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Cardiovascular Calcification in Hutchinson-Gilford Progeria and Correlation with Age-Related Degenerative Calcification

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Cardiovascular Calcification and Bone Mineralization

Part of the book series: Contemporary Cardiology ((CONCARD))

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Abstract

Hutchinson-Gilford progeria syndrome (HGPS) and other progeroid (premature aging) syndromes are attributable to lamin A processing defects; these result in aberrant nuclear architectures that impact normal transcription and translation, resulting in a premature aging phenotype at the cellular, tissue, and organismal levels. Patients with HGPS are characterized by severe accelerated cardiovascular disease, including valvular calcification and vessel wall stiffening and calcification as well as atherosclerosis, typically culminating in premature death due to myocardial infarction or stroke. Understanding the mechanisms of the early and aggressive vascular and valvular calcification (and vascular atherosclerosis) in the progeroid syndromes may cast important light on the pathways (and therapies) relevant for physiologic aging and senescence.

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Abbreviations

EC:

Endothelial cells

eNOS:

Endothelial nitric oxide synthase

eNTPD1:

Ectonucleoside triphosphate diphosphohydrolase-1

FACE-1:

Farnesylated protein converting enzyme-1

HGPS:

Hutchinson-Gilford progeria syndrome

KLF2:

Krüppel-like factor 2

PPi:

Inorganic pyrophosphate

ROS:

Reactive oxygen species

TNAP:

Tissue non-specific alkaline phosphatase

VSMC:

Vascular smooth muscle cells

ZMPSTE24:

Zinc metalloproteinase STE24

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Acknowledgments

The author wishes to recognize with appreciation the outstanding and innovative research and the comprehensive HGPS reviews developed by Dr. Vicente Andrés and colleagues at Centro Nacional de Investigaciones Cardiovasculares Carlos III in Madrid, Spain. In addition, Dr. Leslie B. Gordon (Hasbro Children’s Hospital and the Alpert Medical School of Brown University in Providence, RI, and Medical Director of the Progeria Research Foundation) has been a tireless and eloquent international leader and advocate in the pursuit of the understanding and treatment of HGPS; without Dr. Gordon and her colleagues at the PRF, we would know only a fraction of what is understood about the disease.

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Authors and Affiliations

  1. Harvard Medical School and Brigham and Women’s Hospital, Boston, MA, USA

    Richard N. Mitchell

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  1. Richard N. Mitchell
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Correspondence to Richard N. Mitchell .

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Editors and Affiliations

  1. Department of Medicine, Brigham and Women’s Hospital, Harvard Medical School, Boston, MA, USA

    Elena Aikawa

  2. Department of Biomedical Engineering, Florida International University, Miami, FL, USA

    Joshua D. Hutcheson

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Mitchell, R.N. (2020). Cardiovascular Calcification in Hutchinson-Gilford Progeria and Correlation with Age-Related Degenerative Calcification. In: Aikawa, E., Hutcheson, J. (eds) Cardiovascular Calcification and Bone Mineralization. Contemporary Cardiology. Humana, Cham. https://doi.org/10.1007/978-3-030-46725-8_11

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  • DOI: https://doi.org/10.1007/978-3-030-46725-8_11

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  • Publisher Name: Humana, Cham

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  • Online ISBN: 978-3-030-46725-8

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