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Early Embryonic Development #3

Dr. Johnson
Week 7, Monday 9/22/14
Lesson Objectives
1. Review gastrulation
2. Learn mechanism of neurulation
3. Learn neural crest derivatives
4. Describe etiology of DiGeorge syndrome
5. Define neural tube defects (NTDs)
6. Describe role of -fetoprotein in prenatal diagnosis of NTDs
7. Describe different kinds of NTDs
PART 1: Development of the CNS
A. Consequences of Gastrulation
1. There are three primary germ layers established:
a. Ectoderm forms outer surface and CNS
b. Mesoderm forms CT, muscle, CV system and GU system
c. Endoderm forms lining of gut tube
2. Basic body plan begins to unfold
B. Early CNS
1. In early development, deep tissues beneath superficial layer
on outer surface of embryo induce formation of mid-line neural plate
2. Neural plate expanded in cranial part-becomes brain
3. Neural plate narrow in caudal part-become spinal cord
4. Neural plate folds up into neural tube-neural crest leaves
neuroepithelium-neural tubes sinks beneath surface of the body's

C. Neural Crest I
1. Along dorsal midline of neural tube , neural crest cells exit the
neuroepithelium with an epithelial to mesenchymal transitionso this
means that the neural crest cells will lose their epithelial qualities and
will become more like Connective Tissues
2. These become widely dispersed throughout the body
3. They differentiate into a highly diverse, pluripotent group of
cells
D. Neural Crest II
1. These cells make important contributions to nervous system,
ex is Schwann cells which originate from neural crest cellsthey form
the myelin of the Peripheral Nervous System neurons
2. Also these cells, form the dorsal roote ganglia, some CAN
ganglia and numerous autonomic ganglianote that the dorsal root
ganglia consists of sensory neurons that originate in the body and
extend toward the CNS
3. These cells make gut intrinsic innervation-myenteric plexus
and submucosal plexus
4. NC make adrenal medulla
5. NC makes part of the meninges surrounding CNS
E. Neural Crest III
1. NC forms part of head mesenchyme, forming parts of facial
bones, muscles and ligaments and contributes to sclera of eye
2. NC makes odontoblasts, dentin producing cells of teeth
3. NC makes melanocytes, pigment producing cells in skin and
elsewhere in body

* Conotruncal septum is the opening between the pulmonary and


systemic sections of the heart during developmentmade of NC cells

F. DiGeorge Syndrome
1. This disorder 22q11.2 Syndrome (deletion in #22)
2. About 1/2500
3. Characterized by parathyroid aplasia (tetany and seizures),
thymic aplasia (decreased T cell function>opportunistic infections, ex
oral thrush, subtle craniofacial anomalies(short philtrum,
hypertelorism, microstomia), and cardiovascular anomalies
4. Results from abnormal migration and differentiation of neural
crest cells

G. Neuroepithelium
1. Primitive neural tube consists of highly proliferative,
pseudostratified epithelium
2. Stem cells divide rapidly by mitosis, one daughter cell
differentiates, other continues to divide
3. Some stem cells form neuroblasts, which differentiate into
neurons
4. Other stem cells form glioblasts, which differentiate into glia

H. Wall of Neural Tube Thickens


1. By repeated mitoses of stem cells, thickness of
neuroepithelium increases dramatically
2. Thickening is large in spinal cord
3. Thickening is even larger in brain

PART 2: The Brain


A. General Info
1. Cranial end of neural tube grows more rapidly than caudal end
2. Cranial end forms brain, caudal end forms spinal cord
3. Cranial end forms three primary brain vesicles,
prosencephalon(forebrain), mesencephalon(midbrain), and
rhombencephalon(hindbrain)

B. Neural Tube Defects (NTDs)


1. Common kind of congenital birth defects
2. Typically caused by failure of neural tube closure and/or
induction of bony neural arches of spinal cord
3. Range in severity from asymptomatic spina bifida occulta to
devastating and fatal anencephaly
C. Etiology of NTDs
1. Multifactorial etiology-some genetic and some environmentaldifficult to sort out
2. Higher incidence among Irish in Dublin
3. Slightly lower incidence in Irish Americans in Boston
4. Low incidence in African Americans in Boston
5. Prenatal folic acid supplementation reduces incidence about
80%

1. Spina Bifida Occulta: least severe, in this case there is bifida


vertebrae, there is no herniation so no tugging/pulling of
nerve tissue so skin forms instead of normal bony arch to
protect spinal cord will present with small hairy patch of skin
under which the bifida vertebrae is present
2. Meningocele shows bulging
3. Meningomyelocele the spinal cord forms in the CSF filled sac,
pulling on nervous tissue, severe neurodefecits
4. Neural plate doesnt close: Rachischisis so there is no neural
tube

* The picture on the left shows the spinal cord which is exposed.
Neuroectodem is degenerative so everything downstream of it will not
work properly
* The image on th right shows that the occipatl bone does not
form so large amount of nervous tissue herniating out

* This is an anencephalic fetusthere is no brain just


degenerated nervous tissue

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