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One level up: abnormal proteolytic regulation of IGF activity plays a role in human pathophysiology

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dc.contributor.author Argente, Jesús
dc.contributor.author Chowen, Julie A.
dc.contributor.author Pérez Jurado, Luis Alberto
dc.contributor.author Frystyk, Jan
dc.contributor.author Oxvig, Claus
dc.date.accessioned 2017-12-13T16:15:11Z
dc.date.available 2017-12-13T16:15:11Z
dc.date.issued 2017
dc.identifier.citation Argente J, Chowen JA, Pérez-Jurado LA, Frystyk J, Oxvig C. One level up: abnormal proteolytic regulation of IGF activity plays a role in human pathophysiology. EMBO Mol Med. 2017 Oct;9(10):1338-1345. DOI: 10.15252/emmm.201707950
dc.identifier.issn 1757-4676
dc.identifier.uri http://hdl.handle.net/10230/33477
dc.description.abstract The discovery of a mutation in a specific gene can be very important for determining the pathophysiology underlying the disease of a patient and may also help to decide the best treatment protocol on an individual basis. However, sometimes the discovery of mutations in new proteins advances our comprehension in a more widespread manner. The growth hormone (GH)/insulin‐like growth factor (IGF)‐1 axis is fundamental for systemic growth, but is also involved in many other important processes. Our understanding of this system in physiology and pathophysiology has advanced throughout the years with each discovery of mutations in members of this axis. This review focuses on the most recent discovery: mutations in the metalloproteinase pregnancy‐associated plasma protein‐A2 (PAPP‐A2), one of the proteases involved in liberating IGF‐1 from the complexes in which it circulates, in patients with delayed growth failure. We also discuss the advances in the stanniocalcins (STC1 and STC2), proteins that modulate PAPP‐A2, as well as PAPP‐A. These new advances not only bring us one step closer to understanding the strict spatial and temporal control of this axis in systemic growth and maturation, but also highlight possible therapeutic targets when this system goes awry.
dc.description.sponsorship The authors are funded by Fondos de Investigación Sanitaria and fondos FEDER (Grants PI1302195 and PI1600485 to JA), Ministerio de Ciencia e Innovación (BFU2014‐51836‐C2‐2‐R to JAC), Centro de Investigación Biomédica en Red Fisiopatología de Obesidad y Nutrición (CIBEROBN), Instituto de Salud Carlos III (JA), and Fundación Endocrinología y Nutrición.
dc.format.mimetype application/pdf
dc.language.iso eng
dc.publisher Wiley-Blackwell
dc.relation.ispartof EMBO Mol Med. 2017 Oct;9(10):1338-1345
dc.rights © 2017 The Authors. Published under the terms of the CC BY 4.0 license. This is an open access article under the terms of the Creative Commons Attribution 4.0 License (https://creativecommons.org/licenses/by/4.0/), which permits use, distribution and reproduction in any medium, provided the original work is properly cited.
dc.rights.uri http://creativecommons.org/licenses/by/4.0/
dc.rights.uri https://creativecommons.org/licenses/by/4.0/
dc.rights.uri https://creativecommons.org/licenses/by/4.0/
dc.title One level up: abnormal proteolytic regulation of IGF activity plays a role in human pathophysiology
dc.type info:eu-repo/semantics/article
dc.identifier.doi http://dx.doi.org/10.15252/emmm.201707950
dc.subject.keyword IGFBPs
dc.subject.keyword IGF‐1
dc.subject.keyword PAPP‐A
dc.subject.keyword PAPP‐A2
dc.subject.keyword Stanniocalcins (STC1, STC2)
dc.relation.projectID info:eu-repo/grantAgreement/ES/1PE/BFU2014‐51836‐C2‐2‐R
dc.rights.accessRights info:eu-repo/semantics/openAccess
dc.type.version info:eu-repo/semantics/publishedVersion


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