicine. 360 (11): 1075–1084. doi:10.1056/NEJMoa0808138. PMID 19279339.`{{cite journal}}`: CS1 maint: multiple names: authors list (link)
11. ^ Asimaki A, Kapoor S, Plovie E, Arndt AK, Adams E, Liu Z, James CA, Judge DP, Calkins H, Churko J, Wu JC, MacRae CA, Kléber AG, Saffitz JE (2014). "Identification of a New Modulator of the Intercalated Disc in a Zebrafish Model of Arrhythmogenic Cardiomyopathy". Science Translational Medicine. 6 (240): 240ra74. doi:10.1126/scitranslmed.3008008. PMC 4471875. PMID 24920660.`{{cite journal}}`: CS1 maint: multiple names: authors list (link)