A1 Refereed original research article in a scientific journal
HERITABLE DENTIN DEFECTS - NOSOLOGY, PATHOLOGY, AND TREATMENT
Authors: RANTA H, LUKINMAA PL, WALTIMO J
Publisher: WILEY-LISS
Publication year: 1993
Journal: American journal of medical genetics
Journal name in source: AMERICAN JOURNAL OF MEDICAL GENETICS
Journal acronym: AM J MED GENET
Volume: 45
Issue: 2
First page : 193
Last page: 200
Number of pages: 8
ISSN: 0148-7299
DOI: https://doi.org/10.1002/ajmg.1320450209
Abstract
Heritable dentin defects have been divided into 2 main categories: dentinogenesis imperfecta (DI) and dentin dysplasia (DD). Recent studies have shown that they share many features in common. Of the connective tissue diseases, only osteogenesis imperfecta (OI) has been linked to these disorders. So far, no definitive relation between the type of OI and the dental involvement can be established. Familial occurrence of DI with OI cannot be comprehensively explained by mutations in type I collagen genes. No information about the gene defects in DD is available. At the ultrastructural level, the organization of the normally cross-striated collagen fibers in the dentin matrix varies markedly in patients affected by DI.
Heritable dentin defects have been divided into 2 main categories: dentinogenesis imperfecta (DI) and dentin dysplasia (DD). Recent studies have shown that they share many features in common. Of the connective tissue diseases, only osteogenesis imperfecta (OI) has been linked to these disorders. So far, no definitive relation between the type of OI and the dental involvement can be established. Familial occurrence of DI with OI cannot be comprehensively explained by mutations in type I collagen genes. No information about the gene defects in DD is available. At the ultrastructural level, the organization of the normally cross-striated collagen fibers in the dentin matrix varies markedly in patients affected by DI.