Cover Image

Orai1 deficiency leads to heart failure and skeletal myopathy in zebrafish

Mutations in the store-operated Ca2+ entry pore protein ORAI1 have been reported to cause myopathies in human patients but the mechanism involved is not known. Cardiomyocytes express ORAI1 but its role in heart function is also unknown. Using reverse genetics in zebrafish, we demonstrated that inact...

Full description

Saved in:
Bibliographic Details
Main Authors: Völkers, Mirko (Author) , Dolatabadi, Nima (Author) , Gude, Natalie (Author) , Most, Patrick (Author) , Sussman, Mark A. (Author) , Hassel, David (Author)
Format: Article (Journal)
Language:English
Published: [2012]
In: Journal of cell science
Year: 2012, Volume: 125, Issue: 2, Pages: 287-294
ISSN:1477-9137
DOI:10.1242/jcs.090464
Online Access:Verlag, kostenfrei, Volltext: http://dx.doi.org/10.1242/jcs.090464
Verlag, kostenfrei, Volltext: http://jcs.biologists.org/content/125/2/287
Get full text
Author Notes:Mirko Völkers, Nima Dolatabadi, Natalie Gude, Patrick Most, Mark A. Sussman and David Hassel

MARC

LEADER 00000caa a2200000 c 4500
001 1581681194
003 DE-627
005 20241107161921.0
007 cr uuu---uuuuu
008 181009s2012 xx |||||o 00| ||eng c
024 7 |a 10.1242/jcs.090464  |2 doi 
035 |a (DE-627)1581681194 
035 |a (DE-576)511681194 
035 |a (DE-599)BSZ511681194 
035 |a (OCoLC)1341019680 
040 |a DE-627  |b ger  |c DE-627  |e rda 
041 |a eng 
084 |a 33  |2 sdnb 
100 1 |a Völkers, Mirko  |d 1978-  |e VerfasserIn  |0 (DE-588)133026442  |0 (DE-627)530939932  |0 (DE-576)299573753  |4 aut 
245 1 0 |a Orai1 deficiency leads to heart failure and skeletal myopathy in zebrafish  |c Mirko Völkers, Nima Dolatabadi, Natalie Gude, Patrick Most, Mark A. Sussman and David Hassel 
264 1 |c [2012] 
300 |a 8 
336 |a Text  |b txt  |2 rdacontent 
337 |a Computermedien  |b c  |2 rdamedia 
338 |a Online-Ressource  |b cr  |2 rdacarrier 
500 |a Gesehen am 09.10.2018 
520 |a Mutations in the store-operated Ca2+ entry pore protein ORAI1 have been reported to cause myopathies in human patients but the mechanism involved is not known. Cardiomyocytes express ORAI1 but its role in heart function is also unknown. Using reverse genetics in zebrafish, we demonstrated that inactivation of the highly conserved zebrafish orthologue of ORAI1 resulted in severe heart failure, reduced ventricular systolic function, bradycardia and skeletal muscle weakness. Electron microscopy of Orai1-deficient myocytes revealed progressive skeletal muscle instability with loss of myofiber integrity and ultrastructural abnormalities of the z-disc in both skeletal and cardiac muscle. Isolated Orai1-deficient cardiomyocytes showed loss of the calcineurin-associated protein calsarcin from the z-discs. Furthermore, we found mechanosignal transduction was affected in Orai1-depleted hearts, indicating an essential role for ORAI1 in establishing the cardiac signaling transduction machinery at the z-disc. Our findings identify ORAI1 as an important regulator of cardiac and skeletal muscle function and provide evidence linking ORAI1-mediated calcium signaling to sarcomere integrity and cardiomyocyte function. 
700 1 |a Dolatabadi, Nima  |e VerfasserIn  |4 aut 
700 1 |a Gude, Natalie  |e VerfasserIn  |4 aut 
700 1 |a Most, Patrick  |d 1969-  |e VerfasserIn  |0 (DE-588)124606431  |0 (DE-627)363440267  |0 (DE-576)29440919X  |4 aut 
700 1 |a Sussman, Mark A.  |e VerfasserIn  |4 aut 
700 1 |a Hassel, David  |e VerfasserIn  |0 (DE-588)135801613  |0 (DE-627)693785608  |0 (DE-576)300653808  |4 aut 
