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Neuroscience-special topic > Exam 2: Neural Crest and Facial Disorders > Flashcards

Exam 2: Neural Crest and Facial Disorders Flashcards

1
Q

Neural crest cells have what 3 characteristics?

A

transient
migratory
multipotent

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2
Q

Neural crest cells are only found in

A

vertebrate embryos

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3
Q

neural crest cells important for embryo development, forming __ germ layer

A

4th germ layer

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4
Q

where are neural crest cells in head/face?

A

bones.
Pharyngeal arch
cranial morphogenesis

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5
Q

Mesoderm, ectoderm, endoderm, where are neural crest cells from?

A

Ectoderm, but special. They can turn into anything

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6
Q

How do neural crest cells migrate in the chicken embryo?

A

Dorsal –> Ventral

back to front

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7
Q

tools for cell fate mapping

A

1) vital dye labelling

2) lineage tracing

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8
Q

Describe vital dye labeling

A

using certain dye to label cells on back.

Culture embryos and see where labelled cells go. Then injection shows where cells migrate. Where you inject determines where they migrate.

Neural crest cells migrate based on where they originated.

HOWEVER, injections could be contaminated, need something more precise.

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9
Q

How do you use Cre-Loxp system for lineage trace?

A

Mate Cre with LacZ and stop allele

Offspring gets lacZ- recognizes certain color. Label cells without taking them out.

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10
Q

What do we see from Wnt1Cre tracing?

A

migrate extensively and go far.
Wnt1cre to skull

c-shape morphology in E8.5-15, in forebrain, midbrain, medial arch.

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11
Q

To study the origin of the scale: what labels? What results ?

A

mesp1-cre/R26R- label mesoderm cells

Wnt1-cre/R2R- label neural crest cells

Wnt- frontal
Mes- parietal

Conclusion? Scalp has 2 origins.

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12
Q

Neural crest derivatives

A

1) PNS
2) Endocrine and paraendocrine derivatives
3) Pigement cells
4) Facial cartilage and bone
5) connective tissue
many very specific cell types

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13
Q

neural crest development- 4 stages

A

1) Induction
2) Delamination
3) Migration
5) Specification

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14
Q

Induction

A

transformed from ectoderm into neural crest cell

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15
Q

Delamination

A

through epithelial-mesenchymal transformation (EMT)

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16
Q

Migration

A

toward ventral destination

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17
Q

Specification

A

multipotent neural crest cells differentiate into specific lineages

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18
Q

Where do neural crest cells originate from?

A

Ectoderm cells at the neural plate border

Edge of neural tube are neural crest cells, follow development from ectoderm to tube

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19
Q

Neural plate inductive signals

A

Bmps, Wnts, FGFs, Notch

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20
Q

Neural plate border specifiers,

A

Dll5, Pax3/7

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21
Q

Neural plate crest specifiers

A

FoxD3, Twist, Snail, Sox10

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22
Q

Neural crest effectors

A

MlTF, Rho GTPases, Kit, Ret

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23
Q

How do neural crest cells migrate

A

Dorsal –> Ventral
dorsal ventral pathway

dorsal lateral –> ventral lateral

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24
Q

What are they hypothesis for why D –> V

A

1) Growth Factor Gradients

2) Fibrolactin

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25
Q

Neural crest cells migration patterns: Cranial cells

A

Cranial cells spread SHEET (frontonasal prominence cells)

spread in STREMS (pharyngeal arch cells, including cardio and neural crest cells)

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26
Q

Neural crest cell migration patterns:

Trunk cells

A

dorsal root ganglia

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27
Q

Neural crest cell migration patterns:

Vagal and sacral neural crest cells

A

adrenal gland

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28
Q

Example of cardiac cells which are in cranial neural crest cells

A

top part of heart

pulmonary artery, arch, arota

29
Q

Trunk neural crest cells migrate in 1 of 2 pathways

A

1) Neurons and glial cells (ventromedial)
2) Melanocytes (dorsolateral)

Dorsolateral: both sides
Ventromedial: into the heart, determine glial cells

30
Q

Transplantation of trunk neural crest cells

A

radiolabeled donor chicken embryo.

neural crest cells transplant to another chick embryo.

Cells Black = neural crest

transplant donor –> host (chicken/quail)

31
Q

4 types of cell neural crest can make

**not: doesn’t mean these cells only from neural crest, just that it CAN make them

A

1) Mesenchymal (firoblasts, muscle, heart)
2) Neuronal cells
3) Secretory cells (chromaffin, parafollicular, calcitonin producing)
4) pigmented cells (melanocytes)

32
Q

Specification of neural crest:

Edn1 and Nppc

A

Cartilage/bone

33
Q

Specification of neural crest:

Neuregulin and Edn3

A

Schwann cells

34
Q

Specification of neural crest:

Neururin, Gdnf, Nts

A

neurons

35
Q

Specification of neural crest:

Edn3, alpha-MSH, Steel (steel and Edn3 together)

A

Melanocytes

36
Q

Why express each different gene?

