Amb motiu del tancament d'estiu, la validació de documents es reprendrà a partir del 28 d'agost de 2026. Disculpeu les molèsties.
Con motivo del cierre de verano, la validación de documentos se reanudará a partir del 28 de agosto de 2026. Disculpad las molestias
Due to the summer closure, document validation will resume starting August 28, 2026. We apologize for any inconvenience.

Files

618733.pdf (4.08 MB)

Document type

Article

Version

Published version

Publication date

2010-04-28

Publication license

cc-by (c) Sarmah, Swapnalee et al., 2010
Please use this identifier to cite or link to this item: https://hdl.handle.net/2445/103125

Sec24D-dependent transport of extracellular matrix proteins is required for zebrafish skeletal morphogenesis

Journal Title

Authors

Director/Tutor

Journal ISSN

Volume Title

Related resource

https://doi.org/10.1371/journal.pone.0010367

Abstract

Protein transport from endoplasmic reticulum (ER) to Golgi is primarily conducted by coated vesicular carriers such as COPII. Here, we describe zebrafish bulldog mutations that disrupt the function of the cargo adaptor Sec24D, an integral component of the COPII complex. We show that Sec24D is essential for secretion of cartilage matrix proteins, whereas the preceding development of craniofacial primordia and pre-chondrogenic condensations does not depend on this isoform. Bulldog chondrocytes fail to secrete type II collagen and matrilin to extracellular matrix (ECM), but membrane bound receptor β1-Integrin and Cadherins appear to leave ER in Sec24D-independent fashion. Consequently, Sec24D-deficient cells accumulate proteins in the distended ER, although a subset of ER compartments and Golgi complexes as visualized by electron microscopy and NBD C6-ceramide staining appear functional. Consistent with the backlog of proteins in the ER, chondrocytes activate the ER stress response machinery and significantly upregulate BiP transcription. Failure of ECM secretion hinders chondroblast intercalations thus resulting in small and malformed cartilages and severe craniofacial dysmorphology. This defect is specific to Sec24D mutants since knockdown of Sec24C, a close paralog of Sec24D, does not result in craniofacial cartilage dysmorphology. However, craniofacial development in double Sec24C/Sec24D-deficient animals is arrested earlier than in bulldog/sec24d, suggesting that Sec24C can compensate for loss of Sec24D at initial stages of chondrogenesis, but Sec24D is indispensable for chondrocyte maturation. Our study presents the first developmental perspective on Sec24D function and establishes Sec24D as a strong candidate for cartilage maintenance diseases and craniofacial birth defects.

Subject

Proteïnes de membrana, Proteïnes portadores, Matriu extracel·lular, Peix zebra, Esquelet, Morfogènesi

Subject (English)

Membrane proteins, Carrier proteins, Extracellular matrix, Zebra danio, Skeleton, Morphogenesis

Citation

Collections

Articles publicats en revistes (Ciències Fisiològiques)

Citation

SARMAH, Swapnalee, et al. Sec24D-dependent transport of extracellular matrix proteins is required for zebrafish skeletal morphogenesis. PLoS One. 2010. Vol. 5, num. 4, pags. e10367. ISSN 1932-6203. [consulted: 13 of August of 2026]. Available at: https://hdl.handle.net/2445/103125

Export metadata

JSON - METS

Share record