{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,21]],"date-time":"2026-08-21T12:05:30Z","timestamp":1787313930749,"version":"build-2736575974"},"reference-count":20,"publisher":"The Endocrine Society","issue":"2","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2015,2,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Context:<\/jats:title>\n                    <jats:p>C-type natriuretic peptide (CNP) is a crucial regulator of endochondral bone growth. In a previous report of a child with acromesomelic dysplasia, Maroteaux type (AMDM), caused by loss-of-function of the CNP receptor (natriuretic peptide receptor-B [NPR-B]), plasma levels of CNP were elevated. In vitro studies have shown that activation of the MAPK kinase (MEK)\/ERK MAPK pathway causes functional inhibition of NPR-B. Achondroplasia, hypochondroplasia, and thanatophoric dysplasia are syndromes of short-limbed dwarfism caused by activating mutations of fibroblast growth factor receptor-3, which result in overactivation of the MEK\/ERK MAPK pathway.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Objective:<\/jats:title>\n                    <jats:p>The purpose of this study was to determine whether these syndromes exhibit evidence of CNP resistance as reflected by increases in plasma CNP and its amino-terminal propeptide (NTproCNP).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Design:<\/jats:title>\n                    <jats:p>This was a prospective, observational study.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Subjects:<\/jats:title>\n                    <jats:p>Participants were 63 children and 20 adults with achondroplasia, 6 children with hypochondroplasia, 2 children with thanatophoric dysplasia, and 4 children and 1 adult with AMDM.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results:<\/jats:title>\n                    <jats:p>Plasma levels of CNP and NTproCNP were higher in children with achondroplasia with CNP SD scores (SDSs) of 1.0 (0.3\u20131.4) (median [interquartile range]) and NTproCNP SDSs of 1.4 (0.4\u20131.8; P &amp;lt; .0005). NTproCNP levels correlated with height velocity. Levels were also elevated in adults with achondroplasia (CNP SDSs of 1.5 [0.7\u20132.1] and NTproCNP SDSs of 0.5 [0.1\u20131.0], P &amp;lt; .005). In children with hypochondroplasia, CNP SDSs were 1.3 (0.7\u20131.5) (P = .08) and NTproCNP SDSs were 1.9 (1.8\u20132.3) (P &amp;lt; .05). In children with AMDM, CNP SDSs were 1.6 (1.4\u20133.3) and NTproCNP SDSs were 4.2 (2.7\u20136.2) (P &amp;lt; .01).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions:<\/jats:title>\n                    <jats:p>In these skeletal dysplasias, elevated plasma levels of proCNP products suggest the presence of tissue resistance to CNP.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1210\/jc.2014-2814","type":"journal-article","created":{"date-parts":[[2014,11,11]],"date-time":"2014-11-11T13:38:52Z","timestamp":1415713132000},"page":"E355-E359","source":"Crossref","is-referenced-by-count":38,"title":["C-Type Natriuretic Peptide Plasma Levels Are Elevated in Subjects With Achondroplasia, Hypochondroplasia, and Thanatophoric Dysplasia"],"prefix":"10.1210","volume":"100","author":[{"given":"Robert C.","family":"Olney","sequence":"first","affiliation":[{"name":"Nemours Children's Clinic (R.C.O.), Jacksonville, Florida 32207"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Timothy C. R.","family":"Prickett","sequence":"additional","affiliation":[{"name":"University of Otago (T.C.R.P., E.A.E.), Christchurch 8011, New Zealand"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eric A.","family":"Espiner","sequence":"additional","affiliation":[{"name":"University of Otago (T.C.R.P., E.A.E.), Christchurch 8011, New Zealand"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"William G.","family":"Mackenzie","sequence":"additional","affiliation":[{"name":"Nemours\/Alfred I. duPont Hospital for Children (W.G.M., A.L.D., C.D., M.B.B.), Wilmington, Delaware 19803"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Angela L.","family":"Duker","sequence":"additional","affiliation":[{"name":"Nemours\/Alfred I. duPont Hospital for Children (W.G.M., A.L.D., C.D., M.B.B.), Wilmington, Delaware 19803"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Colleen","family":"Ditro","sequence":"additional","affiliation":[{"name":"Nemours\/Alfred I. duPont Hospital for Children (W.G.M., A.L.D., C.D., M.B.B.), Wilmington, Delaware 19803"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bernhard","family":"Zabel","sequence":"additional","affiliation":[{"name":"University Hospital Freiburg (B.Z.), 79106 Freiburg, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tomonobu","family":"Hasegawa","sequence":"additional","affiliation":[{"name":"Keio University School of Medicine (T.H.), Tokyo 108\u20138345, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hiroshi","family":"Kitoh","sequence":"additional","affiliation":[{"name":"Nagoya University School of Medicine (H.K.), Nagoya 464\u20138601, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Arthur S.","family":"Aylsworth","sequence":"additional","affiliation":[{"name":"University of North Carolina (A.S.A.), Chapel Hill, North Carolina 27599\u20132100"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael B.","family":"Bober","sequence":"additional","affiliation":[{"name":"Nemours\/Alfred I. duPont Hospital for Children (W.G.M., A.L.D., C.D., M.B.B.), Wilmington, Delaware 19803"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"80","reference":[{"key":"2020071614372918200_B1","doi-asserted-by":"crossref","first-page":"S6","DOI":"10.1016\/j.ghir.2006.03.016","article-title":"C-type natriuretic peptide in growth: a new paradigm","volume":"16","author":"Olney","year":"2006","journal-title":"Growth Horm IGF Res"},{"key":"2020071614372918200_B2","doi-asserted-by":"crossref","first-page":"27","DOI":"10.1086\/422013","article-title":"Mutations in the transmembrane natriuretic peptide receptor NPR-B impair skeletal growth and cause acromesomelic 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