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Titolo:
Dwarfism and early death in mice lacking C-type natriuretic peptide
Autore:
Chusho, H; Tamura, N; Ogawa, Y; Yasoda, A; Suda, M; Miyazawa, T; Nakamura, K; Nakao, K; Kurihara, T; Komatsu, Y; Itoh, H; Tanaka, K; Saito, Y; Katsuki, M; Nakao, K;
Indirizzi:
Kyoto Univ, Sch Med, Dept Med & Clin Sci, Kyoto 6068507, Japan Kyoto UnivKyoto Japan 6068507 Dept Med & Clin Sci, Kyoto 6068507, Japan Suntory Inst Biomed Res, Shimamoto, Osaka 6180024, Japan Suntory Inst Biomed Res Shimamoto Osaka Japan 6180024 saka 6180024, Japan Univ Tokyo, Inst Med Sci, Lab Anim Res Ctr, Tokyo 1080071, Japan Univ Tokyo Tokyo Japan 1080071 i, Lab Anim Res Ctr, Tokyo 1080071, Japan
Titolo Testata:
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA
fascicolo: 7, volume: 98, anno: 2001,
pagine: 4016 - 4021
SICI:
0027-8424(20010327)98:7<4016:DAEDIM>2.0.ZU;2-Y
Fonte:
ISI
Lingua:
ENG
Soggetto:
GROWTH-FACTOR RECEPTOR-3; TRANSGENIC MICE; SKELETAL OVERGROWTH; MOUSE; MUTATIONS; ACHONDROPLASIA; GENE; EXPRESSION; SYSTEM; CNP;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Life Sciences
Citazioni:
32
Recensione:
Indirizzi per estratti:
Indirizzo: Ogawa, Y Kyoto Univ, Sch Med, Dept Med & Clin Sci, Kyoto 6068507, Japan Kyoto Univ Kyoto Japan 6068507 & Clin Sci, Kyoto 6068507, Japan
Citazione:
H. Chusho et al., "Dwarfism and early death in mice lacking C-type natriuretic peptide", P NAS US, 98(7), 2001, pp. 4016-4021

Abstract

Longitudinal bone growth is determined by endochondral ossification that occurs as chondrocytes in the cartilaginous growth plate undergo proliferation, hypertrophy, cell death, and osteoblastic replacement. The natriuretic peptide family consists of three structurally related endogenous ligands, atrial, brain, and C-type natriuretic peptides (ANP, BNP, and CNP), and is thought to be involved in a variety of homeostatic processes. To investigatethe physiological significance of CNP in vivo, we generated mice with targeted disruption of CNP (Nppc(-/-) mice). The Nppc(-/-) mice show severe dwarfism as a result of impaired endochondral ossification. They are all viable perinatally, but less than half can survive during postnatal development. The skeletal phenotypes are histologically similar to those seen in patients with achondroplasia, the most common genetic form of human dwarfism. Targeted expression of CNP in the growth plate chondrocytes can rescue the skeletal defect of Nppc(-/-) mice and allow their prolonged survival. This study demonstrates that CNP acts locally as a positive regulator of endochondral ossification in vivo and suggests its pathophysiological and therapeuticimplication in some forms of skeletal dysplasia.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 11/07/20 alle ore 19:44:43