
Professor
Neurology
+1 415 734-2508
Steven Finkbeiner, MD, PhD is the Director of the Taube/Koret Center for Neurodegenerative Disease Research. He studies how inherited gene mutations lead to neuronal dysfunction and degeneration in ALS, frontotemporal dementia, Huntington’s and Parkinson's disease.
Publications
Intersecting impact of CAG repeat and huntingtin knockout in stem cell-derived cortical neurons.
Neurobiology of disease
Intersecting impact of CAG repeat and Huntingtin knockout in stem cell-derived cortical neurons.
bioRxiv : the preprint server for biology
Generalizable Prediction of Alzheimer Disease Pathologies with a Scalable Annotation Tool and an High-Accuracy Model.
medRxiv : the preprint server for health sciences
Huntingtin contains an ubiquitin-binding domain and regulates lysosomal targeting of mitochondrial and RNA-binding proteins.
Proceedings of the National Academy of Sciences of the United States of America
A dynamic in vitro model of Down syndrome neurogenesis with trisomy 21 gene dosage correction.
Science advances
Three dimensional and four dimensional live imaging to study mechanisms of progressive neurodegeneration.
The Journal of biological chemistry
Disease related changes in ATAC-seq of iPSC-derived motor neuron lines from ALS patients and controls.
Nature communications
A Trisomy 21-linked Hematopoietic Gene Variant in Microglia Confers Resilience in Human iPSC Models of Alzheimer's Disease.
bioRxiv : the preprint server for biology
A foundational atlas of autism protein interactions reveals molecular convergence.
bioRxiv : the preprint server for biology
Enhancing Spatial Transcriptomics Analysis by Integrating Image-Aware Deep Learning Methods.
Pacific Symposium on Biocomputing. Pacific Symposium on Biocomputing
A Comprehensive Enumeration of the Human Proteostasis Network. 2. Components of the Autophagy-Lysosome Pathway.
bioRxiv : the preprint server for biology
AutoComet: A fully automated algorithm to quickly and accurately analyze comet assays.
Redox biology
The Foundational Data Initiative for Parkinson Disease: Enabling efficient translation from genetic maps to mechanism.
Cell genomics
Frontotemporal Dementia Patient Neurons With Progranulin Deficiency Display Protein Dyshomeostasis.
bioRxiv : the preprint server for biology
NeuroLINCS Proteomics: Defining human-derived iPSC proteomes and protein signatures of pluripotency.
Scientific data
Fluorescently labeled nuclear morphology is highly informative of neurotoxicity.
Frontiers in toxicology
Huntington's disease iPSC models-using human patient cells to understand the pathology caused by expanded CAG repeats.
Faculty reviews
Genetic and Epigenetic Interplay Define Disease Onset and Severity in Repeat Diseases.
Frontiers in aging neuroscience
The E3 ligase TRIM1 ubiquitinates LRRK2 and controls its localization, degradation, and toxicity.
The Journal of cell biology
Answer ALS, a large-scale resource for sporadic and familial ALS combining clinical and multi-omics data from induced pluripotent cell lines.
Nature neuroscience
Single-cell transcriptomics of human iPSC differentiation dynamics reveal a core molecular network of Parkinson's disease.
Communications biology
Progranulin deficiency promotes neuroinflammation and neuron loss following toxin-induced injury.
The Journal of clinical investigation
Huntington's disease mouse models: unraveling the pathology caused by CAG repeat expansion.
Faculty reviews
Transcriptional signatures in iPSC-derived neurons are reproducible across labs when differentiation protocols are closely matched.
Stem cell research
Persistent mRNA localization defects and cell death in ALS neurons caused by transient cellular stress.
Cell reports
Genetically encoded cell-death indicators (GEDI) to detect an early irreversible commitment to neurodegeneration.
Nature communications
Longitudinal tracking of neuronal mitochondria delineates PINK1/Parkin-dependent mechanisms of mitochondrial recycling and degradation.
Science advances
ALS-associated mutation FUS-R521C causes DNA damage and RNA splicing defects.
The Journal of clinical investigation
Guidelines for the use and interpretation of assays for monitoring autophagy (4th edition)1.
Autophagy
Functional genomics, genetic risk profiling and cell phenotypes in neurodegenerative disease.
Neurobiology of disease
Publisher Correction: Deubiquitinase Usp12 functions noncatalytically to induce autophagy and confer neuroprotection in models of Huntington's disease.
Nature communications
The Autophagy Lysosomal Pathway and Neurodegeneration.
Cold Spring Harbor perspectives in biology
Author Correction: Deubiquitinase Usp12 functions noncatalytically to induce autophagy and confer neuroprotection in models of Huntington's disease.
Nature communications
Approaches to develop therapeutics to treat frontotemporal dementia.
Neuropharmacology
Cell death assays for neurodegenerative disease drug discovery.
Expert opinion on drug discovery
Automated four-dimensional long term imaging enables single cell tracking within organotypic brain slices to study neurodevelopment and degeneration.
Communications biology
Genetic Regulation of Neuronal Progranulin Reveals a Critical Role for the Autophagy-Lysosome Pathway.
