A5026324392- Amyotrophic Lateral Sclerosis Research
- Parkinson's Disease Mechanisms and Treatments
- Alzheimer's disease research and treatments
- Autophagy in Disease and Therapy
- Pluripotent Stem Cells Research
- Biomedical Ethics and Regulation
- Cerebral Palsy and Movement Disorders
- Neurogenesis and neuroplasticity mechanisms
- Adipose Tissue and Metabolism
- Science, Research, and Medicine
- Single-cell and spatial transcriptomics
- Aquaculture disease management and microbiota
- Genetic Neurodegenerative Diseases
- Mechanics and Biomechanics Studies
- Congenital Anomalies and Fetal Surgery
- Tissue Engineering and Regenerative Medicine
- Cellular transport and secretion
- Biofuel production and bioconversion
- Immune cells in cancer
- Genetics, Aging, and Longevity in Model Organisms
- Microtubule and mitosis dynamics
- Glioma Diagnosis and Treatment
- Epigenetics and DNA Methylation
- Cell Image Analysis Techniques
- Tuberous Sclerosis Complex Research
University of California, San Francisco
2015-2025
University Memory and Aging Center
2017-2021
Primary tauopathies are characterized neuropathologically by inclusions containing abnormal forms of the microtubule-associated protein tau (MAPT) and clinically diverse neuropsychiatric, cognitive, motor impairments. Autosomal dominant mutations in MAPT gene cause heterogeneous frontotemporal lobar degeneration with tauopathy (FTLD-Tau). Common rare variants increase risk for sporadic FTLD-Tau, including progressive supranuclear palsy (PSP) corticobasal (CBD). We generated a collection...
Mutations in the human progranulin gene resulting protein haploinsufficiency cause frontotemporal lobar degeneration with TDP-43 inclusions. Although progress has been made understanding normal functions of and TDP-43, molecular interactions between these proteins remain unclear. Progranulin is proteolytically processed into granulins, but role granulins pathogenesis neurodegenerative disease unknown. We used a <i>Caenorhabditis elegans</i> model neuronal proteinopathy to specifically...
TSC1/hamartin haploinsufficiency increases risk for tauopathy by promoting tau acetylation and accumulation.
Abstract Background Progranulin loss-of-function mutations are linked to frontotemporal lobar degeneration with TDP-43 positive inclusions (FTLD-TDP- Pgrn ). (PGRN) is an intracellular and secreted pro-protein that proteolytically cleaved into individual granulin peptides, which increasingly thought contribute FTLD-TDP- disease pathophysiology. Intracellular PGRN processed granulins in the endo-lysosomal compartments. Therefore, better understand conversion of granulins, we systematically...
Lipid dyshomeostasis and tau pathology are present in frontotemporal lobar degeneration (FTLD) Alzheimer's disease (AD). However, the relationship between lipid remains unclear. We report that GRAM Domain Containing 1B (GRAMD1B), a nonvesicular cholesterol transporter, is increased excitatory neurons of human neural organoids (HNOs) with MAPT R406W mutation. Human FTLD, AD cases, PS19 mice also have GRAMD1B expression. show overexpression increases levels free cholesterol, droplets, impairs...
Abstract Patient-derived cells hold great promise for precision medicine approaches in human health. Human dermal fibroblasts have been a major source of reprogramming and differentiating into specific cell types disease modeling. Postmortem dura mater has suggested as primary vitro modeling neurodegenerative diseases. Although fibroblast-like from mouse previously described, their utility direct differentiation protocols not fully established. In this study, derived postmortem are directly...
Progranulin (PGRN) is a tightly regulated, secreted glycoprotein involved in wide range of biological processes that tremendous interest to the scientific community due its involvement neoplastic, neurodevelopmental, and neurodegenerative diseases. In particular, progranulin haploinsufficiency leads frontotemporal dementia. While performing experiments with HIS-tagged recombinant human (rh) PGRN protein, we observed measurable depletion protein from solution adsorption onto polypropylene...
Abstract Background - Progranulin loss-of-function mutations are linked to frontotemporal lobar degeneration with TDP-43 positive inclusions (FTLD-TDP- Pgrn ). (PGRN) is an intracellular and secreted pro-protein that proteolytically cleaved into individual granulin peptides, which increasingly thought contribute FTLD-TDP- disease pathophysiology. Intracellular PGRN processed granulins in the endo-lysosomal compartments. Therefore, better understand conversion of granulins, we systematically...
Abstract The progressive failure of protein homeostasis is a hallmark aging and common feature in neurodegenerative disease. As the enzymes executing final stages autophagy, lysosomal proteases (or cathepsins) are key contributors to maintenance with age. Here, we identify cysteine-rich granulin peptides as new class regulators aspartyl protease activity. Granulins produced an age stress-dependent manner through cleavage disease protein, progranulin. Once liberated, granulins selectively...
Abstract Age-associated neurodegenerative disorders demonstrating tau-laden intracellular inclusions, including Alzheimer’s disease (AD), frontotemporal lobar degeneration (FTLD) and progressive supranuclear palsy (PSP), are collectively known as tauopathies. The vast majority of human tauopathies accumulate non-mutant tau rather than mutant forms the protein, yet cell animal models for lacking. We previously linked a monoallelic mutation in TSC1 gene to accumulation FTLD. Now, we have...
Protocol includes banking dural cells into cryovials for long term storage in liquid nitrogen.
Protocol includes splitting outgrowth of dural cell from a 6 well plate into one T75 flask for expansion.
Protocol includes feeding of T75 flask, T150 or the back up 6 well plate.
Isolation of cells from human dura mater. Protocol includes tissue freezing, cutting the with surgical tools, plating into a 6 well plate, placing coverslip on top tissue, and adding cell culture media.
Protocol includes splitting growing cell line from a T75 flask into two T150 flasks for expansion.
Protocols included in this collection describe cell isolation from human dura mater, culturing, and banking.
Protocol for feeding dural cells after outgrowth from tissue in a 6 well plate.
Detection of mycoplasma utilizing the bulldog-bio kit. Steps include, gDNA isolation from cells, nanodrop, PCR, and running an agarose gel to detect within a cell culture line. Image https://cellculturedish.com/cell-culture-basics-mycoplasma-101-a-practical-guide-to-prevention-detection-and-elimination-of-mycoplasma-contamination/
Abstract Background: Progranulin loss-of-function mutations are linked to frontotemporal lobar degeneration with TDP-43 positive inclusions (FTLD-TDP- Pgrn ). (PGRN) is an intracellular and secreted pro-protein that proteolytically cleaved into individual granulin peptides, which increasingly thought contribute FTLD-TDP- disease pathophysiology. Intracellular PGRN processed granulins in the endo-lysosomal compartments. Therefore, better understand conversion of granulins, we systematically...
Abstract Background - Progranulin loss-of-function mutations are linked to frontotemporal lobar degeneration with TDP-43 positive inclusions (FTLD-TDP- Pgrn ). (PGRN) is an intracellular and secreted pro-protein that proteolytically cleaved into individual granulin peptides, which increasingly thought contribute FTLD-TDP- disease pathophysiology. Intracellular PGRN processed granulins in the endo-lysosomal compartments. Therefore, better understand conversion of granulins, we systematically...
ABSTRACT Patient-derived cells hold great promise for precision medicine approaches in human health. Fibroblast have been a major source of reprogramming and differentiating into specific cell types disease modeling. Such can be isolated at various stages during life (presymptomatic, symptomatic, postmortem) thus potentially used to model different phases progression. In certain circumstances, however, tissues are not collected only postmortem the available fibroblasts. Fibroblasts cultured...