Zhou et al., "Missense mutations abolishing DNA binding of the osteoblast-specific transcription factor OSF2/CBFA1 in cleidocranial dysplasia
," Nature Genetics, vol.
: A report of two cases with brief review.
Functional analysis of RUNX2 mutations in Japanese patients with cleidocranial dysplasia
demonstrates novel genotype-phenotype correlations.
Characterisation of novel RUNX2 mutation with alanine tract expansion from Japanese cleidocranial dysplasia
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Dysregulation of chondrogenesis in human cleidocranial dysplasia
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. Lung India 2010; 27: 176-177.
is a rare syndrome usually having autosomal dominant inheritance.
Hundreds of radiographs and color photographs illustrate the placement and activation of appliances, palatally impacted canines, abnormal premolar orientation, infraocclusion, dentigerous cysts, traumatic impaction, cleidocranial dysplasia
. New to the second edition are five chapters on incisor root resorption, lingual appliances, and wider application of orthodontic implants.
Cleidocranial Dysplasia Cleidocranial dysplasia
is characterized by lack of normal development or absence of the clavicle bone, late ossification of cranial sutures, frontal and parietal bossing, shortness of stature and orodental abnormalities.
Olsen and his group came across Cbfa1 during a search for the genetic mutations that cause cleidocranial dysplasia
syndrome (CCD), a rare human skeletal disorder.
The conditions commonly associated with an increased prevalence of supernumerary teeth include cleft lip and palate, cleidocranial dysplasia
and Gardner syndrome (10).
Differential gene expressions by dental follicle needed for osteoclastogenesis, osteogenesis and pressure from underlying succedaneous teeth are responsible for timely shedding and eruption process of succedaneous teeth (3) and several local, systemic causes including syndromes (Cleidocranial dysplasia
, Gardner's syndrome) and metabolic or hormonal diseases have been attributed in literature for impacted and embedded teeth.
Multiple supernumerary teeth are usually associated with development disorders or syndromes such as cleidocranial dysplasia
or Gardner syndrome.
The cleidocranial dysplasia
, also known as Marie and Sainton Disease, Scheuthauer Marie-Sainton Syndrome and Mutational dysostosis (Shafer et al., 1979) is a rare disease which can occur either spontaneously or by a dominant autosomal inheritance, with no predilection of genre or ethnic group (Silva et al., 1995; Neville et al., 2004).