Dentinogenesis and Inherited Dentin Defects Flashcards

(81 cards)

1
Q

What is Dentin by volume

A

50% mineral

30% organic matrix

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2
Q

what makes up the Organic matrix of Dentin by weight

A

90% Collagen
90% of non-collagen is Derived from DSPP (Dentin Sialophosphoportein)
-also: dentin matrix protein, osteonectin, bone sialoprotein, osteopontin, proteoglycans…

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3
Q

what do Odontoblasts differentiate from

A

Dental papilla cells

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4
Q

A long cytoplasmic extension encased in dentin

A

Odontoblastic process

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5
Q

Rate of Dentin deposition

A

4 micrometers per days

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6
Q

How is Dentin deposited as

A

Incrementally as lines of Owen (or Von ebner)

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7
Q

what does the pulp chamber and root canal shrink in size over life

A

Dentin formation occures throughout life

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8
Q

layers of the pulp from outisde to insde

A

Odontoblastic layer
CEll-free zone
Cell-rich zone
Pulper core

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9
Q

what is found in the cell-free zone

A

Nerve and capillary plexus

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10
Q

what is found in the cell rich zone

A

Extensive vascular system

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11
Q

how do Lines of Von ebner grow

A

Grow 1 day at a time horizontal and at a right angle to dentinal tubules

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12
Q

what do Lines of von ebner form

A

Accentuated lines due to disturbances in mineralization

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13
Q

why is Dentin sensitive

A

By odontoblast processes

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14
Q

where does more terminal branching of dentin tubules occure

A

In root dentin (more than in coronal dnetin)

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15
Q

Shape of Dentin tubules in the crown and the root

A

Crown: S shaped
Root: Strait

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16
Q

size of dentinal tubules

A

1-3 micrometers in diameter

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17
Q

does the crown or root have more dentin tubules

A

More in the crown

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18
Q

are there more dnetin tubles near the pulp or the surface

A

More near the pulp

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19
Q

what is Predentin

A

The fibrillar organic matrix before its calcification

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20
Q

what is primary dnetin

A

Dentin made during tooth formation

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21
Q

what type of dentin makes up the tooth

A

primary dentin

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22
Q

other name for Primary Dnetin

A

Circumpulpal dentin

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23
Q

the outer layer of primary dnetin

A

Mantle dentin

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24
Q

when is secondary dentin formed

A

By the slow continual deposition of dentin by odontoblasts following the completetion of the roots

