Utilizing the chicken as an animal model for human craniofacial ciliopathies.

Dev Biol

Division of Plastic Surgery, Department of Surgery, Cincinnati Children's Hospital Medical Center, Cincinnati, OH 45229, USA; Division of Developmental Biology, Department of Pediatrics, Cincinnati Children's Hospital Medical Center, Cincinnati, OH 45229, USA. Electronic address:

Published: July 2016

AI Article Synopsis

  • Chickens have been a valuable model for studying craniofacial development and disease due to their size and accessibility, allowing for various experimental techniques.
  • Two well-known mutant lines, talpid(2) and talpid(3), have distinct genetic causes but both exhibit craniofacial ciliopathies linked to human conditions like Oral-facial-digital syndrome and Joubert syndrome.
  • The review focuses on these mutants, discussing their implications for understanding the molecular and cellular basis of ciliopathies, and emphasizes the potential of combining classical experiments with modern techniques like transgenics and genome editing to further advance research.

Article Abstract

The chicken has been a particularly useful model for the study of craniofacial development and disease for over a century due to their relatively large size, accessibility, and amenability for classical bead implantation and transplant experiments. Several naturally occurring mutant lines with craniofacial anomalies also exist and have been heavily utilized by developmental biologist for several decades. Two of the most well known lines, talpid(2) (ta(2)) and talpid(3) (ta(3)), represent the first spontaneous mutants to have the causative genes identified. Despite having distinct genetic causes, both mutants have recently been identified as ciliopathic. Excitingly, both of these mutants have been classified as models for human craniofacial ciliopathies: Oral-facial-digital syndrome (ta(2)) and Joubert syndrome (ta(3)). Herein, we review and compare these two models of craniofacial disease and highlight what they have revealed about the molecular and cellular etiology of ciliopathies. Furthermore, we outline how applying classical avian experiments and new technological advances (transgenics and genome editing) with naturally occurring avian mutants can add a tremendous amount to what we currently know about craniofacial ciliopathies.

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Source
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4842343PMC
http://dx.doi.org/10.1016/j.ydbio.2015.10.024DOI Listing

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