A5031330009- Lysosomal Storage Disorders Research
- Cellular transport and secretion
- Sphingolipid Metabolism and Signaling
- Fungal and yeast genetics research
- Genomics and Chromatin Dynamics
- Autophagy in Disease and Therapy
- Calcium signaling and nucleotide metabolism
- Microtubule and mitosis dynamics
- Spectroscopy and Chemometric Analyses
- Biomedical Research and Pathophysiology
- Wnt/β-catenin signaling in development and cancer
- Metabolomics and Mass Spectrometry Studies
- Parkinson's Disease Mechanisms and Treatments
Stanford University
2021-2024
Research Network (United States)
2024
Dartmouth College
2017-2018
Lysosomes critically rely on bis(monoacylglycero)phosphate (BMP) to stimulate lipid catabolism, cholesterol homeostasis, and lysosomal function. Alterations in BMP levels monogenic complex neurodegeneration suggest an essential function human health. However, the site mechanism responsible for synthesis have been subject debate decades. Here, we report that Batten disease gene product CLN5 is elusive synthase (BMPS). BMPS-deficient cells exhibited a massive accumulation of precursor...
Batten disease, the most prevalent form of neurodegeneration in children, is caused by mutations CLN3 gene, which encodes a lysosomal transmembrane protein. loss leads to significant accumulation glycerophosphodiesters (GPDs), end products glycerophospholipid catabolism lysosome. Despite GPD storage being robustly observed upon loss, role GPDs neuropathology remains unclear. Here, we demonstrate that act as potent inhibitors lysosome using human cell lines and mouse models. Mechanistically,...
Bis(monoacylglycero)phosphate (BMP), a distinct anionic phospholipid predominantly found in late endosomes and lysosomes, plays pivotal role supporting lysosomal functions maintaining metabolic homeostasis. Its impaired function is associated with an array of disorders, notably neurodegenerative diseases. However, the identification quantitation BMP remains difficult due to its structural similarity isomer phosphatidylglycerol (PG), thus necessitating robust analytical methods for accurate...
Cell size control requires mechanisms that link cell growth with Cdk1 activity. In fission yeast, the protein kinase Cdr2 forms cortical nodes include inhibitor Wee1 along Wee1-inhibitory Cdr1. We investigated how inhibit during growth. Biochemical fractionation revealed were megadalton structures enriched for activated Cdr2, which increases in level interphase live-cell total internal reflection fluorescence microscopy videos, and Cdr1 remained constant at over time, but localized to short...
Mutations in lysosomal genes cause neurodegeneration and neurological storage disorders (LSDs). Despite their essential role brain homeostasis, the cell-type-specific composition function of lysosomes remain poorly understood. Here, we report a quantitative protein atlas lysosome from mouse neurons, astrocytes, oligodendrocytes, microglia. We identify dozens novel proteins reveal diversity across cell types. Notably, discovered SLC45A1, mutations which monogenic disease, as neuron-specific...
Abstract Cell size control requires mechanisms that link cell growth with Cdk1 activity. In fission yeast, the protein kinase Cdr2 forms cortical nodes include inhibitor Wee1, along Wee1-inhibitory Cdr1. We investigated how inhibit Wee1 during growth. Biochemical fractionation revealed were megadalton structures enriched for activated Cdr2, which increases in level interphase live-cell TIRF movies, and Cdr1 remained constant at over time, but localized to short bursts. Recruitment of...