Evidence for impaired axonal regeneration in PMP22 duplication: studies in nerve xenografts.

Whether axonal regeneration in Charcot-Marie-Tooth (CMT) neuropathies is impaired has not been addressed in detail. Our studies in nude mice harboring xenografts from patients with different primary Schwann cell (SC) genetic defects suggested an intimate association between the onset of myelination and impairment in the growth capacity of nude mice axons engulfed by the mutant SCs. To assess the effects of peripheral myelin protein 22 (PMP22) gene duplication on the regeneration process, we conducted morphometric studies to generate temporal growth profiles of myelinated axons within the xenografts obtained from CMT1A patients and from healthy controls.

Intracellular processing and toxicity of the truncated androgen receptor: nuclear congophilia-associated cell death.

The pathogenesis of the selective motor neuron death in spinal bulbar muscular atrophy (SBMA) is not fully understood. Similar to observations with other mutant polyglutamine (poly Q) expanded proteins, truncated androgen receptor (AR) with expanded poly Q tract cause intracellular aggregates; however, the precise relationship between aggregates and disease pathogenesis is unresolved. In order to have a better understanding of the cellular processing and toxicity of the mutant AR, we focused on a short N-terminal portion of AR containing normal or expanded poly Q repeats, and have carried out biochemical, immunocytochemical, cytochemical and ultrastructural studies of BHK cells at different intervals after transfection.

[The ultrastructure of the capillaries and pericapillary area in the formations of the rat forebrain in hypokinesia].

Capillaries and pericapillary tissue were studied in central and lateral nuclei of amygdala, cingulate cortex, hippocampal fields CA1 and CA3 and sensomotor, cortex in rats, exposed to hypokinesia 40, 90 and 120 days lasted. Changes, growing more numerous and variable in proportion with the time of hypokinesia were found. The most essential changes were hypertrophy of glial elements with either decreased or increased functional activity.