The enervated (enrTg(MbpReg)36Pop) mutation resulted from random insertion into a gene of unknown function of a transgene comprising approximately 120 tandem copies of a 1.3 kb segment of the upstream regulatory region of the myelin basic protein gene (Mbp), ending 50 bp upstream of the transcription initiation codon (Kelly et al. 1994). Homozygous mutant mice are characterized by a gait abnormality, stiffened and/or splayed hind legs, and a hunched posture with some exhibiting kyphosis of the thoracic spine. These symptoms progress gradually to severe motor dysfunction. Skeletal muscles show necrosis and phagocytosis. Interactions between Schwann cells and axons are also disrupted. Although many peripheral axons are well myelinated, the nerve and nerve roots contain very large bundles of juxtaposed, bare axons, reminiscent of Schwann cell-axon interactions in early development. Nerve regeneration following injury is also impaired in homozyous mutant mice. There are fewer myelinated fibers per cross sectional area and thinner myelin sheaths surrounding regenerating axons in the nerves of homozygous mutant mice compared to wild type mice at 28 d after crush injury to the sciatic nerve. Abnormal Schwann cell-axon interactions remain in enervated mutant mice as evidenced by the relatively frequent ultrastructural finding of unmyelinated large diameter axons in the regenerating nerves.

These BPFD #36 mice carry an insertional mutation characterized by a gradual progression to severe motor dysfunction.


Typically mice are recovered in 12-16 weeks. Contact Customer Service to place an order or for more information.

<a target="_blank" href="https://www.jax.org/jax-mice-and-services/customer-support" rel="noreferrer">Contact Customer Service</a> for more information.