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Microdontia
Unusually small teeth; more common in females, associated with hypodontia
Relative microdontia
Normal-sized teeth that appear small because the jaw/maxilla is larger than normal
Diffuse true microdontia
All teeth affected; seen in Down syndrome, pituitary dwarfism, rare hereditary disorders
Most common tooth for isolated microdontia
Maxillary lateral incisor (peg-shaped crown, normal root)
Isolated microdontia inheritance pattern
Autosomal dominant with incomplete penetrance
Macrodontia
Teeth larger than normal
Relative macrodontia
Normal-sized teeth in a small jaw; can cause crowding and impaction
Diffuse macrodontia causes
Pituitary gigantism, pineal hyperplasia with hyperinsulinism, hemifacial hypertrophy
Gemination
A single enlarged/joined tooth where tooth count is NORMAL when the anomalous tooth is counted as one
Gemination location
Anterior maxilla most common; bilateral is less frequent
Fusion
A single enlarged/joined tooth where tooth count reveals a MISSING tooth when the anomalous tooth is counted as one
Fusion location
Anterior maxilla most common; bilateral less frequent
Concrescence
Union of two adjacent teeth by cementum alone, WITHOUT confluence of underlying dentin
Concrescence common cause
Post-inflammatory, usually in large carious molars with periapical pathosis (posterior maxilla most common)
Turner's hypoplasia
Enamel defects on a permanent tooth caused by periapical inflammatory disease of the overlying deciduous tooth
Turner's tooth
The affected permanent tooth resulting from Turner's hypoplasia
Determining factors of Turner's hypoplasia severity
Stage of tooth development, length of time infection remains untreated, virulence of organism, host resistance, traumatic injury
Turner's hypoplasia most commonly affects
Permanent bicuspids (premolars), since they sit next to primary molars that get infected
Syphilitic hypoplasia
Dental changes due to congenital syphilis; currently very rare; causes enamel hypoplasia
Hutchinson's teeth
Anterior teeth shaped like straight-edge screwdrivers with a central notch on the incisal edge (from congenital syphilis)
Mulberry molars
Molars with constricted occlusal table and disorganized surface anatomy resembling a mulberry (from congenital syphilis)
Talon cusp
Well-delineated additional cusp on an anterior tooth extending at least half the distance from the CEJ to the incisal edge
Talon cusp most common location
Maxillary lateral incisor
Talon cusp genetic influence
Documented in twins
Talon cusp permanent teeth distribution
55% maxillary lateral, 33% maxillary central, 6% mandibular incisors, 4% maxillary canine
Talon cusp associated with
Supernumerary teeth, odontoma, impacted teeth, peg-shaped lateral incisors, syndromes (Mohr, Rubinstein-Taybi, Sturge-Weber)
Ankylosis
Cessation of eruption after emergence due to anatomic fusion of cementum with alveolar bone
Ankylosis cause
Unknown; may be trauma, injury, chemical/thermal irritation
Ankylosis other terms
Infraocclusion, secondary retention, submergence, reimpaction, reinclusion
Ankylosis etiology factors
Disturbed local metabolism, trauma, genetically decreased PDL gap, disturbed root resorption/repair, genetic predisposition
Ankylosis most common tooth
Primary first molar, mandible
Ankylosis X-ray finding
Absence of periodontal ligament space
Dens invaginatus (dens in dente)
Deep surface invagination of the crown or root lined by enamel; two types: coronal and radicular
Coronal dens invaginatus most common tooth
Maxillary lateral incisor (also central incisors); maxillary predominance
Dens invaginatus Type I
Confined to the crown
Dens invaginatus Type II
Extends below CEJ and ends in a blind sac
Dens invaginatus Type III
Extends through the root and perforates the apical or lateral radicular area
Radicular dens invaginatus
Rare; due to inappropriate invagination of Hertwig's epithelial root sheath with a strip of enamel along the root surface
Taurodontism
Enlargement of body/pulp chamber of a multirooted tooth with apical displacement of pulpal floor and root bifurcation ("bull teeth")
Hypotaurodontism
Mild degree of taurodontism
Mesotaurodontism
Moderate degree of taurodontism
Hypertaurodontism
Severe degree of taurodontism
Taurodontism field effect
First molar least affected, increasing severity toward second and third molars
Hypercementosis
Non-neoplastic deposition of excessive cementum continuous with normal radicular cementum
Hypercementosis common tooth
Premolars; incidence increases with age
Hypercementosis X-ray finding
Thickening/blunting of root; PDL space is maintained around area of proliferation
Dilaceration
Abnormal angulation or bend in the root (or less often the crown) of a tooth
Dilaceration etiology
Trauma to tooth germ causing displacement/formation at abnormal angle; can also be secondary to cyst, odontoma, or supernumerary tooth
Dilaceration most affected teeth
Permanent maxillary and mandibular incisors
Cell fate determinants
Cell fate depends on origin, temporal-spatial history, and is determined by genes/environment (autonomous/intrinsic and non-autonomous/extrinsic factors)
Neural crest alone (before day 12)
When cultured alone, forms cartilage and neural crest tissue but NOT teeth or bone
Mandibular epithelium alone (before day 12)
When cultured alone, forms none of teeth, bone, cartilage, or neural crest -- cannot act alone
Neural crest + mandibular (1st arch) epithelium
Recombination that produces teeth, bone, cartilage, AND neural crest -- shows epithelium is instructive
Neural crest + limb (or 2nd arch) epithelium
Produces bone, cartilage, and neural crest but NOT teeth -- shows only first arch epithelium is odontogenic
Odontogenic epithelium + skin mesenchyme (after day 12)
Forms skin, not teeth -- after day 12 epithelium alone can't direct fate
Skin epithelium + odontogenic ectomesenchyme (after day 12)
