Exam III Review Flashcards
Weaver mutation
Specific behavioral defects related to abnormal function of the cerebellum; abnormally of the radial glial cells in cerebrum and consequent abnormal migration of the cells that normally form the granular layer of the cerebellar cortex.
Reeler mutation
Abnormal behavior and absence of normal cortical layers
What is the relation of reelin to reeler?
Reelin is defective in the reeler mutant; it is a stop signal for radial neuronal migration
What are the major derivatives of the diencephalon?
Epi thalamus, thalamus, hypothalamus
What are the 3 patterning centers in the forebrain and what signaling molecules do they use?
Rostral patterning center (FGF-8)
Dorsal patterning center (BMPs and Wnts)
Ventral patterning center (Shh)
The topographical arrangement of the myelencephalon is almost identical to the spinal cord. What is the major topographical change?
Pronounced expansion of the roof plate to from the thin roof over the 4th ventricle
Expression of which set of genes seems to be responsible for the differentiation of specific nuclei in the myelencephalon?
Hox genes
What type of transformation do the nueral crest cells undergo when they break free from the neural tube?
Epithelial - mesenchymal transformation
What factors are involved in the breaking free of neural crest to the neural tube?
Snail-1, snail-2 and Foxd3
Neural crest cells form many components of the nervous system, but they do NOT form what?
Motor neurons
Explain what happened in the following experiment: Transplant thoracic neural crest cells to the head region and vice versa
Neural crest cells from the trunk transplanted into head form most neural crest derivates, BUT cannot from cartilage or skeletal elements** even though this is normal for neural crest.
Circumpharyngeal neural crest arise from where?
The area of somites 1-7
What is the circumpharyngeal ridge?
And arc-shaped aggregation of cells passing behind the 6th pharyngeal arch which migrate ventrally and then cranially to provide a pathway for CN XII and related musculature
Where do most neural crest cells from somites 1-3 pass ?
Into pharyngeal arches 4 and 6 or form cardiac crescent
Neural crest cells from R4-7 make up what?
Make up the Vagal crest and form parasympathetic innervation for digestive tract
Vagal neural crest arises from ?
Circumpharyngeal crest and exit from levels of somites 1-7
Vagal neural crest cells are mostly associated with what?
Gut enteric system (parasympathetics)
What factor maintains the competence of neural crest cells to differentiate into autonomic neurons?
Mash-1
Waardenburg’s Syndrome - neurocristopathy of trunk and cranial NC
Caused by Pax3 mutations. Involves various combinations of pigmentation defects (commonly a white strip of hair), deafness, cleft palate, ocular hypertelorism. 1 type has hypoplasia of limb muscles.
CHARGE - neurocristopathy of trunk and cranial NC
Colomba, heart disease, atresia of nasal choanae, retardation of development, genital hypoplasia in males and anomalies of the ear.
DiGeorges’ syndrome - neurocristopathy of cranial NC
Associated w/ a deletion on chromosome 22; characterized by hypoplasia and reduced function of the thymus, thyroid, and parathyroid glands and cardiovascular defects, including persistent truncus arteriosis and abnormalities of the aortic arch
Neurofibromas - neurocristopathy tumor and proliferation defect
Neurofibromas are peripheral nerve tumors. A disease associated w/ them is Neurofibromatosis aka Von Recklinghausens which is a common genetic disease manifested by multiple tumors of NC origin. Occasional gigantism of limb/digit and various other conditions
The preplacodal region encircles what?
The cranial plate
The preplacodal region is induced by what?
Cranial mesoderm (and neural tube); involves activation of FGF pathway and inhibition of Wnt and BMP
As BMP concentration progresses, what is the order of differentiation?
Placodes –> neural crest –> neural plate
Review major inductive events chart **
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The optic stalk is a lateral evagination of the what?
Diencephalon - it enlarges distally to from the optic vesicle
The optic vesicle asymmetrically invaginates to from a cup-like structure with a groove along one side of it, what is the groove?
The choroid fissure - it is continuous w/ the groove in the optic stalk which is continuous with the posterior chamber of the eye
What artery uses the choroid fissure and optic stalk to pass into the posterior chamber of the eye?
Hyaloid artery
In mammals, absence of what gene results in early optic vesicle formation but then no more eye formation?
Pax6
High concentrations what what inhibit the expression of Pax6?
Shh
Low concentrations of Shh in distal optic stalk has what effect?
Permits expression of Pax6 and development of optic vesicle which will lead to formation of the retina
High concentrations of Shh in proximal optic stalk has what effect on eye formation?
