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The Non-collagenous Dentin Matrix Proteins are Involved in Dentinogenesis Imperfecta Type II (DGI-II)

Department of Oral Biology, School of Dentistry, M/C 690, University of Illinois at Chicago, Chicago, IL 60612, USA

T. Mah

Department of Basic and Behavioral Sciences, Northwestern University, Chicago, IL 60611, USA

R. Srinivasan

Department of Oral Biology, School of Dentistry, M/C 690, University of Illinois at Chicago, Chicago, IL 60612, USA

Y. Takagi

Department of Pediatric Dentistry, School of Dentistry, Tokyo Medical and Dental University, 1-5-45, Yushima, Bunkyo-Ku, Tokyo 113-8549, Japan

A. Veis

Department of Basic and Behavioral Sciences, Northwestern University, Chicago, IL 60611, USA

A. George

Department of Oral Biology, School of Dentistry, M/C 690, University of Illinois at Chicago, Chicago, IL 60612, USA

Dentinogenesis Imperfecta type II (DGI-II) is a localized form of mesodermal dysplasia of the dentin affecting both the primary and permanent dentitions. This is an autosomal-dominant disease in which there is a disorder in dentin mineralization. Several studies have localized DGI-II to human chromosome 4 in the region 4q 12-21. Many ECM genes—such as OPN, DMP1, DMP2, DMP3 (DSPP), and BSP—have been mapped to the same locus. Biochemical studies indicated that dentin phosphophoryn (DMP2) might be a candidate gene in DGI-II. In this study, we have used histological and RFLP analyses of tissues from a DGI-II-affected patient, as compared with two normal controls, to determine if DMP1, 2, or 3 was linked to DGI-II. The histology of the affected tooth was very different in the DGI-II patient as compared with the normals. In particular, the dentinal tubules in the DGI-1I patient were very irregular, which could be the result of perturbations in the process of dentin formation. Patient and control DNA samples were digested with EcoRI or Pstl and Southern-hybridized with the DMP1, DMP2, and DMP3 cDNAs. Few differences in the restriction pattern were observed between affected and normal samples for DMP1 and DMP3-3' region (phosphophoryn-like sequences) probes. On the other hand, DMP2 showed a dramatic shift in the restriction pattern in DGI-II. This study suggests that the different restriction enzyme digestion profiles of the DNA from the DGI-II patient, as probed by DMP2, might be related to the defective mineralization of dentin in DGI-II.

Key Words: dentinogenesis imperfecta type II • non-collagenous proteins • dentin matrix proteins and biomineralization

Journal of Dental Research, Vol. 79, No. 3, 835-839 (2000)
DOI: 10.1177/00220345000790030901


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