Forms a tooth -- after day 12 the ectomesenchyme directs tooth development
Key conclusion of recombination experiments
Before E12, epithelium initiates odontogenesis; after E12, epithelium loses odontogenic potential and ectomesenchyme takes over directing tooth development
Three stages of tooth morphogenesis
Initiation (dental lamina) -> Morphogenesis (bud, cap) -> Differentiation (bell)
Hox genes
Family of homeobox-containing genes first discovered in Drosophila (antennapedia); encode homeodomain transcription factors that control segment identity and specialization
Antennapedia gene
Drosophila Hox gene; mutation causes a leg to grow in place of an antenna, showing Hox genes control segment/appendage identity
Homeodomain
~60 amino acid region of a ~400 amino acid transcription factor protein; binds specific DNA sequences to turn genes on/off, can regulate hundreds of genes
Homeobox
The DNA sequence within a Hox/homeobox gene that, when translated, produces the homeodomain protein region
Synteny
The conserved co-linear order of Hox genes across species during evolution
Co-linearity of Hox genes
The spatial order of Hox genes on the chromosome matches the anterior-to-posterior order of their expression in the embryo
HOX gene clusters in mammals
HOXA, HOXB, HOXC, HOXD -- mammalian equivalent of Drosophila Hox complex, arranged in chromosomal clusters
Msx
Homeobox gene family; originally identified in muscle development in Drosophila; in mammals, expressed in incisor-forming ectomesenchyme
Dlx
Homeobox gene family; originally identified in limb development in Drosophila; combines with Msx and Barx to pattern tooth type
Barx
Homeobox gene family; originally identified in retina development in Drosophila; expressed in molar-forming ectomesenchyme
Genes required to form the dental lamina
Msx1, Msx2, Dlx2, and Barx1 expressed in the overlap region of ectomesenchyme
Incisor identity gene code
Msx-1 and Msx-2 expression in ectomesenchyme
Canine identity gene code
Msx-1, Msx-2, and Dlx-2 expression (combination of anterior and posterior signals)
Molar identity gene code
Barx-1 and Dlx-2 expression
Odontogenic homeobox code model
Concept that combinatorial (overlapping) expression of homeobox genes (Barx1, Dlx1/2, Msx1, Msx2, Alx3) in ectomesenchyme before tooth germ initiation determines tooth type and pattern
FGF8
Growth factor from epithelium that induces/promotes tooth germ formation at future tooth positions
BMP4 (epithelial role)
Growth factor from epithelium that inhibits/restricts where tooth germs can form, helping mark tooth position
Pax9
Transcription factor expressed in ectomesenchyme; activated by FGF8, inhibited by BMP4; marks position of each tooth; ectomesenchyme depends on ligands for Pax9 early but becomes independent by bud stage
SHH (Sonic Hedgehog)
Secreted growth factor from dental epithelium; SHH-soaked beads applied to oral epithelium are sufficient to cause invaginations resembling tooth buds; involved throughout development with multiple roles including a concentration gradient later on
Reciprocal signaling stages
Initiation stage = epithelial signaling; Bud stage = mesenchymal signaling; Cap stage = enamel knot signaling
Epithelial signaling (initiation stage) genes
SHH, BMP4, FGF8 acting on dental mesenchyme; activate Msx1, Pax9, Ptc, Gli1, ActbA
BMP4 expression shift
BMP4 is expressed in dental lamina epithelium initially, then switches to the ectomesenchyme during the bud stage -- this switch indicates the shift in developmental control from epithelium to mesenchyme
Enamel knot
Specific inner enamel epithelium (IEE) signaling center at the cap stage; non-dividing epithelial cells at cusp tips that express p21, organize crown/cusp morphogenesis
Ectomesenchymal BMP4 (cap stage)
Required to induce formation of the enamel knot; enamel knot cells then express BMP4 and FGF4
p21
Cell cycle regulator expressed in enamel knot cytoplasm; causes primary and secondary enamel knot cells to stop dividing (non-dividing signaling center)
Primary enamel knot
First enamel knot to form at the cap stage; induces formation of secondary enamel knots and determines cusp pattern
Secondary enamel knots
Form later (bell stage), induced by the primary enamel knot; position determines multi-cusped tooth pattern; regulate dental papilla cells and laterally regulate IEE cells
Single-cusped vs multi-cusped teeth
Determined by whether only a primary enamel knot forms (single cusp) or primary knot induces secondary knots (multi-cusp)
Enamel knot signaling factors
BMP, FGF, SHH -- knot cells signal laterally to tell neighboring IEE cells not to become knot cells and to keep dividing
Cervical loop
Region of the enamel organ at the bell/late bell stage that becomes the root
Dental lamina
Initial thickening of oral epithelium that marks where teeth will form in each jaw
Dental placode
Localized thickening of epithelium marking the start of tooth initiation, associated with SHH expression
Odontogenic ectomesenchyme
Neural crest-derived mesenchyme beneath the epithelium that becomes competent to direct tooth development
Order of tooth structure formation (late stages)
Bell stage -> late bell stage -> root formation and eruption; produces enamel, dentin, pulp, root, and surrounding jaw bone
MSX gene function/product
Msh-like gene; transcription factor; located inside the nucleus
DLX gene function/product
Distaless homologue; transcription factor; located inside the nucleus
BARX gene function/product
BarH1 homologue; transcription factor; located inside the nucleus
BMP4 function/product
Bone morphogenetic protein; growth factor; located outside the cell
SHH function/product
Sonic hedgehog; growth factor; located outside the cell
FGF function/product
Fibroblast growth factor; growth factor; located outside the cell
Number of genes identified in tooth development
More than 90 genes encoding transcription factors and growth factors (signaling molecules)
Membrane thickness
5-8 nm thick