Biomaterial Database

Ultrastructure of dentin matrix in heritable dentin defects. 1995

J Waltimo, and H Ranta, and P L Lukinmaa

Department of Pedodontics, Institute of Dentistry, University of Helsinki, Finland.

Heritable dentin defects form a group of diseases which exclusively affect dentin among the various dental tissues. While one type is associated with the generalized connective tissue disorder, osteogenesis imperfecta, other types occur as single traits. The clinical manifestations of the dentin defects vary from insignificant to severe enough to cause aesthetical and functional failure of the teeth. Scanning and transmission electron microscopic studies, reviewed in this paper, have markedly clarified the ultrastructure of the aberrant dentin matrix. Both similar and different changes seem to occur in the various forms of heritable dentin defects. Abnormalities in the appearance and organization pattern of collagen fibers in the defective dentin partly resemble those observed in skin in generalized connective tissue diseases. The similarity of ultrastructural findings in dentin defects, which are currently classified as distinct entities, and even in diseases affecting other tissues, could be related to the complicated interactions between the extracellular matrix macromolecules. Thus, many of the changes observed may be secondary in nature. Ultrastructural studies can help us to understand the pathogenesis of the different types of heritable dentin defects as well as aid in diagnostics and classification of these diseases.

UI	MeSH Term	Description	Entries
D008855	Microscopy, Electron, Scanning	Microscopy in which the object is examined directly by an electron beam scanning the specimen point-by-point. The image is constructed by detecting the products of specimen interactions that are projected above the plane of the sample, such as backscattered electrons. Although SCANNING TRANSMISSION ELECTRON MICROSCOPY also scans the specimen point by point with the electron beam, the image is constructed by detecting the electrons, or their interaction products that are transmitted through the sample plane, so that is a form of TRANSMISSION ELECTRON MICROSCOPY.	Scanning Electron Microscopy,Electron Scanning Microscopy,Electron Microscopies, Scanning,Electron Microscopy, Scanning,Electron Scanning Microscopies,Microscopies, Electron Scanning,Microscopies, Scanning Electron,Microscopy, Electron Scanning,Microscopy, Scanning Electron,Scanning Electron Microscopies,Scanning Microscopies, Electron,Scanning Microscopy, Electron
D010013	Osteogenesis Imperfecta	COLLAGEN DISEASES characterized by brittle, osteoporotic, and easily fractured bones. It may also present with blue sclerae, loose joints, and imperfect dentin formation. Most types are autosomal dominant and are associated with mutations in COLLAGEN TYPE I.	Fragilitas Ossium,Lobstein Disease,Brittle Bone Disease,Lobstein's Disease,Osteogenesis Imperfecta Tarda,Osteogenesis Imperfecta with Blue Sclerae,Osteogenesis Imperfecta, Type 1,Osteogenesis Imperfecta, Type I,Disease, Lobstein,Disease, Lobstein's,Lobsteins Disease,Ossiums, Fragilitas,Osteogenesis Imperfecta Tardas
D003094	Collagen	A polypeptide substance comprising about one third of the total protein in mammalian organisms. It is the main constituent of SKIN; CONNECTIVE TISSUE; and the organic substance of bones (BONE AND BONES) and teeth (TOOTH).	Avicon,Avitene,Collagen Felt,Collagen Fleece,Collagenfleece,Collastat,Dermodress,Microfibril Collagen Hemostat,Pangen,Zyderm,alpha-Collagen,Collagen Hemostat, Microfibril,alpha Collagen
D003804	Dentin	The hard portion of the tooth surrounding the pulp, covered by enamel on the crown and cementum on the root, which is harder and denser than bone but softer than enamel, and is thus readily abraded when left unprotected. (From Jablonski, Dictionary of Dentistry, 1992)	Dentine,Dentines,Dentins
D003805	Dentin Dysplasia	An apparently hereditary disorder of dentin formation, marked by a normal appearance of coronal dentin associated with pulpal obliteration, faulty root formation, and a tendency for peripheral lesions without obvious cause. (From Dorland, 27th ed)	Dentin Dysplasias,Dysplasia, Dentin,Dysplasias, Dentin
D003811	Dentinogenesis Imperfecta	An autosomal dominant disorder of tooth development characterized by opalescent dentin resulting in discoloration of the teeth. The dentin develops poorly with low mineral content while the pulp canal is obliterated.	Hereditary Opalescent Dentin,Capdepont Teeth,Dentinogenesis Imperfecta 1,Dentinogenesis Imperfecta without Osteogenesis Imperfecta,Dentinogenesis Imperfecta, Shields Type 2,Dentinogenesis Imperfecta, Shields Type II,Opalescent Dentin,Opalescent Teeth without Osteogenesis Imperfecta,Dentin, Opalescent,Opalescent Dentin, Hereditary,Teeth, Capdepont
D005109	Extracellular Matrix	A meshwork-like substance found within the extracellular space and in association with the basement membrane of the cell surface. It promotes cellular proliferation and provides a supporting structure to which cells or cell lysates in culture dishes adhere.	Matrix, Extracellular,Extracellular Matrices,Matrices, Extracellular
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man