First Author | Henry E | Year | 1987 |
Journal | Mouse News Lett | Volume | 78 |
Pages | 46 | Mgi Jnum | J:24743 |
Mgi Id | MGI:72472 | Citation | Henry E, et al. (1987) Claw paw, a new mutation affecting peripheral nerves. Mouse News Lett 78:46 |
abstractText | Full text of MNL contribution: 6. Claw paw, a new Mutation Affecting Peripheral Nerves. Claw paw (clp) is a new neurological mutant being studied with Dr. Eva Eicher at the Jackson Laboratory, where the mutation occurred in the C57BL/6J strain. The name is suggested because affected homozygotes show, at birth or immediately thereafter, a postural deformity of one or both forelimbs in which the fingers are flexed; the wrist and shoulder may also be flexed, and the elbow may be rotated inward (the "waiter's tip" posture). The joints can be moved passively, though much later in life the distal ones may become fixed, probably secondary to inactivity. Affected limbs are also weak. The most obvious defect on microscopic examination is a generalized delay in myelination, and a persistence of some axons and Schwann cells in the "promyelin" configuration throughout life, a state never seen in the normal peripheral nervous system. Peripheral axons appear to grow to a larger caliber before myelination begins in clp/clp mice than in +/? mice. In some animals, the ratio of myelin sheath thickness to axon caliber is reduced. The histopathological abnormalities are remarkably uniform throughout the entire peripheral nervous system and do not correlate well with the postural abnormalities and weakness, which are often much more severe in one forelimb than the other, and are always much more evident in the forelimbs than the hindlimbs. (E. Henry, Pfizer Central Research, Groton, CT, and Sidman). |