Article
Defective proteoglycan sulfation of the growth plate zones causes reduced chondrocyte proliferation via an altered Indian hedgehog signalling.
Matrix biology : journal of the International Society for Matrix Biology - 1 Jul 2010
Gualeni Benedetta, Facchini Marcella, De Leonardis Fabio, Tenni Ruggero, Cetta Giuseppe, Viola Manuela, Passi Alberto, Superti-Furga Andrea, Forlino Antonella, Rossi Antonio
Abstract excerpt
Mutations in the sulfate transporter gene, SCL26A2, lead to cartilage proteoglycan undersulfation resulting in chondrodysplasia in humans; the phenotype is mirrored in the diastrophic dysplasia (dtd) mouse. It remains unclear whether bone shortening and deformities are caused solely by changes in the cartilage matrix, or whether chondroitin sulfate proteoglycan undersulfation affects also signalling pathways...
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