Publications by authors named "R W Osgood"

Article Synopsis
  • Polycystic Kidney and Hepatic Disease 1-Like 1 (PKHD1L1) is a gene linked to autosomal recessive deafness (DFNB124) and is essential for the proper functioning of sensory hair cells in the cochlea, which are crucial for hearing.
  • The study investigates PKHD1L1 expression in mice throughout various developmental stages and its role in hair-cell bundle structure, revealing that absence of this gene leads to issues with stereocilia starting at 6 weeks of age.
  • PKHD1L1-deficient mice exhibit progressive hearing loss with age and are more vulnerable to permanent damage from noise exposure, highlighting the gene's importance in maintaining auditory function during development and against environmental
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Our sense of hearing is mediated by cochlear hair cells, of which there are two types organized in one row of inner hair cells and three rows of outer hair cells. Each cochlea contains 5-15 thousand terminally differentiated hair cells, and their survival is essential for hearing as they do not regenerate after insult. It is often desirable in hearing research to quantify the number of hair cells within cochlear samples, in both pathological conditions, and in response to treatment.

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Sensory hair cells of the cochlea are essential for hearing, relying on the mechanosensitive stereocilia bundle at their apical pole for their function. Polycystic Kidney and Hepatic Disease 1-Like 1 (PKHD1L1) is a stereocilia protein required for normal hearing in mice, and for the formation of the transient stereocilia surface coat, expressed during early postnatal development. While the function of the stereocilia coat remains unclear, growing evidence supports PKHD1L1 as a human deafness gene.

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Our sense of hearing is mediated by cochlear hair cells, localized within the sensory epithelium called the organ of Corti. There are two types of hair cells in the cochlea, which are organized in one row of inner hair cells and three rows of outer hair cells. Each cochlea contains a few thousands of hair cells, and their survival is essential for our perception of sound because they are terminally differentiated and do not regenerate after insult.

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The segmentation of individual instances of mitochondria from imaging datasets is informative, yet time-consuming to do by hand, sparking interest in developing automated algorithms using deep neural networks. Existing solutions for various segmentation tasks are largely optimized for one of two types of biomedical imaging: high resolution three-dimensional (whole neuron segmentation in volumetric electron microscopy datasets) or two-dimensional low resolution (whole cell segmentation of light microscopy images). The former requires consistently predictable boundaries to segment large structures, while the latter is boundary invariant but struggles with segmentation of large 3D objects without downscaling.

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