odontogenesis: cellular and molecular Flashcards

1
Q

hereditary disease affecting bones and teeth

A

osteogenesis imperfecta (OI)

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2
Q

OI caused by mutations in COL1A1 or ____

A

type I collagen

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3
Q

OI is ____, in which one mutant allele is enough to cause OI phenotype

A

autosomal dominant (genotype and phenotype are the same)

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4
Q

need 2 mutant alleles to change the phenotype

A

autosomal recessive

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5
Q

with autosomal recessive, two ____ are necessary to contribute 2 mutant alleles to offspring

A

carriers

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6
Q

percent chance of offspring having disease in AD and AR

A

AD: 50%- usually shows up in every generation
AR: 25%- does not show up every generation

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7
Q

x-linked disease; mutations in AMELX gene on X chromosome

A

amelogenesis imperfecta (AI)

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8
Q

an affected father with AI passes on mutant X chromosome to _____ only

A

daughters

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9
Q

The process of a generalized cell becoming specialized for a job (changes in size, shape, products, activities, cell division, etc.)

A

cell differentiation

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10
Q

the process that initiates differentiation

A

induction

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11
Q

ability of a cell to receive and respond to a molecular signal

A

competence

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12
Q

the ultimate generalized cell

A

stem cell

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13
Q

in cell signaling, ____ chemical signal directs changes in target cells that express ____

A

secreted; receptors

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14
Q

Proteins that control whether genes will be transcribed into mRNA (to be translated into proteins)

A

transcription factors (TFs)

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15
Q

DLX3 stands for:

A

distal-less homeobox 3

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16
Q

DLX 3 regulates what 3 things

A
  1. hair follicle differentiation
  2. enamel genes
  3. bone
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17
Q

mutations in DLX3 lead to _____

A

TDO syndrome - tricho-dento-osseous syndrome (hair-teeth-bone)

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18
Q

ball of cells about day 3-4 of development

A

morula

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19
Q

hollow fluid-filled ball about day 5 of development

A

blastocyst

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20
Q

inner cell mass of the blastocyst that forms ALL tissues of the embryo- embryonic stem cells

A

embryoblast

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21
Q

other cells of the blastocyst ; the outer cell layer

A

trophoblast

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22
Q

___ are established in the blastocyst at about 13 days

A

axes

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23
Q

with axes established, the embryo is ____ with ___ being dorsal and ____ being ventral

A

bilaminar (2 layer); ectoderm; endoderm

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24
Q

ectoderm is the floor of the ____; it is dorsal and consists of ____ cells

A

amniotic cavity; columnar

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25
Q

endoderm is the roof of the _____; is is ventral and consists of ____ cells

A

second cavity (secondary yolk sac); cuboidal

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26
Q

conversion to a trilaminar embryo, occurs during the 3rd week

A

gastrulation

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27
Q

____ converge toward the midline to form the primitive streak

A

ectodermal cells

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28
Q

ectodermal cells migrate thru the primitive streak between ____ and _____ to form a new ____ layer

A

ectoderm; endoderm; mesoderm

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29
Q

cephalic (more rostral) migrating cells form the ____ to support the embryo

A

notochord

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30
Q

_____ remains bilayer of endoderm and ectoderm (NO mesoderm)

A

buccopharyngeal membrane

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31
Q

____ folding happening during week 4; when folding begins, the cardiac plate is ____ to the future buccopharyngeal membrane

A

rostro-caudal (front-back); rostral

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32
Q

_____ folding also occurs during week 4 and contributes to reorganization to 3D embryo or trilaminar disc

A

lateral (side-side)

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33
Q

one consequence of rostral-caudal folding

A

mouth

34
Q

_____ defines most rostral boundary of the primitive gut

A

buccopharyngeal membrane

35
Q

the buccopharyngeal membrane breaks down to allow ____ to communicate with the ____

A

stomatodeum (primitive oral cavity); foregut

36
Q

NCCs are induced to undergo _____ transformation

A

epithelial-mesenchymal

37
Q

skeletal bones come from ____

A

mesoderm

38
Q

during brain development, the ____ expands as:

A

neural tube expands as forebrain, midbrain, hindbrain

39
Q

the hindbrain forms ____ _____ or bulges, which define the origins of distinct populations of NCCs

A

8 rhombomeres

40
Q

NCCs from midbrain and rhombomeres 1 and 2 contribute to ____

A

branchial arch 1

41
Q

NCCs from rhombomeres greater than/equal to 3 express _____, ancient rostral-caudal patterning genes that define body segments

A

Hox TFs

42
Q

NCCs that migrate to the face and branchial arch 1 are ____

A

Hox-free

43
Q

all TFs include a ____ that allows them to interact with genes

A

DNA-binding domain

44
Q

controls the formation of legs during development

A

HOM-C Homeobox gene

45
Q

loss of function of HOM-C Homeobox gene causes:

A

legs–> antennae (loss of body patterning)

46
Q

gain of function of HOM-C Homeobox gene causes:

A

antennae –> legs (ectopic expression)

47
Q

what patterning TFs direct craniofacial development?

