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Dransfeld, U.

Publications and source records attributed to Dransfeld, U..

3 recordsLinked to original sources

Aggregate Deep Visual Proteomics reveals selective organization of α-synuclein inclusions

-Synuclein inclusions, the lesions typical of Parkinson's disease, have been interpreted as protective deposits, toxic species or failed degradation products, each predicting a different composition and correlating that enrichment with neuron dysfunction and eventual degeneration. Distinguishing them requires measuring what an inclusion concentrates relative to the cell that built it. Here we developed Aggregate Deep Visual Proteomics, which uses ultrasensitive mass spectrometry to profile laser-microdissected inclusions against the remainder of the same cell or a matched host. In HEK293 cells, iPSC-derived dopaminergic neurons and post-mortem patient brain, each inclusion concentrated a selective subset of its cell's proteome, independent of abundance and dominated by membrane, in neurons largely presynaptic. Inclusions took up whole assemblies, with subunits moving together in 421 of 438 complexes, and the V-ATPase alone was enriched in all three systems. Degradation cargo stayed with the receptors that route it, consistent with sequestration and failed degradation acting together. Neuritic and somatic inclusions differed, the former rich in UBQLN2 and the latter in intermediate filaments. In the cell line, phosphorylated -synuclein was tenfold higher in the inclusion than in the rest of the cell, with about one third of the rise already present before an inclusion was visible. Measured against its host, the composition of an inclusion becomes a readout of how it formed and a source of candidate markers and therapeutic targets.

biochemistry↗

Alpha-Synuclein aggregates inhibit ESCRT-III through sequestration and collateral degradation

-Synuclein aggregation is a hallmark of Parkinsons disease and related synucleinopathies. Extracellular -synuclein fibrils enter naive cells via endocytosis, followed by transit into the cytoplasm to seed endogenous -synuclein aggregation. Intracellular aggregates sequester numerous proteins, including subunits of the ESCRT-III system for endolysosome membrane repair, but the toxic effects of these events remain poorly understood. Using cellular models and in vitro reconstitution, we found that -synuclein fibrils interact with an -helix common to ESCRT-III proteins. This interaction results in sequestration of ESCRT-III subunits and triggers their proteasomal destruction in a process of "collateral degradation." These twin mechanisms deplete the available ESCRT-III pool, initiating a toxic feedback loop. The ensuing loss of ESCRT function compromises endolysosome membranes, thereby facilitating escape of aggregate seeds into the cytoplasm, which in turn increases aggregation and ESCRT-III sequestration. We suggest that collateral degradation and triggering of self-perpetuating systems could be general mechanisms of sequestration-induced proteotoxicity. HIGHLIGHTSO_LI-Synuclein fibrils bind and sequester ESCRT-III endolysosome repair proteins C_LIO_LIAn -helical segment common to ESCRT-III mediates fibril-selective interaction C_LIO_LIFibril-bound ESCRT-III subunits undergo "collateral degradation" via the proteasome C_LIO_LIESCRT-III depletion damages endolysosomes and worsens -synuclein aggregation C_LI

biochemistry↗

PLD3 and PLD4 synthesize S,S-BMP, a key phospholipid enabling lipid degradation in lysosomes

Bis(monoacylglycero)phosphate (BMP) is an abundant lysosomal phospholipid required for degradation of lipids, in particular gangliosides. Alterations in BMP levels are associated with neurodegenerative diseases. Unlike typical glycerophospholipids, lysosomal BMP has two chiral glycerol carbons in the S (rather than the R) stereo-conformation, protecting it from lysosomal degradation. How this unusual and yet crucial S,S-stereochemistry is achieved is unknown. Here we report that phospholipases D3 and D4 (PLD3 and PLD4) synthesize lysosomal S,S-BMP, with either enzyme catalyzing the critical glycerol stereo-inversion reaction in vitro. Deletion of PLD3 or PLD4 markedly reduced BMP levels in cells or in murine tissues where either enzyme is highly expressed (brain for PLD3; spleen for PLD4), leading to gangliosidosis and lysosomal abnormalities. PLD3 mutants associated with neurodegenerative diseases, including Alzheimers disease risk, diminished PLD3 catalytic activity. We conclude that PLD3/4 enzymes synthesize lysosomal S,S-BMP, a crucial lipid for maintaining brain health.

biochemistry↗