Phenotypes Associated with This Genotype

Genotype
MGI:5803708

• Allelic Composition: Mbp^shi/Mbp^shi; Pmp2^{tm1.1(KOMP)Vlcg}/Pmp2^{tm1.1(KOMP)Vlcg}
• Genetic Background: involves: C3HeB/FeJ * C57BL/6 * C57BL/6NTac

• ♀: phenotype observed in females
• ♂: phenotype observed in males
• N: normal phenotype

behavior/neurological

limb grasping ( J:211344 )

• mice show severe hind limb clasping at P56

tremors ( J:211344 )

• mice develop a severe generalized tremor during locomotion by 2 weeks of age, indicating CNS defects

nervous system

abnormal myelin sheath morphology ( J:211344 )

• mice show a significant increase in the number of Schmidt-Lanterman incisures on sciatic nerve cross sections relative to control mice

• however, peripheral nerve myelin morphology is not significantly altered at P10 and P56 and the distribution of Na_v- and K_v1.2-channels in teased sciatic nerve fibers is normal at P56, indicating that the structure of the node of Ranvier is preserved

decreased nerve conduction velocity ( J:211344 )

• at P56, mice show a significant reduction in motor nerve conduction velocity that is similar to that observed in Mbp^shi homozygotes