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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
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
* 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