Embryo Project Encyclopedia Articles

Neurocristopathies are a class of pathologies in vertebrates,
including humans, that result from abnormal expression, migration,
differentiation, or death of neural crest cells (NCCs) during embryonic development. NCCs are cells
derived from the embryonic cellular structure called the neural crest.
Abnormal NCCs can cause a neurocristopathy by chemically affecting the
development of the non-NCC tissues around them. They can also affect the
development of NCC tissues, causing defective migration or
proliferation of the NCCs. There are many neurocristopathies
that affect many different types of systems. Some neurocristopathies
result in albinism (piebaldism) and cleft palate in humans. Various
pigment, skin, thyroid, and hearing disorders, craniofacial and heart
abnormalities, malfunctions of the digestive tract, and tumors can be
classified as neurocristopathies. This classification ties a variety of
disorders to one embryonic origin.

Early in the process of development, vertebrate embryos develop a fold on the neural plate where the neural and epidermal ectoderms meet, called the neural crest. The neural crest produces neural crest cells (NCCs), which become multiple different cell types and contribute to tissues and organs as an embryo develops. A few of the organs and tissues include peripheral and enteric (gastrointestinal) neurons and glia, pigment cells, cartilage and bone of the cranium and face, and smooth muscle. The diversity of NCCs that the neural crest produces has led researchers to propose the neural crest as a fourth germ layer, or one of the primary cellular structures in early embryos from which all adult tissues and organs arise. Furthermore, evolutionary biologists study the neural crest because it is a novel shared evolutionary character (synapomorphy) of all vertebrates.