Variations in the Extensor Pollicis Brevis-Extensor Pollicis Longus Tendon Complex.

Despite several reports on the running of the extensor pollicis brevis (EPB) tendons, the classification of tendon insertions remains ununified due to differences in reports. This diversity in tendon patterning is attributed to the process of tendon development. In this study, we assessed the running of the EPB tendons of 44 cadaver hands fixed in ethanol/formalin in detail and examined the existing classification method. The specimens were obtained from 15 women and seven men, with an average age of 86 years. Consistent with previous reports, we observed a wide diversity in the running of the EPB tendons. Further, we found that EPB tendon insertions showed diverse variations in the proportion and running of fibers, making it difficult to classify them into independent patterns. It is speculated that the EPB tendon develops through a different process than that of the muscle body of the EPB and that the entire muscle-tendon module of the EPB is evolving. The diversity of the EPB tendons observed in this study may reflect the ongoing process of evolution. In clinical practice, a wide variation in the running of the EPB tendons should be considered.

Loss of Pax3 causes reduction of melanocytes in the developing mouse cochlea.

Cochlear melanocytes are intermediate cells in the stria vascularis that generate endocochlear potentials required for auditory function. Human PAX3 mutations cause Waardenburg syndrome and abnormalities of skin and retinal melanocytes, manifested as congenital hearing loss (~ 70%) and hypopigmentation of skin, hair and eyes. However, the underlying mechanism of hearing loss remains unclear. Cochlear melanocytes in the stria vascularis originated from Pax3-traced melanoblasts and Plp1-traced Schwann cell precursors, both of which derive from neural crest cells. Here, using a Pax3-Cre knock-in mouse that allows lineage tracing of Pax3-expressing cells and disruption of Pax3, we found that Pax3 deficiency causes foreshortened cochlea, malformed vestibular apparatus, and neural tube defects. Lineage tracing and in situ hybridization show that Pax3+ derivatives contribute to S100+, Kir4.1+ and Dct+ melanocytes (intermediate cells) in the developing stria vascularis, all of which are significantly diminished in Pax3 mutant animals. Taken together, these results suggest that Pax3 is required for the development of neural crest cell-derived cochlear melanocytes, whose absence may contribute to congenital hearing loss of Waardenburg syndrome in humans.