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Total serum transforming growth factor-β1 is elevated in the entire spectrum of genetic aortic syndromes

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Total serum transforming growth factor-β1 is elevated in the entire spectrum of genetic aortic syndromes. / Hillebrand, Mathias; Millot, Nathalie; Sheikhzadeh, Sara; Rybczynski, Meike; Gerth, Sabine; Kölbel, Tilo; Keyser, Britta; Kutsche, Kerstin; Robinson, Peter N; Berger, Juergen; Mir, Thomas S; Zeller, Tanja; Blankenberg, Stefan; Kodolitsch, Yskert; Goldmann, Britta.

in: CLIN CARDIOL, Jahrgang 37, Nr. 11, 01.11.2014, S. 672-679.

Publikationen: SCORING: Beitrag in Fachzeitschrift/Zeitung › SCORING: Zeitschriftenaufsatz › Forschung › Begutachtung

Harvard

Hillebrand, M, Millot, N, Sheikhzadeh, S, Rybczynski, M, Gerth, S, Kölbel, T, Keyser, B, Kutsche, K, Robinson, PN, Berger, J, Mir, TS, Zeller, T, Blankenberg, S, Kodolitsch, Y & Goldmann, B 2014, 'Total serum transforming growth factor-β1 is elevated in the entire spectrum of genetic aortic syndromes', CLIN CARDIOL, Jg. 37, Nr. 11, S. 672-679. https://doi.org/10.1002/clc.22320

APA

Hillebrand, M., Millot, N., Sheikhzadeh, S., Rybczynski, M., Gerth, S., Kölbel, T., Keyser, B., Kutsche, K., Robinson, P. N., Berger, J., Mir, T. S., Zeller, T., Blankenberg, S., Kodolitsch, Y., & Goldmann, B. (2014). Total serum transforming growth factor-β1 is elevated in the entire spectrum of genetic aortic syndromes. CLIN CARDIOL, 37(11), 672-679. https://doi.org/10.1002/clc.22320

Vancouver

Hillebrand M, Millot N, Sheikhzadeh S, Rybczynski M, Gerth S, Kölbel T et al. Total serum transforming growth factor-β1 is elevated in the entire spectrum of genetic aortic syndromes. CLIN CARDIOL. 2014 Nov 1;37(11):672-679. https://doi.org/10.1002/clc.22320

Bibtex

@article{4bb297e757ec4dbcae6357252874bfae,
title = "Total serum transforming growth factor-β1 is elevated in the entire spectrum of genetic aortic syndromes",
abstract = "BACKGROUND: Total serum transforming growth factor-beta 1 (tsTGF-β1) is increased in patients with Marfan syndrome (MFS), but it has not been assessed in thoracic aortic aneurysm and dissection (TAAD), Loeys-Dietz syndrome (LDS), and bicuspid aortic valve disease (BAVD).HYPOTHESIS: tsTGF-β1 is increased in genetic aortic syndromes including TAAD, LDS, MFS, and BAVD.METHODS: We measured tsTGF-β1 and performed sequencing of the genes FBN1, TGFBR1, and TGFBR2 in 317 consecutive patients with suspected or known genetic aortic syndrome (167 men, 150 women; mean age 43 ± 14 years). TAAD was diagnosed in 20, LDS in 20, MFS in 128, and BAVD in 30 patients, and genetic aortic syndrome was excluded in 119 patients.RESULTS: Elevated tsTGF-β1 levels were associated with causative gene mutations (P = 0.008), genetic aortic syndrome (P = 0.009), and sporadic occurrence of genetic aortic syndrome (P = 0.048), whereas only genetic aortic syndrome qualified as an independent predictor of tsTGF-β1 (P = 0.001). The tsTGF-β1 levels were elevated in FBN1 and NOTCH1 mutations vs patients without mutations (both P = 0.004), and in NOTCH1 mutations vs ACTA2/MYH11 mutations (P = 0.015). Similarly, tsTGF-β1 levels were elevated in MFS (P = 0.003) and in BAVD (P = 0.006) vs patients without genetic aortic syndrome. In contrast to specific clinical features of MFS, FBN1 in-frame mutations (P = 0.019) were associated with increased tsTGF-β1 levels.CONCLUSIONS: tsTGF-β1 is elevated in the entire spectrum of genetic aortic syndromes. However, gradual differences in the increases of tsTGF-β1 levels may mirror different degrees of alteration of tsTGF-β1 signaling in different genetic aortic syndromes.",
author = "Mathias Hillebrand and Nathalie Millot and Sara Sheikhzadeh and Meike Rybczynski and Sabine Gerth and Tilo K{\"o}lbel and Britta Keyser and Kerstin Kutsche and Robinson, {Peter N} and Juergen Berger and Mir, {Thomas S} and Tanja Zeller and Stefan Blankenberg and Yskert Kodolitsch and Britta Goldmann",
note = "{\textcopyright} 2014 Wiley Periodicals, Inc.",
year = "2014",
month = nov,
day = "1",
doi = "10.1002/clc.22320",
language = "English",
volume = "37",
pages = "672--679",
journal = "CLIN CARDIOL",
issn = "0160-9289",
publisher = "John Wiley and Sons Inc.",
number = "11",

