Midline1 and the development of the cranial peripheral nervous system

Latta, Elizabeth; Saffrey, Jill and Golding, Jon (2008). Midline1 and the development of the cranial peripheral nervous system. In: Joint meeting of the British and Spanish Developmental Biology Societies, 24-27 Sep 2008, Seville, Spain.

Abstract

Genetic abnormalities involving the skull and facial region account for around 1/3 of birth defects. Opitz BBB/G syndrome is one such disorder that gives rise to craniofacial malformations, as well as midline defects that cause gastrointestinal anomalies. Patients with X-linked Opitz BBB/G syndrome have loss of function mutations in the gene Midline 1 (Mid1).

Using a chick model of cranial development, we demonstrate the expression pattern of the chick orthologue of Mid1 and report a novel function for cMid1 in the formation of the cranial ganglia.

In order to study the role of cMid1, we ectopically expressed cMid1 in subpopulations of neural crest cells (NCC’s) from rhombomere 4 (r4), normally devoid of the protein. We find that ectopic targeting of cMid1 to r4 NCCs transiently induces the formation of a larger facial ganglion. Subsequent work to down regulate endogenous cMid1 activity in rhombomere 2 demonstrates a reduction in the size of the developing trigeminal ganglia. Current work aimed at elucidating the mechanisms underlying these observations will be presented.

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