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Article Dans Une Revue BONE Année : 2024

A proteomic study of the downregulation of TRIM37 on chondrocytes: Implications for the MULIBREY syndrome

Résumé

MULIBREY nanism which results from autosomal recessive mutations in TRIM37 impacts skeletal development, leading to growth delay with complications in multiple organs. In this study, we employed a combined proteomics and qPCR screening approach to investigate the molecular alterations in the CHON-002 cell line by comparing CHON-002 wild-type (WT) cells to CHON-002 TRIM37 knockdown (KD) cells. Our proteomic analysis demonstrated that TRIM37 depletion predominantly affects the expression of extracellular matrix proteins (ECM). Specifically, nanoLC-MS/MS experiments revealed an upregulation of SPARC, and collagen products (COL1A1, COL3A1, COL5A1) in response to TRIM37 KD. Concurrently, large-scale qPCR assays targeting osteogenesis-related genes corroborated these dysregulations of SPARC at the mRNA level. Gene ontology enrichment analysis highlighted the involvement of dysregulated proteins in ECM organization and TGF-β signaling pathways, indicating a role for TRIM37 in maintaining ECM integrity and regulating chondrocyte proliferation. These findings suggest that TRIM37 deficiency in chondrocytes change ECM protein composition and could impairs long bone growth, contributing to the pathophysiology of MULIBREY nanism.

Domaines

Hématologie

Dates et versions

hal-04654312 , version 1 (19-07-2024)

Identifiants

Citer

Benjamin Brigant, Valérie Metzinger-Le Meuth, Victor Boyartchuk, Hakim Ouled-Haddou, Ida Chiara Guerrera, et al.. A proteomic study of the downregulation of TRIM37 on chondrocytes: Implications for the MULIBREY syndrome. BONE, 2024, pp.117205. ⟨10.1016/j.bone.2024.117205⟩. ⟨hal-04654312⟩
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