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A perineurioma is a benign peripheral nerve sheath neoplasm thought to be derived from cells of the perineurium, the cellular sheathing of peripheral nerve fascicles. The histological, immunohistochemical (positive for epithelial membrane antigen, collagen type IV, laminin and vimentin but not S-100 protein) and ultrastructural features of a perineurioma form the basis for diagnosis. Two types of perineuriomas are recognized, intraneural and soft tissue (extraneural) forms. Sclerosing perineurioma is considered a variant of the soft tissue perineurioma. In all but one of the reported cases, this subtype presented as a solitary clinical lesion with a predilection for the fingers and palms of young adults. In 2002, Huang and Sung described a 16-year-old boy with one sclerosing perineurioma on each hand. The unusual case presented herein is a 21-year-old woman manifesting more than 30 cutaneous sclerosing perineuriomas on the hands and arms. This case is the most extensive presentation of sclerosing perineuriomas documented to date and extends the clinical presentation spectrum of these lesions.

HECT (homologous to the E6AP C terminus) ubiquitin ligases have diverse functions in eukaryotic cells. In screens for proteins that bind to the HECT ubiquitin ligase WWP1, we identified Spartin, which is also known as SPG20. This protein is truncated in a neurological disease, Troyer syndrome. In this study, we show that SPG20 associates with the surface of lipid droplets (LDs) and can regulate their size and number. SPG20 binds to another LD protein, TIP47, and both proteins compete with an additional LD protein, adipophilin/adipocyte differentiation-related protein, for occupancy of LDs. The mutant SPG20 present in Troyer syndrome does not possess these activities. Depletion of SPG20 using RNA interference increases the number and size of LDs when cells are fed with oleic acid. Binding of WWP1 to SPG20 and the consequent ubiquitin transfer remove SPG20 from LDs and reduce the levels of coexpressed SPG20. These experiments suggest functions for ubiquitin ligases and SPG20 in the regulation of LD turnover and potential pathological mechanisms in Troyer syndrome.

New neurons continue to be generated in the dentate gyrus throughout adulthood. Previous studies have shown that a significant proportion of new granule cells labeled with the thymidine analogue bromodeoxyuridine (BrdU) are lost from the adult dentate gyrus within 2 weeks. How long this loss continues and the extent to which it represents cell death, as opposed to dilution of label, is unclear. To address these questions, adult rats were injected with BrdU, and BrdU labeling in the dentate gyrus was compared at several survival time points. Double labeling with BrdU and the cell cycle marker Ki-67 showed that BrdU is detectable for up to 4 days in some cells that continue to divide, indicating that any decrease in the number of BrdU-labeled cells after 4 days is likely to reflect cell death rather than BrdU dilution. Death of new cells in the granule cell layer occurred at a steady rate between 6 and 28 days after labeling, resulting in loss of 50% of BrdU-labeled cells over this 22-day period. New granule cells that survived this first month lived for at least 5 additional months. In contrast, 26% of the granule cells labeled with BrdU at the peak of dentate gyrus development on postnatal day (P) 6 died between 1 and 6 months after labeling. These findings suggest that granule cells born during adulthood that become integrated into circuits and survive to maturity are very stable and may permanently replace granule cells born during development.