Nerve conduction velocity measurements reveal the functional deficit in ceramide galactosyl transferase-deficient (cgt-/-) mice.
Biochemical and ultrastructural studies of ceramide galactosyltransferase (CGT) in a CGT-deficient mouse line (cgt-/-) were complemented by nerve conduction velocity (NCV) measurements in motor nerves (sciatic nerve in the hind limbs) of wild type (wt) and cgt-/- mice. Stimulation and recording electrodes were adapted to the small size of developing mice during their myelination period. Motor NCVs in wt mice ranged between 16 and 26 m/s but in cgt-/- mice between 6 and 13 m/s, which corresponds to the conductance of unmyelinated peripheral nerves. These electrophysiologic data provide additional functional parameters to the neuropathology of a new form of a dysmyelinosis.
Haupt
Walter F
WF
Department of Neurology, University of Cologne, Joseph-Stelzmann-Str. 9 D-50924 Cologne, Germany. walter.haupt@medizin.uni-koeln.de
Stoffel
Wilhelm
W
D000200:Action Potentials / Q000235:genetics / Q000502:physiology; D000818:Animals; D002490:Central Nervous System / Q000473:pathology / Q000648:ultrastructure; D004558:Electric Stimulation / Q000379:methods; D004594:Electrophysiology / Q000379:methods; D005700:Galactosyltransferases / Q000172:deficiency* / Q000235:genetics / Q000378:metabolism; D051379:Mice; D008810:Mice, Inbred C57BL; D018345:Mice, Knockout; D008854:Microscopy, Electron; D051146:N-Acylsphingosine Galactosyltransferase; D009431:Neural Conduction / Q000235:genetics / Q000502:physiology*; D010525:Peripheral Nerves / Q000473:pathology / Q000648:ultrastructure; D011930:Reaction Time / Q000235:genetics / Q000502:physiology; D012584:Sciatic Nerve / Q000503:physiopathology* / Q000528:radiation effects / Q000648:ultrastructure
D005700:Galactosyltransferases; D051146:N-Acylsphingosine Galactosyltransferase