Figure 3.

Phenotype of adult PMP22-deficient mice. A–D, Distinct β-gal expression is evident in the ribs (A), aortic arch of the heart (B), brainstem deep nuclei (C, arrows), cranial nerve roots (C, arrowheads), and the peripheral roots of a spinal cord preparation (D). E, Behavioral phenotypes of 3-month-old littermates. Wild type (+/+) mice have splayed hindlimbs and relaxed forelimbs, whereas PMP22-deficient littermates (−/−) clasp their hindlimbs and forelimbs close to their body (top right) and display gait abnormalities (bottom). F, Teased nerve fibers from genotyped P21 littermates. Nerve fibers of PMP22−/− mice exhibit frequent tomacula (arrowhead). Magnification, 40×.