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25
what happens the change of primary and secondary dentin
tubular structures is continuous but changes direction
26
why does pulp recession occure
Deposotion of secondary dentin is fastest on the roof and floor of the pulp chamber
27
why does Tertiary dentin form
Local reaction to attrition, caries, or dental restoration
28
what determines the architecture (quality) of tertiary dentin
Depends on the intensity and duration of the stimulus
29
Tubules present in the dentin
Continuous Sparse Irregular Absent
30
Types of Tertiary dentin
Reactionary Dentin | Reparative Dentin
31
what formes Reactionary dentin
Preexisting odontoblasts
32
what forms Reparative dentin
Newly differentiated odontoblast-like cells
33
When Newly differentiated odontoblast-like cells are included in the dentin
Osteodentin
34
what is Sclerotic Dentin
Translucent dentin due to calcifaction of the dentinal tubules as a result of injury or aging (also called transparent dentin)
35
why does Globular mineralization of dentin occur
High rates of Dentin Deposition leading to an irregular mineralization front
36
why does Linear Mineralization of dentin occur
Slow rates of deposition leadding to more uniform
37
when globular zones of mineral fail to fuse into a homogeneous mass due to hypomineralized dentin
Interglobular dentin
38
what is found in interglobular dentin
Dentin tubules pass through but no peritubular dentin is present
39
dentin that surrounds the dentinal tubules
Peritubular dentin
40
Organic and innorganic parts of PEritubular dentin
Low in Collagen Higher in DSPP (Dentin sialophosphoprotein) higher mineralization than intertubular dentin
41
HArdness of Intertubular dentin at the DEJ vs Pulp
DEJ is 4x as hard
42
Peritubular dentin hardness at the DEJ and Pulp
equal
43
Peritubular dentin vs Intertubular dentin at the pulp
PTD is 20x harder
44
Peritubular dentin vs Intertubular dentin at the DEJ
PTD is 5x harder
45
does Dentin or enamel mineralize first
Dentin is first
46
what is DGI type I
Dental phenotype in persons with Osteogenesis imperfected
47
what are thy symptomes of DGI type I
Teeth show marked discoloration and attrition in both deciduous and permanent dentition
48
what happens to the Pulp of DGI type I
Obliterated before or soon after tooth eruption
49
what mutations cuase osteogenesis imperfected
Mutation in the COL1A1 and COL1A2 genes
50
how is collagen formed
Two type I collagen alpha 1 chains fold with one alpha 2 chain to form a triple helix
51
Mutation of COL1A1 and COL1A2 lead to
``` Reduced Collagen (less severe phenotype) Prduce defective collagen (severe phenotype) ```
52
How is Osteogenesis imperfecta passed on
Autosomal-dominant
53
how does a patient radiograph look when they have DGI
Bulbous crowns Pulpal obliteration short roots Tooth impactions (both upper and second molars)
54
what mutation causes Dentinogeneisis imperfecta
utation in the dentin sialophosphorportein gene (DSPP)
55
what cells express DSPP
Preameloblasts, preodontoblasts, ODonotblasts
56
Strucutural domains of DSPP
DSP-proteoglycan | DPP- acic protein
57
Isoelectic point of DPP
1.0 (lots of phosphoserines)
58
mutations of DSPP cause
DD-II, DGI-II, DGI-III
59
what cleaves DSPP into DSP and DPP
Extracellular proteases
60
when missing DSPP what is shown in a radiograph
Enlarged pulp cavity Decreased mineral density Globular mineralized zones less mineralized dentin and more predentin
61
Types of Dentin Dysplasia
DD-I | DD-II
62
Types of Dentinogenesis imperfecta
DGI-I DGI-II DGI-III
63
where do mutations occure in DSPP to cause dentin defects
5' splicing | Framshift mutation at the 3' end (exon 5)
64
how id Dentinogeneis imperfecta type II different from OI
DGI-II only affects the teeth
65
Commonness of DGI-II
1 in 6k to 8k
66
what causes DGI-II
Mutations in DSPP
67
How do people with DGI-II look
Blue gray, amber brown and opalescent teeth
68
how do People with DGI-II look in radiographs
Teeth have bulbous crowns, Narrow roots | Pulp chambers and canals are gone or small
69
what happens to teeth of DGI-II from occlusal stress
Enamel may split from dentin
70
when was DGI-III found
Brandywine isolate from southern maryland and washing D.C.
71
how can DGI-III be differentiated from DGI-I and DGI-II
all appeaer similar except for DGI-III can have multiple pulp exposures in deciduous teeth
72
Radiographs of DGI-III show
Variation in appears (pulpal obliteration, normal, or shell teeth
73
commonness of Dentin Dysplasia Type I (DD-I)
1 in 100,000
74
How do the teeth look in DD-I
Both permanent and deciduous teeth are normal shape, form and color
75
How does DD-I look in a radiograph
Short roots with unusual mobility and early exfoliation Cresecent shaped pulpal remnant parallel to the CEJ in the permanent dentition and total pulpal obliteration in the deciduous dentition
76
How does the Teeth look in DD-II
Decidous teeth have deatures of DGI-II Permanent teeth are normal shape, form, and color Normal root length
77
What is found in the pulp cavities of DD-II
Thistle tube deformity | PUlp stones
78
What causes DGI-II, DGI-III, and DD-II
All about the same disease caused by dominant-negative effects (not haploinsufficiency)
79
what affects the severity of DSPP mutations
the effect of the DSPP mutation may lead to the different phenotypes (ie if its it DD-II, DGI-II, or DGI-III)
80
what does DD-II DGI-II DGI-III cause
DD-II: partial obliteration, mild discoloration DGI-II: Pulpal obliteration, Discoloration in both dentitions DGI-III: pulpal exposure, early abscess
81
How will dentin dissorders be organized in the future
No DGI-I, become named based on the disease phenotype | DD-II, DGI-II, and DGI-III all called DSPP mutations with considerations to severity