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Muenke syndrome mutation, FgfR3P²⁴⁴R, causes TMJ defects.

T Yasuda¹, H D Nah, J Laurita

¹Division of Orthopaedic Surgery, The Children's Hospital of Philadelphia, Abramson Research Center, 3516 Civic Center Blvd, Philadelphia, PA 19107, USA.

Journal of Dental Research

|May 25, 2012

Summary

Muenke syndrome mutations in fibroblast growth factor receptor 3 (FGFR3) disrupt temporomandibular joint (TMJ) development. This study reveals FGFR3 mutations impair hedgehog signaling and endochondral ossification, impacting TMJ structure and cellular organization.

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Area of Science:

Developmental Biology
Genetics
Orthodontics

Background:

Muenke syndrome, caused by FGFR3 mutations, leads to craniofacial deformities.
The role of FGFR3 in temporomandibular joint (TMJ) development is not fully understood.

Purpose of the Study:

To investigate the impact of an activating FGFR3 mutation on TMJ development and growth in mice.
To elucidate the molecular mechanisms underlying FGFR3-mediated TMJ abnormalities.

Main Methods:

Utilized a mouse model with a specific FgfR3 mutation (FgfR3(P244R)).
Employed in situ hybridization to analyze gene expression patterns (FgfR3, Ihh, Ptch1).
Assessed chondroprogenitor proliferation, chondrocyte differentiation, and bone formation markers.

Main Results:

FgfR3(P244R) mutants showed reduced Indian hedgehog (Ihh) signaling and chondrogenesis.
Abnormalities included disturbed primary bone spongiosa formation, increased osteoclast activity, and reduced trabecular bone.
Degenerative changes in articular cartilage, abnormal TMJ fossa development, and articular disc fusion were observed.

Conclusions:

Activating FGFR3 mutations disrupt TMJ development by impairing hedgehog signaling and endochondral ossification.
A balance between FGF and hedgehog signaling is crucial for TMJ integrity and cellular organization.