Treatment With Recombinant Human Insulin-Like Growth Factor-1 Improves Growth in Patients With PAPP-A2 Deficiency | Zendy

M.T. Muñoz-Calvo | Zendy; Vicente Barrios | Zendy; Jesús Pozo | Zendy; Julie A. Chowen | Zendy; Gabriel Ángel MartosMoreno | Zendy; Federico Hawkins | Zendy; Andrew Dauber | Zendy; Horacio M. Domené | Zendy; Shoshana Yakar | Zendy; Ron G. Rosenfeld | Zendy; Luis A. PérezJurado | Zendy; Claus Oxvig | Zendy; Jan Frystyk | Zendy; Jesús Argente | Zendy

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Treatment With Recombinant Human Insulin-Like Growth Factor-1 Improves Growth in Patients With PAPP-A2 Deficiency

Author(s) -

M.T. Muñoz-Calvo,

Vicente Barrios,

Jesús Pozo,

Julie A. Chowen,

Gabriel Ángel MartosMoreno,

Federico Hawkins,

Andrew Dauber,

Horacio M. Domené,

Shoshana Yakar,

Ron G. Rosenfeld,

Luis A. PérezJurado,

Claus Oxvig,

Jan Frystyk,

Jesús Argente

Publication year - 2016

Publication title -

the journal of clinical endocrinology and metabolism

Language(s) - English

Resource type - Journals

SCImago Journal Rank - 2.206

H-Index - 353

eISSN - 1945-7197

pISSN - 0021-972X

DOI - 10.1210/jc.2016-2751

Subject(s) - endocrinology , medicine , hypoglycemia , recombinant dna , short stature , growth factor , mutation , pregnancy associated plasma protein a , gene , biology , insulin , pregnancy , receptor , gestation , genetics , first trimester

Pregnancy-associated plasma protein-A2 (PAPP-A2) is a metalloproteinase that specifically cleaves IGFBP-3 and IGFBP-5. Mutations in the PAPP-A2 gene have recently been shown to cause postnatal growth failure in humans, with specific skeletal features, due to the resulting decrease in IGF-1 bioavailability. However, a pharmacological treatment of this entity is yet to be established.

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