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Knock-out of nexilin in mice leads to dilated cardiomyopathy and endomyocardial fibroelastosis

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Knock-out of nexilin in mice leads to dilated cardiomyopathy and endomyocardial fibroelastosis. / Aherrahrou, Zouhair; Schlossarek, Saskia; Stoelting, Stephanie; Klinger, Matthias; Geertz, Birgit; Weinberger, Florian; Kessler, Thorsten; Aherrahou, Redouane; Moreth, Kristin; Bekeredjian, Raffi; Hrabě de Angelis, Martin; Just, Steffen; Rottbauer, Wolfgang; Eschenhagen, Thomas; Schunkert, Heribert; Carrier, Lucie; Erdmann, Jeanette.

In: BASIC RES CARDIOL, Vol. 111, No. 1, 2016, p. 6-6.

Research output: SCORING: Contribution to journal › SCORING: Journal article › Research › peer-review

Harvard

Aherrahrou, Z, Schlossarek, S, Stoelting, S, Klinger, M, Geertz, B, Weinberger, F, Kessler, T, Aherrahou, R, Moreth, K, Bekeredjian, R, Hrabě de Angelis, M, Just, S, Rottbauer, W, Eschenhagen, T, Schunkert, H, Carrier, L & Erdmann, J 2016, 'Knock-out of nexilin in mice leads to dilated cardiomyopathy and endomyocardial fibroelastosis', BASIC RES CARDIOL, vol. 111, no. 1, pp. 6-6.

APA

Aherrahrou, Z., Schlossarek, S., Stoelting, S., Klinger, M., Geertz, B., Weinberger, F., Kessler, T., Aherrahou, R., Moreth, K., Bekeredjian, R., Hrabě de Angelis, M., Just, S., Rottbauer, W., Eschenhagen, T., Schunkert, H., Carrier, L., & Erdmann, J. (2016). Knock-out of nexilin in mice leads to dilated cardiomyopathy and endomyocardial fibroelastosis. BASIC RES CARDIOL, 111(1), 6-6.

Vancouver

Aherrahrou Z, Schlossarek S, Stoelting S, Klinger M, Geertz B, Weinberger F et al. Knock-out of nexilin in mice leads to dilated cardiomyopathy and endomyocardial fibroelastosis. BASIC RES CARDIOL. 2016;111(1):6-6.

Bibtex

@article{cefae04f56674f6eb1e3fb2757bf6243,
title = "Knock-out of nexilin in mice leads to dilated cardiomyopathy and endomyocardial fibroelastosis",
abstract = "Cardiomyopathy is one of the most commoncauses of chronic heart failure worldwide. Mutations inthe gene encoding nexilin (NEXN) occur in patients withboth hypertrophic and dilated cardiomyopathy (DCM);however, little is known about the pathophysiologicalmechanisms and relevance of NEXN to these disorders.Here, we evaluated the functional role of NEXN using aconstitutive Nexn knock-out (KO) mouse model.Heterozygous (Het) mice were inter-crossed to producewild-type (WT), Het, and homozygous KO mice. At birth,32, 46, and 22 % of the mice were WT, Het, and KO,respectively, which is close to the expected Mendelianratio. After postnatal day 6, the survival of the Nexn KOmice decreased dramatically and all of the animals diedby day 8. Phenotypic characterizations of the WT and KOmice were performed at postnatal days 1, 2, 4, and 6. Atbirth, the relative heart weights of the WT and KO micewere similar; however, at day 4, the relative heart weightof the KO group was 2.3-fold higher than of the WTgroup. In addition, the KO mice developed rapidly progressivecardiomyopathy with left ventricular dilation andwall thinning and decreased cardiac function. At day 6,the KO mice developed a fulminant DCM phenotypecharacterized by dilated ventricular chambers and systolicdysfunction. At this stage, collagen deposits and someelastin deposits were observed within the left ventriclecavity, which resembles the features of endomyocardialfibroelastosis (EFE). Overall, these results furtheremphasize the role of NEXN in DCM and suggest a novelrole in EFE.",
author = "Zouhair Aherrahrou and Saskia Schlossarek and Stephanie Stoelting and Matthias Klinger and Birgit Geertz and Florian Weinberger and Thorsten Kessler and Redouane Aherrahou and Kristin Moreth and Raffi Bekeredjian and {Hrab{\v e} de Angelis}, Martin and Steffen Just and Wolfgang Rottbauer and Thomas Eschenhagen and Heribert Schunkert and Lucie Carrier and Jeanette Erdmann",
year = "2016",
language = "English",
volume = "111",
pages = "6--6",
journal = "BASIC RES CARDIOL",
issn = "0300-8428",
publisher = "D. Steinkopff-Verlag",
number = "1",

