When this protective sheath is too thin, as
in hypomyelination, the electrical impulses can get lost between nerves and cause the nerves and corresponding muscles to malfunction.
Not exact matches
Although genetic deletion of BACE1 results
in abolished amyloid pathology
in AD model mice, it also results
in neurodevelopmental phenotypes such as
hypomyelination and synaptic loss, observed
in schizophrenia and autism - like phenotype.
Starting with transplants of human oligodendrocytes
in the late 1980s [40], and more recently with populations of human oligodendrocyte progenitor cells isolated from the developing or adult CNS, or from human embryonic stem cells, it has been possible to generate extensive myelination upon transplantation into spinal cord injury or into congenital mouse models of
hypomyelination [41]--[48].
Male Springer Spaniel puppies suffer most from
hypomyelination because the form of genetic transmission is different
in this breed.
Two types of
hypomyelination have been described to date, Shaking Pup Syndrome
in the English Springer Spaniel, and a second type found primarily
in the Weimaraner, but also reported
in a single study as being found
in Chow Chows.
Congenital
hypomyelination is seen as a familial / inherited disorder
in Springer Spaniels, Chow Chows, Weimaraners, and Bernese Mountain Dogs, usually with signs developing around 2 — 8 wk of age.
Use of trichlorfon during pregnancy can cause a congenital tremor
in piglets due to both cerebellar hypoplasia and
hypomyelination.