Table 1.

Immunohistochemistry reagents

Primary antibody	Vendor, order number	Concentration	Secondary antibody	Mechanism
Anti-αSMA	A2547, Sigma-Aldrich Chemie, Darmstadt, Germany	1:5000	RAM-PO (1:250) (DAKO p0260)	Smooth muscle cell differentiation
Anti-cleaved-caspase-3	9661, Cell Signaling, Beverly, USA	1:250	GAR (1:200) and NGS (1:66) (Vector Laboratories, USA, BA-1000 and S1000)	Apoptosis
Anti-SM22α	AB10135, Abcam, Cambridge, UK	1:100	GAR and NGS	Smooth muscle cell differentiation
Anti-smoothelin	16101, ProgenBiotechnik, Heidelberg, Germany	1:200	HAM (1:200) and NHS (1:66) (Vector Laboratories, USA, BA-2000) (Brunschwig Chemie, Switzerland, S-2000)	Smooth muscle cell differentiation
Anti-lamin A/C	MAB3211, Millipore, Billerica, USA	1:200	HAM and NHS	Myoblast differentiation
Anti-progerin	Kindly provided by K. Djabali (Department of Dermatology, Colombia University, NY, USA)	1:50	GAR and NGS	Cardiovascular aging
Anti-eNOS	PA1037, Thermo Scientific, Rockford, USA	1:100	GAR and NGS	Susceptibility for aortopathy in BAV
Anti-TGFβ	MO-C40009E, Anogen, ON, Canada	1:1000	HAM and NHS	Susceptibility for aortopathy in BAV
Anti-MMP9	MCA2736, ThermoFisher, Waltham, USA	1:100	HAM and NHS	Susceptibility for aortopathy in BAV
Anti-c-Kit	A4502, Dako, Heverlee, Belgium	1:00	GAR and NGS	Susceptibility for aortopathy in BAV
Anti-pc-Kit	ab62154, Abcam, Cambridge, UK	1:100	GAR and NGS	Susceptibility for aortopathy in BAV
Anti-HIF1α	SC-53546, Santa Cruz Biotechnology, TX, USA	1:500	HAM	Susceptibility for aortopathy in BAV
Anti-FBN1	MAB1919, Millipore, Billerica, Germany	1:100	HAM and NHS	Fibrillin-1 expression

αSMA alpha-smooth muscle actin, SM22α smooth muscle-22-alpha, eNOS endothelial nitric oxide, TGFβ transforming growth factor-beta, MMP9 matrix metalloproteinase-9, pc-Kit phosphorylated c-Kit, HIF1α hypoxia-inducible-factor-1-alpha, FBN1 fibrillin-1, GAR goat-anti-rabbit-biotin, NGS normal goat serum, HAM horse-anti-mouse-biotin, NHS normal horse serum, RAM-PO peroxidase-conjugated rabbit anti-mouse