773 0 8 |i Enthalten in  |t Journal of cell science  |d Cambridge : Company of Biologists Limited, 1853  |g 125(2012), 2, Seite 287-294  |h Online-Ressource  |w (DE-627)300595433  |w (DE-600)1483099-1  |w (DE-576)090854837  |x 1477-9137  |7 nnas  |a Orai1 deficiency leads to heart failure and skeletal myopathy in zebrafish 
773 1 8 |g volume:125  |g year:2012  |g number:2  |g pages:287-294  |g extent:8  |a Orai1 deficiency leads to heart failure and skeletal myopathy in zebrafish 
856 4 0 |u http://dx.doi.org/10.1242/jcs.090464  |x Verlag  |x Resolving-System  |z kostenfrei  |3 Volltext 
856 4 0 |u http://jcs.biologists.org/content/125/2/287  |x Verlag  |z kostenfrei  |3 Volltext 
951 |a AR 
992 |a 20181009 
993 |a Article 
994 |a 2012 
998 |g 135801613  |a Hassel, David  |m 135801613:Hassel, David  |d 910000  |d 910100  |e 910000PH135801613  |e 910100PH135801613  |k 0/910000/  |k 1/910000/910100/  |p 6  |y j 
998 |g 124606431  |a Most, Patrick  |m 124606431:Most, Patrick  |d 910000  |d 910100  |e 910000PM124606431  |e 910100PM124606431  |k 0/910000/  |k 1/910000/910100/  |p 4 
998 |g 133026442  |a Völkers, Mirko  |m 133026442:Völkers, Mirko  |d 910000  |d 910100  |e 910000PV133026442  |e 910100PV133026442  |k 0/910000/  |k 1/910000/910100/  |p 1  |x j 
999 |a KXP-PPN1581681194  |e 3028239205 
BIB |a Y 
SER |a journal 
JSO |a {"id":{"doi":["10.1242/jcs.090464"],"eki":["1581681194"]},"name":{"displayForm":["Mirko Völkers, Nima Dolatabadi, Natalie Gude, Patrick Most, Mark A. Sussman and David Hassel"]},"type":{"bibl":"article-journal","media":"Online-Ressource"},"language":["eng"],"note":["Gesehen am 09.10.2018"],"title":[{"title":"Orai1 deficiency leads to heart failure and skeletal myopathy in zebrafish","title_sort":"Orai1 deficiency leads to heart failure and skeletal myopathy in zebrafish"}],"origin":[{"dateIssuedDisp":"[2012]","dateIssuedKey":"2012"}],"person":[{"display":"Völkers, Mirko","family":"Völkers","given":"Mirko","role":"aut"},{"family":"Dolatabadi","role":"aut","given":"Nima","display":"Dolatabadi, Nima"},{"display":"Gude, Natalie","family":"Gude","given":"Natalie","role":"aut"},{"role":"aut","given":"Patrick","family":"Most","display":"Most, Patrick"},{"display":"Sussman, Mark A.","family":"Sussman","given":"Mark A.","role":"aut"},{"display":"Hassel, David","role":"aut","given":"David","family":"Hassel"}],"relHost":[{"physDesc":[{"extent":"Online-Ressource"}],"recId":"300595433","disp":"Orai1 deficiency leads to heart failure and skeletal myopathy in zebrafishJournal of cell science","origin":[{"dateIssuedDisp":"1853-","dateIssuedKey":"1853","publisherPlace":"Cambridge","publisher":"Company of Biologists Limited"}],"note":["Gesehen am 22.04.2021"],"type":{"bibl":"periodical","media":"Online-Ressource"},"language":["eng"],"title":[{"subtitle":"Company of Biologists","title_sort":"Journal of cell science","title":"Journal of cell science"}],"corporate":[{"role":"isb","display":"The Company of Biologists"}],"titleAlt":[{"title":"Quarterly journal of microscopical science"}],"pubHistory":["1.1853 - 8.1860; N.S. 1.1861 - 87.1946; 3.Ser. 88.1947 - 106.1965; 1.1966 -"],"part":{"issue":"2","extent":"8","text":"125(2012), 2, Seite 287-294","pages":"287-294","volume":"125","year":"2012"},"id":{"zdb":["1483099-1"],"issn":["1477-9137"],"eki":["300595433"]}}],"physDesc":[{"extent":"8 S."}],"recId":"1581681194"} 
SRT |a VOELKERSMIORAI1DEFIC2012 

Similar Items