Rat wct mutation prevents differentiation of maturation-stage ameloblasts resulting in hypo-mineralization in incisor teeth.
A recent study provided genetic and morphological evidence that rat autosomal-recessive mutation, whitish chalk-like teeth (wct), induced tooth enamel defects resembling those of human amelogenesis imperfecta (AI). The wct locus maps to a specific interval of rat chromosome 14 corresponding to human chromosome 4q21 where the ameloblastin and enamelin genes exist, although these genes are not included in the wct locus. The effect of the wct gene mutation on the enamel matrix synthesis and calcification remains to be elucidated. This study clarifies how the wct gene mutation influences the synthesis of enamel matrix and its calcification by immunocytochemistry for amelogenin, ameloblastin and enamelin, and by electron probe micro-analysis (EPMA). The immunoreactivity for enamel proteins such as amelogenin, ameloblastin, and enamelin in the ameloblasts in the homozygous teeth was the same as that in the heterozygous teeth from secretory to transitional stages, although the homozygous ameloblasts became detached from the enamel matrix in the transitional stage. The flattened ameloblasts in the maturation stage of the homozygous samples contained enamel proteins in their cytoplasm. Thus, the wct mutation was found to prevent the morphological transition of ameloblasts from secretory to maturation stages without disturbing the synthesis of enamel matrix proteins, resulting in the hypo-mineralization of incisor enamel and cyst formation between the enamel organ and matrix. This mutation also prevents the transfer of iron into the enamel.
Osawa
Masaru
M
Division of Anatomy and Cell Biology of the Hard Tissue, Department of Tissue Regeneration and Reconstruction, Niigata University Graduate School of Medical and Dental Sciences, 2-5274 Gakkocho-dori, Chuo-ku, Niigata, 951-8514, Japan.
Kenmotsu
Shin
S
Masuyama
Taku
T
Taniguchi
Kazuyuki
K
Uchida
Takashi
T
Saito
Chikara
C
Ohshima
Hayato
H
D000565:Ameloblasts / Q000473:pathology*; D000818:Animals; D002454:Cell Differentiation / Q000235:genetics; D025063:Chromosome Disorders / Q000235:genetics*; D003746:Dental Enamel Proteins / Q000096:biosynthesis; D004195:Disease Models, Animal; D004577:Electron Probe Microanalysis / Q000379:methods; D007150:Immunohistochemistry; D007180:Incisor / Q000473:pathology*; D008297:Male; D009154:Mutation; D051381:Rats; D011922:Rats, Mutant Strains; D014057:Tomography, X-Ray Computed; D017001:Tooth Demineralization / Q000473:pathology*; D014965:X-Rays
D003746:Dental Enamel Proteins; C109069:enamel matrix proteins