}

RIS

TY - JOUR

T1 - Total serum transforming growth factor-β1 is elevated in the entire spectrum of genetic aortic syndromes

AU - Hillebrand, Mathias

AU - Millot, Nathalie

AU - Sheikhzadeh, Sara

AU - Rybczynski, Meike

AU - Gerth, Sabine

AU - Kölbel, Tilo

AU - Keyser, Britta

AU - Kutsche, Kerstin

AU - Robinson, Peter N

AU - Berger, Juergen

AU - Mir, Thomas S

AU - Zeller, Tanja

AU - Blankenberg, Stefan

AU - Kodolitsch, Yskert

AU - Goldmann, Britta

N1 - © 2014 Wiley Periodicals, Inc.

PY - 2014/11/1

Y1 - 2014/11/1

N2 - BACKGROUND: Total serum transforming growth factor-beta 1 (tsTGF-β1) is increased in patients with Marfan syndrome (MFS), but it has not been assessed in thoracic aortic aneurysm and dissection (TAAD), Loeys-Dietz syndrome (LDS), and bicuspid aortic valve disease (BAVD).HYPOTHESIS: tsTGF-β1 is increased in genetic aortic syndromes including TAAD, LDS, MFS, and BAVD.METHODS: We measured tsTGF-β1 and performed sequencing of the genes FBN1, TGFBR1, and TGFBR2 in 317 consecutive patients with suspected or known genetic aortic syndrome (167 men, 150 women; mean age 43 ± 14 years). TAAD was diagnosed in 20, LDS in 20, MFS in 128, and BAVD in 30 patients, and genetic aortic syndrome was excluded in 119 patients.RESULTS: Elevated tsTGF-β1 levels were associated with causative gene mutations (P = 0.008), genetic aortic syndrome (P = 0.009), and sporadic occurrence of genetic aortic syndrome (P = 0.048), whereas only genetic aortic syndrome qualified as an independent predictor of tsTGF-β1 (P = 0.001). The tsTGF-β1 levels were elevated in FBN1 and NOTCH1 mutations vs patients without mutations (both P = 0.004), and in NOTCH1 mutations vs ACTA2/MYH11 mutations (P = 0.015). Similarly, tsTGF-β1 levels were elevated in MFS (P = 0.003) and in BAVD (P = 0.006) vs patients without genetic aortic syndrome. In contrast to specific clinical features of MFS, FBN1 in-frame mutations (P = 0.019) were associated with increased tsTGF-β1 levels.CONCLUSIONS: tsTGF-β1 is elevated in the entire spectrum of genetic aortic syndromes. However, gradual differences in the increases of tsTGF-β1 levels may mirror different degrees of alteration of tsTGF-β1 signaling in different genetic aortic syndromes.

AB - BACKGROUND: Total serum transforming growth factor-beta 1 (tsTGF-β1) is increased in patients with Marfan syndrome (MFS), but it has not been assessed in thoracic aortic aneurysm and dissection (TAAD), Loeys-Dietz syndrome (LDS), and bicuspid aortic valve disease (BAVD).HYPOTHESIS: tsTGF-β1 is increased in genetic aortic syndromes including TAAD, LDS, MFS, and BAVD.METHODS: We measured tsTGF-β1 and performed sequencing of the genes FBN1, TGFBR1, and TGFBR2 in 317 consecutive patients with suspected or known genetic aortic syndrome (167 men, 150 women; mean age 43 ± 14 years). TAAD was diagnosed in 20, LDS in 20, MFS in 128, and BAVD in 30 patients, and genetic aortic syndrome was excluded in 119 patients.RESULTS: Elevated tsTGF-β1 levels were associated with causative gene mutations (P = 0.008), genetic aortic syndrome (P = 0.009), and sporadic occurrence of genetic aortic syndrome (P = 0.048), whereas only genetic aortic syndrome qualified as an independent predictor of tsTGF-β1 (P = 0.001). The tsTGF-β1 levels were elevated in FBN1 and NOTCH1 mutations vs patients without mutations (both P = 0.004), and in NOTCH1 mutations vs ACTA2/MYH11 mutations (P = 0.015). Similarly, tsTGF-β1 levels were elevated in MFS (P = 0.003) and in BAVD (P = 0.006) vs patients without genetic aortic syndrome. In contrast to specific clinical features of MFS, FBN1 in-frame mutations (P = 0.019) were associated with increased tsTGF-β1 levels.CONCLUSIONS: tsTGF-β1 is elevated in the entire spectrum of genetic aortic syndromes. However, gradual differences in the increases of tsTGF-β1 levels may mirror different degrees of alteration of tsTGF-β1 signaling in different genetic aortic syndromes.

U2 - 10.1002/clc.22320

DO - 10.1002/clc.22320

M3 - SCORING: Journal article

C2 - 25113270

VL - 37

SP - 672

EP - 679

JO - CLIN CARDIOL

JF - CLIN CARDIOL

SN - 0160-9289

IS - 11

ER -