A

lineages in different origins, different segments. Tell morphology under microscope. What makes 1 segment different from the others.

37
Q

____ is a crucial regulator of neural crest cell differentiation

A

Sox10

38
Q

Cranial neural crest cells become…

A

cartilage/bone
connective tissue
pigment
glial/peripheral neurons

39
Q

Trunk neural crest cells become…

A

pigment

glial/peripheral

40
Q

Vagal and Sacral neural crest cells become…

A

gut enteric ganglia

41
Q

how do neural crest genes decide the factor of derivatives?

A

interact with cofactors

42
Q

congenital craniofacial disorders

A

scalps have different patterns. We know the lines connected to different tracks and they have different patterns.

43
Q

craniofacial anomalies observed in __ human newborns with birth defects

A

3/4

44
Q

Occurrence of cleft lip/palate

A

1:700

45
Q

Occurrence of cleft craniosynostosis

A

1:2,500

46
Q

*****What is craniosynostosis?

A

premature closure of scalp

bones fuse.
Normally, babies born, scalp has several bones which separate so brain can grow.

Since bones not separated, grow and pressure builds. Effects development/speaking ability.

47
Q

cleft lip and palate

A

unilateral and bilateral
severe nose/upper lip split

1/1,000-1/700 world wide.

highest in Asians

Cleft lip/palate (45%), palate (35%), lip (20%)

48
Q

Causes of cleft lip and palate

A

genetic factors

environmental factors- mother with alcohol early, cigarette, prenatal nutrition, drug exposure, other environment factors

80%- only lip/palate, probably genetic

49
Q

mammalian craniofacial morphogenesis

A

feltro?- place where face fits like puzzle, meets at top line. why faces have nose groove.

Happens in womb between 2-3 months

50
Q

Upper lip formation in mammals

2 things

A

mnp and lnp form triangle region.

Two separate regions that fuse for upper lip.

when they get close, ectoderm cells covering migrate and undergo apoptosis. Forms integrated tissues.

*If cells don’t die- cleft lip

51
Q

What indicates that apoptosis is a pathway for the epithelia seam breaking down during upper lip fusion?

A

Expression of active Caspase 3 at the boundary between MNP/LNP

52
Q

___ regulates upper lip fusion via maintaining cell proliferation and activating apoptosis at LNP/MNP boundary.

A

Lrp6

activating apoptosis at LNP/MNP boundary

if cell does not meet in middle, cleft lip in mouse.

53
Q

Palatogenesis

A

formation of secondary palate.
upper lip: MxP and MNP

palate: Mx cells?

54
Q

critical steps of PALATE development

A

Growth
Elevation
Adhesion/fusion

Any step disruption = cleft palate

55
Q

Polarity of developing palate

A

anterior posterior
Lateral Medial Lateral

Barx1 is only lower part of palate. Certain polarity. Regulated by different genes, which interact and monitor.

56
Q

Polarity of Developing palate: The anterior palatal shelves elevate in a flip-up process,

A

flip-up process

57
Q

Polarity of Developing Palate: the posterior palatal shelves undergo

A

remodeling and reorientation through horizontal outgrowth from the medial wall

58
Q

Cellular process for palate fusion

A

1) apoptosis
2) extrusion of medial edge sema (MES) cells

this is what happens to ectoderm cells, but not in palate. instead, cells attach and migrate to the side (displacement) actin cables left in. Forms epithelial triangle which eventually disappears.

59
Q

Molecular control palate patterning

A

takes a number of genes

Epitheial cell development important

cells lose integrity and fuse.

60
Q

5 categories of palatal shelf defects that result in cleft palate

A

1) failure of palatal shelf formation
2) fusion with tongue or manidibel
3) failure of palatal shelf elevation
4) failure to meet following elevation
5) persistence of medial edge epithelium

61
Q

Calvaria development and craniosynostosis

A

premature suture fusion

1/2,500 births

62
Q

plagiocephally

A

20-30%
fused coronal suture
(one side)

63
Q

Sacphocephaly

A

40-60%

Fused saggital suture

64
Q

Trigoncephaly

A

fused metopic suture

65
Q

what causes types of craniosynostosis

A

mutations of Fgf2 cause autoactivation of receptor and abnormal differentiation of suture cells

66
Q

How do we rescue craniosynostosis mouse.

A

Fdfr2 mutation is functional mutation. Receptor can be activated without ligand binding.

Erk signal downstream.
Erk signal shows WT mutant rescuing.

67
Q

calvaria derived from what two origins?

A

neural crest

mesoderm

68
Q

Suture cells are ____ cells, which serve as ____

A

mesenchymal stem cells

serve as connection between different bones

69
Q

Development of homeostasis of calvaria is under rigorous control of

A

genetic factors, such as FgFr2

Neuroscience-special topic (8 decks)

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