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Quittot, N.

Publications and source records attributed to Quittot, N..

3 recordsLinked to original sources

Dynamic conformational ensembles of soluble Tau encode neuronal toxicity prior to aggregation

Tau aggregation is a defining feature of Alzheimers disease and related tauopathies, yet the conformational states of Tau in neurons prior to aggregation remain poorly understood. Existing structural models are derived largely from fibrillar assemblies and provide limited insight into the dynamic, soluble Tau species that initiate pathology. Here, we combine hydrogen-deuterium exchange mass spectrometry with super-resolution imaging and neuronal models to define the conformational ensemble of soluble Tau under physiological and disease-relevant conditions. We show that soluble Tau populates distinct, dynamic conformations characterized by regional stabilization and long-range intramolecular interactions that are invisible to fibril-based structures. Disease-associated perturbations selectively remodel these conformational ensembles, exposing aggregation-prone regions and altering Tau subcellular organization in neurons. Notably, these Tau species inhibit axonal transport, which is essential for neuronal health, linking specific ensemble states to neuronal toxicity. These findings establish soluble Tau conformation as a dynamic, regulatable state that precedes aggregation and encodes disease relevance. By defining the structural logic of Tau before fibril formation, this work provides a framework for understanding early tauopathy mechanisms and for targeting Tau pathology at its earliest stages. SUMMARYTau pathology is a hallmark of Alzheimers disease (AD) and related dementias (ADRDs). Although Tau is often described as intrinsically disordered, it is a dynamic protein with distinct but poorly defined conformations. Here we conduct a systematic time-resolved structure-function analysis of normal and pathologic Tau, including hyperphosphorylated, mutant Tau, and posttranslational-modification-mimetic Tau. To characterize dynamic conformational changes of Tau, we combined state-of-the-art hydrogen deuterium exchange mass spectrometry with structured illumination microscopy, demonstrating a novel Tau-MT binding mode: "dynamic oscillation". To correlate Tau structure with neuronal function, we evaluated axonal transport as a sensitive readout of neuronal health. Many toxic Tau forms share a common signature of increased exposure of the N-terminal phosphate activating domain (PAD) in vitro and in vivo. Aberrant exposure of PAD correlates with Tau pathology and axonal transport defects. Tau phosphorylation at S262 alone is sufficient to alter Tau-microtubule interactions beyond R1-R4 motifs, globally changing Tau conformation, disrupting "dynamic oscillation" on MTs, and inhibiting axonal transport. Frontotemporal dementia-associated P301L-Tau remains associated with microtubules but also inhibits axonal transport. Our results reveal a well-defined conformation of soluble WT Tau in neurons and its highly dynamic interaction with microtubules, altered by AD/ADRD-Tau forms. Our multidisciplinary approach comprising biochemical manipulations, innovative MS tools, advanced microscopy, cellular assays, and mouse and human data pair Tau conformations with distinct neuronal functions and pathologies in health and disease.

neuroscience↗

The Ligand Preference of LRP1 is Regulated by O-glycans

Low-density lipoprotein receptor (LDLR) and LDLR-related proteins (LRPs) are endocytic receptors serving as essential physiological regulators of multiple processes including cholesterol clearance, protein reabsorption and neuronal protein trafficking. We originally discovered O-glycans in linkers of the ligand-binding domains of LDLR/LRP receptors and showed that these play critical roles for uptake of LDL by LDLR and albumin by LRP2. Remarkably, these linker O-glycans are introduced exclusively by GALNT11, one out of 20 polypeptide GalNAc-transferase isoenzymes. Here, we investigate the role of linker O-glycans on the large ([~]600 kDa) and widely expressed multiligand LRP1 receptor implicated in diseases including neuropathies. In genetically engineered cell models we activated endogenous full-coding LRP1 with and without O-glycans and demonstrate that while the uptake of certain ligands, such as RAP and ApoE, was unaffected by O-glycans, the uptake of the neurotoxic molecules tau and amyloid beta was altered and in opposite directions. This demonstrates that O-glycans in ligand-binding domains can differentially modulate ligand affinity and specificity of LRP1. Our findings highlight an overlooked regulatory mechanism of endocytic receptors and identify the ligand repertoire of LRP1 as being influenced by O-glycans, with potential implications for neurodegenerative disease.

molecular biology↗

Rare bioactive tau oligomers from Alzheimer brain support both templated misfolding and fibril formation

In Alzheimers disease, both classical neurofibrillary tangles, and diffusible, aqueous soluble (High Molecular Weight, or HMW) species are able to support templated misfolding. How these tau proteoforms relate is uncertain. Using sequential size exclusion and anion exchange chromatography, we fractionated the HMW tau population and found both seed competent, and seed not competent proteoforms. Super resolution, atomic force, and immunogold electron microscopy confirmed that the size and conformation of both bioactive and non-bioactive tau proteoforms are similar, with dimers, trimers, and tetramers predominating. The presence of surface phosphorylation correlates with seeding capacity. Bioactive tau at fMol concentrations can induce seeding in a reporter cell. The soluble bioactive species support aggregation of a truncated repeat domain tau construct into thioflavin T positive fibrils and retain seeding activity over serial amplification in vitro and in cellulo, whereas non-bioactive oligomeric species do not. Together, these findings indicate that oligomeric assembly is required but not sufficient for seeding; instead, specific biochemical attributes of a rare oligomeric tau subset confer self-propagating, prion-like templated misfolding.

neuroscience↗