Of hedgehogs and hereditary bone tumors: re-examination of the pathogenesis of osteochondromas

Kevin B Jones; Jose A Morcuende

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Of hedgehogs and hereditary bone tumors: re-examination of the pathogenesis of osteochondromas

Journal article

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Peer reviewed

Of hedgehogs and hereditary bone tumors: re-examination of the pathogenesis of osteochondromas

Kevin B Jones and Jose A Morcuende

The Iowa orthopaedic journal, Vol.23, pp.87-95

2003

PMCID: PMC1888388

PMID: 14575257

Appears in Diamond Open Access

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https://www.ncbi.nlm.nih.gov/pmc/articles/PMC1888388/View

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Abstract

The osteochondroma is a common, benign, primary tumor of bone. A mechanism for its pathogenesis has not been identified, but loss of function of EXT genes is implicated in sporadic and hereditary multiple osteochondromas. Recent advances in the understanding of other molecular signaling pathways in the physis cast doubt on the latest pathogenetic theories. These advances are reviewed and used as the basis for a revised theory for pathogenesis: A clone of proliferating chondrocytes without functional EXT1 (or EXT2) expression fails to produce heparan sulfate; lack of heparan sulfate at the cell surface disrupts fibroblast growth factor signaling and Indian hedgehog diffusion, leading to focal overproliferation and adjacent bone collar deficiency, respectively; together these effects are proposed to contribute to osteochondroma pathogenesis.

Humans

Osteochondroma - genetics

Heparitin Sulfate - metabolism

N-Acetylglucosaminyltransferases - metabolism

Bone Neoplasms - metabolism

Hedgehog Proteins

Osteochondroma - physiopathology

Fibroblast Growth Factors - metabolism

Bone Neoplasms - physiopathology

Genes, Tumor Suppressor - physiology

Osteochondroma - metabolism

Signal Transduction - physiology

Trans-Activators - metabolism

Bone Neoplasms - genetics

Details

Title: Subtitle: Of hedgehogs and hereditary bone tumors: re-examination of the pathogenesis of osteochondromas
Creators: Kevin B Jones - Department of Orthopaedic Surgery, University of Iowa, Iowa City, IA 52242, USA
Jose A Morcuende
Resource Type: Journal article
Publication Details: The Iowa orthopaedic journal, Vol.23, pp.87-95
Publisher: Dept. of Orthopaedics, The University of Iowa; United States
PMID: 14575257
PMCID: PMC1888388
ISSN: 1541-5457
eISSN: 1555-1377
Language: English
Date published: 2003
Academic Unit: Stead Family Department of Pediatrics; Orthopedics and Rehabilitation
Record Identifier: 9984040375102771

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