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Biology subjects

Won, W.-J.

Publications and source records attributed to Won, W.-J..

3 recordsLinked to original sources

Amyloid-beta, alpha-synuclein and tau aggregated co-pathologies enhance neuropathology and neuroinflammation

Alzheimers (AD) and Parkinson disease (PD) pathology often co-occur. Amyloid-{beta} and phosphorylated tau are found in 30-50% of idiopathic PD cases, while -synuclein inclusions are present in 50% of AD cases. These co-pathologies are linked to increased mortality and earlier onset of cognitive decline. Immune activation is a hallmark of these neurodegenerative diseases, but current models primarily examine each pathology in isolation. How these co-pathologies drive inflammation and neuronal loss remains poorly understood. We therefore developed a mouse model combining tau, amyloid-{beta}, and -synuclein. We found that co-pathologies synergistically trigger an amplified neuroimmune response, with expanded populations of CD4+ and CD8+ tissue-resident memory T cells and CD68+ microglia, compared to single pathologies. These changes were abundant in the hippocampus and cortex, regions with elevated protein pathology load and enhanced neuronal loss. Our findings demonstrate that co-pathologies enhance proteinopathy and synergistically enhance immune activation and neurodegeneration, suggesting that combinatorial therapeutic strategies that target both co-pathologies and inflammation, may be disease modifying. SummaryWebster et al. demonstrate that co-occurring Alzheimers and Parkinson disease protein pathologies, common in cognitively impaired patient populations, amplify proteinopathy and synergistically enhance CNS neuroinflammatory responses and neurodegeneration. This work supports the need for combinatorial therapeutic strategies and positions neuroinflammation as an important link for co-pathology enhanced neurodegeneration.

neuroscience↗

Alpha-synuclein overexpression can drive microbiome dysbiosis in mice

Growing evidence indicates that persons living with Parkinson disease (PD), have a unique composition of indigenous gut microbes. Given the long prodromal or pre-diagnosed period, longitudinal studies of the human and rodent gut microbiome prior to symptomatic onset and for the duration of the disease period are currently lacking. PD is characterized in part by accumulation of the protein -synuclein (-syn) into insoluble aggregates, in both the central and enteric nervous systems. As such, a number of experimental rodent and non-human primate models of -syn overexpression recapitulate some of hallmark pathophysiologies of PD. These animal models provide an opportunity to assess how the gut microbiome changes with age under disease relevant conditions. Here, we used a transgenic mouse strain, the Thy1-hSYN "line 61" mice which over express wild-type human -syn to test how the gut microbiome composition responds in this model of PD pathology during aging. Using shotgun metagenomics, we find significant, age and genotype dependent bacterial taxa that become altered over age. We reveal that -syn overexpression can drive alterations to the gut microbiome composition and suggest that it limits the expansion of diversity through age. Given emerging data on potential contributions of the gut microbiome to PD pathologies, our data provide an experimental foundation to understand how the PD-associated microbiome may arise as a trigger or co-pathology to disease.

microbiology↗

Fc receptor-like 6 (FCRL6) defines pre-BCR dependent and independent pathways of natural antibody selection

B-1a cells produce natural antibodies (Abs) to neutralize pathogens and clear neo self-antigens, but the fundamental selection mechanisms that shape their polyreactive repertoires are poorly understood. Here, we identified a B cell progenitor subset defined by Fc receptor-like 6 (FCRL6) expression, harboring innate-like defense, migration, and differentiation properties conducive for natural Ab generation. Compared to FCRL6- pro B cells, the repressed mitotic, DNA damage repair, and signaling activity of FCRL6+ progenitors, yielded VH repertoires with biased distal Ighv segment accessibility, constrained diversity, and hydrophobic and charged CDR-H3 sequences. Beyond nascent autoreactivity, VH11 productivity, which predominates phosphatidylcholine-specific B-1a B cell receptors (BCRs), was higher for FCRL6+ cells as was pre-BCR formation, which was required for Myc induction and VH11, but not VH12, B-1a development. Thus, FCRL6 revealed unexpected heterogeneity in the developmental origins, regulation, and selection of natural Abs at the pre-BCR checkpoint with implications for autoimmunity and lymphoproliferative disorders.

immunology↗