A

Otx2: orthodenticle homeobox 2
Msx: muscle segment homeobox
Dlx: distal-less homeobox
Barx: BarH-like homeobox

48
Q

NCCs contribute to the ____ in the branchial arches

A

mesoderm

49
Q

arches are external bumps which are internally, a mix of ___ and ____

A

NCCs and mesoderm

50
Q

the oral cavity is lined with ____ while the rest of the digestive tract is lined with ____

A

ectoderm; endoderm

51
Q

branchial arch 1 gives rise to:

A
  1. maxilla and mandible

2. meckel’s cartilage portion

52
Q

meckel’s cartilage gives rise to:

A
  • malleus and incus of middle ear
  • sphenomalleolar ligament
  • sphenomandibular ligament
53
Q

branchial groove/cleft 1 gives rise to:

A

external auditory meatus

54
Q

pouch 1 gives rise to:

A

tympanic membrane
tympanic cavity
mastoid antrum
eustachian tube

55
Q

Dlx1/2 mutant mice show altered ____ and lack of _____

A

craniofacial morphology; maxillary molars

56
Q

Dlx1/2 affects ____ BA1 or the _____

A

proximal; maxillary process

57
Q

disease caused by failure/impairment of NCC migration to the facial region; causes underdevelopment of craniofacial region and mandible

A

Treacher-Collins syndrome aka mandibulofacial dysostosis

58
Q

_____ interactions drive tooth formation

A

epithelial-mesenchymal

59
Q

signaling in odontogenesis is described as ____, ____, and _____

A

reciprocal, reiterative, sequential

60
Q

which tissue holds the odontogenic potential during initiation stage?

A

epithelium first, then mesenchyme

61
Q

morphogenesis begins during the ____ stage of odontogenesis

A

cap

62
Q

non-dividing enamel organ cells in the cap stage; expresses numerous signaling molecules; directs proliferation of surrounding epithelial cells

A

primary enamel knot

63
Q

2-3 cell layers thick, adjacent to the inner enamel epithelium (IEE)

A

stratum intermedium (SI)

64
Q

contains star-shaped cells with lots of space in between them

A

stellate reticulum (SR)

65
Q

non-dividing enamel organ cells in bell stage, appearing at sites of cusps (NOT in incisors); express signaling molecules; direct proliferation of surrounding epithelial cells

A

secondary enamel knot(s)

66
Q

secondary enamel knot(s) stimulate terminal differentiation of _____ to begin _____ (always begins at cusp tips)

A

odontoblasts; dentinogenesis

67
Q

in the late bell stage, the dental papilla forms ____ and the inner enamel epithelium forms _____

A

odontoblasts; ameloblasts

68
Q

autosomal dominant mutations in PAX9 transcription factor (expressed in dental mesenchyme early in development)

A

oligodontia

69
Q

most oligodontia affected individuals are missing maxillary and mandibular _____

A

2nd and 3rd molars

70
Q

autosomal dominant mutations in RUNX2 transcription factor, expressed in dental mesenchyme; causes supernumerary primary teeth

A

hyperdontia

71
Q

RUNX2 probably ____ regulates tooth-initating signal like ____

A

negatively; Wnt

72
Q

constitutively active Wnt signaling in mouse caused:

A
  • supernumerary teeth
  • ectopic enamel knots
  • numerous and malformed teeth
73
Q

embryonic stem cells are _____ and have the ability to differentiate into all 3 germ layers and divide indefinitely

A

pluripotent

74
Q

adult (postnatal) stem cells are _____ and have the ability to differentiate within limits; they divide _____

A

multipotent; asymmetrically (not indefinitely)

75
Q

dental stem cells have several populations of _____ stem cells

A

adult/postnatal

76
Q

Absence of all primary or secondary teeth; tooth agenesis

A

anodontia

77
Q

6 or more missing teeth

A

oligodontia

78
Q

1-5 missing teeth

A

hypodontia

79
Q

more than the normal numbers of teeth (supernumerary teeth)

A

hyperdontia

80
Q

dental stem cells can be from what locations?

A

dental pulp, periodontal ligament (PDL), dental follicle, apical papilla