Decreases expression of Pax6 and induces expression of Pax2
Function of Pax2
Will provide guidance of axons of ganglion cells from retina
Review Lens chart
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What factor is necessary for corneal induction?
Pax6
In cornea formation, the underlying lens vesicle induces the overlying ectoderm to do what?
Transfrom from bilayered epithelium (simple cuboidal basal layer + flattened periderm) to transparent multi-layered cornea
What forms the retina?
The invaginated optic cup forms both the pigmented and the neural retina
Pigmented retina
Outer thin layer of the optic cup after invagination occurs - this is the only pigmented part of the body that doesn’t need neural crest cells
What factors are necessary for the differentiation of the pigmented retina?
Otx2 stimulated by Shh
In the fixation of retinal polarity, what is fixed first?
The nasotemporal (anterior-posterior) axis
How is the nasotemporal axis established ?
By gradients composed of ephrins and receptors
What is fixed second in the fixation of retinal polarity?
The dorsoventral axis
The dorsoventral axis is established by what?
Antagonistic actions of Shh and BMP, along w/ ventropin, Tbx-5, Pax2, and Vax2.
Anopthalmos
Absence of an eye resulting from a mutation in RAX; very rare and can normally be attributed to lack of formation of the optics vesicle
Microphthalamos
Can range from an eyeball that is slightly smaller than normal to one that is almost vestigial; can be associated w/ a large # of genetic defects and various other causes including intrauterine infections. One of the most common components of rubella syndrome.
Colomba
Nonclosure of the choroid fissure of the iris during the 6th or 7th week results in its persistence
Rathke’s pouch
Evagination from the roof of the stomodeal ectoderm
Rathke’s pouch is the primordium for the ?
Anterior pituitary (adenohypophysis)
What cranial nerves arise from what arches?
Arch I: CN V - trigeminal
Arch II: CN VII - facial
Arch III: CN IX - Glossopharyngeal
Arch IV: CN X - vagus
Why does the first arch develop into jaws?
Because of the lack of Hox gene expression
What factor is necessary to keep arch 2 from differentiating into arch 1 and forming the jaws?
Hoxa-2
The neural tube is the basic signaling center in craniofacial development. It’s segmentation occurs as a result of what?
Hox gene expression - segmentation is carried over onto the neural crest cells giving rise to neural crest tissue
Pharyngeal arch derived musculature arises from?
Mesoderm from somitomeres
Pharyngeal arch mesenchyme that doesn’t consist of pharyngeal arch derived musculature is derived from?
Neural crest
Except for _______________, mesodermal structures of the pharyngeal arches are derived from neural crest.
Pharyngeal muscles
The Frontonasal ectodermal zone is induced by. ?
Shh from forebrain
What is the Frontonasal ectodermal zone?
An ectodermal signal center involving the establishment of a dorsoventral gradient of FGF-8 (more dorsal) and Shh (more ventral)
What factors are involved in the shaping of the tip of the snout?
Shh and FGF-8
What are the 3 facial primordia?
- Frontonasal prominence - unpaired
- Nasomedial processes
- Nasolateral processes
What are the primordia for the jaws and from which arch do they arise?
Maxillary processes
Mandibular processes
Meckel’s cartilage
Pharyngeal arch 1*
Maxillary processes consist of ?
Neural crest derived from forebraine and midbrain
Mandibular processes consists of ?
Neural crest derived from midbrain and hindbrain
Meckel’s cartilage
Slender, elongated cartilagenous rod that develops w/I the 1st arch. Derived from neural crest cells. The mandible forms around it.
What type of bone is the mandible?
Membrane bone
What are the derivatives of Meckel’s cartilage?
Articular and Quadrate bones
They’re cartilagenous bone
TMJ
Synovial joint w/ an articular disk. Articular surfaces are the temporal bone and the mandibular condyle.
Formation of the TMJ involved early expression of what?
Barx-1
Fate of the articular and Quadrate bones
Quadrate: moves to middle ear and becomes incus
Articular: moves to middle ear and become malleus
Stapes is another middle ear bone that is derived from which arch?
Arch II
When does the palate form?
Between 6 and 10 weeks of development
What does the palate divide?
The common oronasal cavity into nasal and oral cavity - they remain connection together in the adult as the pharynx (Oro and naso)
What are the primordia for the palate?
Median palatine process (unpaired) and
Lateral palatine process (paired)
What forms the primary palate aka the premaxilla
Median palatine process
What forms the secondary palate?
The lateral palatine process
Dental lamina
C-shaped bands of oral ectoderm overlying neural crest mesenchyme