}

RIS

TY - JOUR

T1 - Knock-out of nexilin in mice leads to dilated cardiomyopathy and endomyocardial fibroelastosis

AU - Aherrahrou, Zouhair

AU - Schlossarek, Saskia

AU - Stoelting, Stephanie

AU - Klinger, Matthias

AU - Geertz, Birgit

AU - Weinberger, Florian

AU - Kessler, Thorsten

AU - Aherrahou, Redouane

AU - Moreth, Kristin

AU - Bekeredjian, Raffi

AU - Hrabě de Angelis, Martin

AU - Just, Steffen

AU - Rottbauer, Wolfgang

AU - Eschenhagen, Thomas

AU - Schunkert, Heribert

AU - Carrier, Lucie

AU - Erdmann, Jeanette

PY - 2016

Y1 - 2016

N2 - Cardiomyopathy is one of the most commoncauses of chronic heart failure worldwide. Mutations inthe gene encoding nexilin (NEXN) occur in patients withboth hypertrophic and dilated cardiomyopathy (DCM);however, little is known about the pathophysiologicalmechanisms and relevance of NEXN to these disorders.Here, we evaluated the functional role of NEXN using aconstitutive Nexn knock-out (KO) mouse model.Heterozygous (Het) mice were inter-crossed to producewild-type (WT), Het, and homozygous KO mice. At birth,32, 46, and 22 % of the mice were WT, Het, and KO,respectively, which is close to the expected Mendelianratio. After postnatal day 6, the survival of the Nexn KOmice decreased dramatically and all of the animals diedby day 8. Phenotypic characterizations of the WT and KOmice were performed at postnatal days 1, 2, 4, and 6. Atbirth, the relative heart weights of the WT and KO micewere similar; however, at day 4, the relative heart weightof the KO group was 2.3-fold higher than of the WTgroup. In addition, the KO mice developed rapidly progressivecardiomyopathy with left ventricular dilation andwall thinning and decreased cardiac function. At day 6,the KO mice developed a fulminant DCM phenotypecharacterized by dilated ventricular chambers and systolicdysfunction. At this stage, collagen deposits and someelastin deposits were observed within the left ventriclecavity, which resembles the features of endomyocardialfibroelastosis (EFE). Overall, these results furtheremphasize the role of NEXN in DCM and suggest a novelrole in EFE.

AB - Cardiomyopathy is one of the most commoncauses of chronic heart failure worldwide. Mutations inthe gene encoding nexilin (NEXN) occur in patients withboth hypertrophic and dilated cardiomyopathy (DCM);however, little is known about the pathophysiologicalmechanisms and relevance of NEXN to these disorders.Here, we evaluated the functional role of NEXN using aconstitutive Nexn knock-out (KO) mouse model.Heterozygous (Het) mice were inter-crossed to producewild-type (WT), Het, and homozygous KO mice. At birth,32, 46, and 22 % of the mice were WT, Het, and KO,respectively, which is close to the expected Mendelianratio. After postnatal day 6, the survival of the Nexn KOmice decreased dramatically and all of the animals diedby day 8. Phenotypic characterizations of the WT and KOmice were performed at postnatal days 1, 2, 4, and 6. Atbirth, the relative heart weights of the WT and KO micewere similar; however, at day 4, the relative heart weightof the KO group was 2.3-fold higher than of the WTgroup. In addition, the KO mice developed rapidly progressivecardiomyopathy with left ventricular dilation andwall thinning and decreased cardiac function. At day 6,the KO mice developed a fulminant DCM phenotypecharacterized by dilated ventricular chambers and systolicdysfunction. At this stage, collagen deposits and someelastin deposits were observed within the left ventriclecavity, which resembles the features of endomyocardialfibroelastosis (EFE). Overall, these results furtheremphasize the role of NEXN in DCM and suggest a novelrole in EFE.

M3 - SCORING: Journal article

VL - 111

SP - 6

EP - 6

JO - BASIC RES CARDIOL

JF - BASIC RES CARDIOL

SN - 0300-8428

IS - 1

ER -