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To investigate the structural role of glial fibrillary acidic protein (GFAP) in vivo, mice carrying a null mutation in GFAP were generated. In 7/14 mutant animals older than 18 months of age, hydrocephalus associated with white matter loss was detected. Mutant mice displayed abnormal myelination including the presence of actively myelinating(More)
The identification of connexin32 (Cx32) in myelinating Schwann cells and the association of Cx32 mutations with peripheral neuropathies suggest a functional role for gap junction proteins in the nerve. However, after nerve crush injury, Cx32 expression dramatically decreases in Schwann cells in the degenerating region, returning to control levels at newly(More)
Mutations in the gene encoding peripheral myelin protein 22 (PMP22) account for several inherited peripheral neuropathies in humans. We now show that transgenic mice expressing antisense PMP22 RNA exhibit modestly reduced levels of PMP22 together with a phenotype that is reminiscent of hereditary neuropathy with liability to pressure palsies (HNPP), a human(More)
OBJECTIVE To assess the sensitivities and specificities of velocity differences between median mixed nerve conduction across the wrist (Medmxpw) and (I) median mixed nerve conduction in the forearm (Medmxf) and (II) palm to D2 sensory conduction (MedpD2). DESIGN AND METHODS We prospectively studied 67 limbs of patients with clinically definite carpal(More)
We have previously described transgenic mice that harbor a dominant-negative antagonist of the POU protein SCIP (termed deltaSCIP). Native SCIP is expressed in promyelinating Schwann cells, where it represses expression of the myelin structural genes. The deltaSCIP mice display morphologic and behavioral abnormalities, including decreased axonal diameter,(More)
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