Dysregulation of the BMP-p38 MAPK signaling pathway in cells from patients with fibrodysplasia ossificans progressiva (FOP)

被引：81

作者：

Fiori, Jennifer L.

Billings, Paul C.

Serrano de la Pena, Lourdes

Kaplan, Frederick S.

Shore, Eileen M.

机构：

[1] Univ Penn, Dept Orthopaed Surg, Sch Med, Philadelphia, PA 19104 USA

[2] Univ Penn, Sch Med, Dept Med, Philadelphia, PA 19104 USA

[3] Univ Penn, Sch Med, Dept Genet, Philadelphia, PA USA

来源：

JOURNAL OF BONE AND MINERAL RESEARCH | 2006年 / 21卷 / 06期

关键词：

fibrodysplasia ossificans progressiva; BMP; p38 mitogen-activated protein kinase; Smad; signaling pathways;

D O I：

10.1359/JBMR.060215

中图分类号：

R5 [内科学];

学科分类号：

1002 [临床医学]; 100201 [内科学];

摘要：

FOP is a disabling disorder in which skeletal muscle is progressively replaced with bone. Lymphocytes, our model systems for examining BMP signaling, cannot signal through the canonical Smad pathway unless exogenous Smad1 is supplied, providing a unique cell type in which the BMP-p38 MAPK pathway can be examined. FOP lymphocytes exhibit defects in the BMP-p38 MAPK pathway, suggesting that altered BMP signaling underlies ectopic bone formation in this disease.

引用

收藏

页码：902 / 909

页数：8

相关论文

共 54 条

[1]

Paresis of a bone morphogenetic protein-antagonist response in a genetic disorder of heterotopic skeletogenesis [J].

Ahn, J ;

de la Peña, LS ;

Shore, EM ;

Kaplan, FS .

JOURNAL OF BONE AND JOINT SURGERY-AMERICAN VOLUME, 2003, 85A (04) :667-674

[2]

Bone morphogenetic protein signalling in NGF-stimulated PC12 cells [J].

Althini, S ;

Usoskin, D ;

Kylberg, A ;

ten Dijke, P ;

Ebendal, T .

BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS, 2003, 307 (03) :632-639

[3]

Smads as transcriptional co-modulators [J].

Attisano, L ;

Wrana, JL .

CURRENT OPINION IN CELL BIOLOGY, 2000, 12 (02) :235-243

[4]

Sonic hedgehog induces the proliferation of primitive human hematopoietic cells via BMP regulation [J].

Bhardwaj, G ;

Murdoch, B ;

Wu, D ;

Baker, DP ;

Williams, KP ;

Chadwick, K ;

Ling, LE ;

Karanu, FN ;

Bhatia, M .

NATURE IMMUNOLOGY, 2001, 2 (02) :172-180

[5]

A phylogenetically conserved cis-regulatory module in the Msx2 promoter is sufficient for BMP-dependent transcription in murine and Drosophila embryos [J].

Brugger, SM ;

Merrill, AE ;

Torres-Vazquez, J ;

Wu, N ;

Ting, MC ;

Cho, JYM ;

Dobias, SL ;

Yi, SE ;

Lyons, K ;

Bei, JR ;

Arora, K ;

Warrior, R ;

Maxson, R .

DEVELOPMENT, 2004, 131 (20) :5153-5165

[6]

Bone morphogenetic proteins, their antagonists, and the skeleton [J].

Canalis, E ;

Economides, AN ;

Gazzerro, E .

ENDOCRINE REVIEWS, 2003, 24 (02) :218-235

[7]

THE NATURAL-HISTORY OF HETEROTOPIC OSSIFICATION IN PATIENTS WHO HAVE FIBRODYSPLASIA OSSIFICANS PROGRESSIVA - A STUDY OF 44 PATIENTS [J].

COHEN, RB ;

HAHN, GV ;

TABAS, JA ;

PEEPER, J ;

LEVITZ, CL ;

SANDO, A ;

SANDO, N ;

ZASLOFF, M ;

KAPLAN, FS .

JOURNAL OF BONE AND JOINT SURGERY-AMERICAN VOLUME, 1993, 75A (02) :215-219

[8]

FIBRODYSPLASIA OSSIFICANS PROGRESSIVA - THE CLINICAL-FEATURES AND NATURAL-HISTORY OF 34 PATIENTS [J].

CONNOR, JM ;

EVANS, DAP .

JOURNAL OF BONE AND JOINT SURGERY-BRITISH VOLUME, 1982, 64 (01) :76-83

[9]

THE FUNCTION AND EVOLUTION OF MSX GENES - POINTERS AND PARADOXES [J].

DAVIDSON, D .

TRENDS IN GENETICS, 1995, 11 (10) :405-411

[10]

Fibrodysplasia ossificans progressiva (FOP), a disorder of ectopic osteogenesis, misregulates cell surface expression and trafficking of BMPRIA [J].

de la Peña, LS ;

Billings, PC ;

Fiori, JL ;

Ahn, J ;

Kaplan, FS ;

Shore, EM .

JOURNAL OF BONE AND MINERAL RESEARCH, 2005, 20 (07) :1168-1176

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