Holoprosencephaly

Finding genes that control mind and behavior

The highly sophisticated abilities of humans, such as memory, learning, cognition and thought, are achieved in the brain as a result of dramatic evolutionary development. Personality, preference, behavioral patterns, emotion ...

Nov 01, 2010
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New insight into most common forebrain malformation

St. Jude Children's Research Hospital scientists have identified one of the molecular mechanisms underlying the genetic brain malformation called holoprosencephaly (HPE). The findings not only yield insights into the most ...

Aug 11, 2008
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Holoprosencephaly (HPE, once known as arhinencephaly) is a cephalic disorder in which the prosencephalon (the forebrain of the embryo) fails to develop into two hemispheres. Normally, the forebrain is formed and the face begins to develop in the fifth and sixth weeks of human pregnancy. Hox genes, which guide placement of embryonic structures, fail to activate along the midline of the head, allowing structures that are normally paired on the left and right to merge. The condition also occurs in other species, as with Cy, the Cyclops kitten.

The condition can be mild or severe. According to the National Institute of Neurological Disorders and Stroke (NINDS), "in most cases of holoprosencephaly, the malformations are so severe that babies die before birth.

When the embryo's forebrain does not divide to form bilateral cerebral hemispheres (the left and right halves of the brain), it causes defects in the development of the face and in brain structure and function.

In less severe cases, babies are born with normal or near-normal brain development and facial deformities that may affect the eyes, nose, and upper lip.

This text uses material from Wikipedia licensed under CC BY-SA

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