Publications by authors named "A Kornberg"
Background: New paradigms of diagnosis and treatment have changed the neurodegenerative trajectory for individuals with spinal muscular atrophy (SMA). Registries are a critical tool to provide real-world data on treatment patterns, their effects and health care provision within this evolving paradigm of care. This study aimed to evaluate the phenotypic and genotypic landscape, treatment patterns and health impact of SMA in Australia through the national registry.
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- Regulatory T (Treg) cells are important for tissue repair by providing growth factors like amphiregulin (Areg), but studying Areg-producing Treg cells has been challenging due to isolation difficulties.
- The researchers developed a new reporter mouse that allows detection of Areg expression in live cells and used it to analyze Treg cells in lung damage models, revealing distinct subpopulations.
- Their analysis showed that Areg-producing Treg cells are favored during damage, with functional differences highlighted, and they identified a novel mechanism, 4-1BB agonism, that promotes tissue repair activity.
Itaconic acid is an emerging platform chemical with extensive applications. Itaconic acid is currently produced by through biological fermentation. However, is a fungal pathogen that needs additional morphology controls, making itaconic acid production on industrial scale problematic.
View Article and Find Full Text PDFBackground: Progressive auditory dysfunction is common in patients with generalized neurodegenerative conditions, but clinicians currently lack the diagnostic tools to determine the location/degree of the pathology and, hence, to provide appropriate intervention. In this study, we present the white-matter microstructure measurements derived from a novel diffusion-weighted magnetic resonance imaging (dMRI) technique in a patient with axonal auditory neuropathy and consider the findings in relation to the auditory intervention outcomes.
Methods: We tracked the hearing changes in an adolescent with Riboflavin Transporter Deficiency (Type 2), evaluating the sound detection/discrimination, auditory evoked potentials, and both structural- and diffusion-weighted MRI findings over a 3-year period.