The Journal of neuroscience : the official journal of the Society for Neuroscience
The Cellular NMD Pathway Restricts Zika Virus Infection and Is Targeted by the Viral Capsid Protein.
mBio
Publisher Correction: Deubiquitinase Usp12 functions noncatalytically to induce autophagy and confer neuroprotection in models of Huntington's disease.
Nature communications
Deubiquitinase Usp12 functions noncatalytically to induce autophagy and confer neuroprotection in models of Huntington's disease.
Nature communications
The Arc of cognition: Signaling cascades regulating Arc and implications for cognitive function and disease.
Seminars in cell & developmental biology
Assessing microscope image focus quality with deep learning.
BMC bioinformatics
iPS cells in the study of PD molecular pathogenesis.
Cell and tissue research
Huntingtin Inclusions Trigger Cellular Quiescence, Deactivate Apoptosis, and Lead to Delayed Necrosis.
Cell reports
Egocentric and allocentric visuospatial working memory in premotor Huntington's disease: A double dissociation with caudate and hippocampal volumes.
Neuropsychologia
The Receptor-interacting Serine/Threonine Protein Kinase 1 (RIPK1) Regulates Progranulin Levels.
The Journal of biological chemistry
Nrf2 mitigates LRRK2- and a-synuclein-induced neurodegeneration by modulating proteostasis.
Proceedings of the National Academy of Sciences of the United States of America
Targeting TEAD/YAP-transcription-dependent necrosis, TRIAD, ameliorates Huntington's disease pathology.
Human molecular genetics
Protein-RNA Networks Regulated by Normal and ALS-Associated Mutant HNRNPA2B1 in the Nervous System.
Neuron
Identification of hepta-histidine as a candidate drug for Huntington's disease by in silico-in vitro- in vivo-integrated screens of chemical libraries.
Scientific reports
Serine 421 regulates mutant huntingtin toxicity and clearance in mice.
The Journal of clinical investigation
Potential Transfer of Polyglutamine and CAG-Repeat RNA in Extracellular Vesicles in Huntington's Disease: Background and Evaluation in Cell Culture.
Cellular and molecular neurobiology
aB-Crystallin overexpression in astrocytes modulates the phenotype of the BACHD mouse model of Huntington's disease.
Human molecular genetics
Cross-species translation of the Morris maze for Alzheimer's disease.
The Journal of clinical investigation
Guidelines for the use and interpretation of assays for monitoring autophagy (3rd edition).
Autophagy
Clinical Trials in a Dish: The Potential of Pluripotent Stem Cells to Develop Therapies for Neurodegenerative Diseases.
Annual review of pharmacology and toxicology
Cytoplasmic sphingosine-1-phosphate pathway modulates neuronal autophagy.
Scientific reports
Sequence-Level Analysis of the Major European Huntington Disease Haplotype.
American journal of human genetics
Amelioration of toxicity in neuronal models of amyotrophic lateral sclerosis by hUPF1.
Proceedings of the National Academy of Sciences of the United States of America
Potential function for the Huntingtin protein as a scaffold for selective autophagy.
Proceedings of the National Academy of Sciences of the United States of America
Prioritized research recommendations from the National Institute of Neurological Disorders and Stroke Parkinson's Disease 2014 conference.
Annals of neurology
Proteostasis in striatal cells and selective neurodegeneration in Huntington's disease.
Frontiers in cellular neuroscience
Autophagy induction enhances TDP43 turnover and survival in neuronal ALS models.
Nature chemical biology
ALS-associated mutation FUS-R521C causes DNA damage and RNA splicing defects.
The Journal of clinical investigation
Mutant LRRK2 toxicity in neurons depends on LRRK2 levels and synuclein but not kinase activity or inclusion bodies.
The Journal of neuroscience : the official journal of the Society for Neuroscience
Executive functions in premanifest Huntington's disease.
Movement disorders : official journal of the Movement Disorder Society
Therapeutic modulation of eIF2a phosphorylation rescues TDP-43 toxicity in amyotrophic lateral sclerosis disease models.
Nature genetics
PML in the Brain: From Development to Degeneration.
Frontiers in oncology
Startups on the menu.
Nature biotechnology
Structure-guided inhibitor design expands the scope of analog-sensitive kinase technology.
ACS chemical biology
Proteostasis of polyglutamine varies among neurons and predicts neurodegeneration.
Nature chemical biology
Targeting H3K4 trimethylation in Huntington disease.
Proceedings of the National Academy of Sciences of the United States of America
Arc in the nucleus regulates PML-dependent GluA1 transcription and homeostatic plasticity.
Nature neuroscience
Comment on "Drug screening for ALS using patient-specific induced pluripotent stem cells".
Science translational medicine
NUB1 snubs huntingtin toxicity.
Nature neuroscience
Modeling Huntington's disease with induced pluripotent stem cells.
Molecular and cellular neurosciences
Astrocyte pathology and the absence of non-cell autonomy in an induced pluripotent stem cell model of TDP-43 proteinopathy.
Proceedings of the National Academy of Sciences of the United States of America
Longitudinal imaging and analysis of neurons expressing polyglutamine-expanded proteins.
Methods in molecular biology (Clifton, N.J.)
Inhibition of RNA lariat debranching enzyme suppresses TDP-43 toxicity in ALS disease models.
Nature genetics
Progranulin deficiency promotes neuroinflammation and neuron loss following toxin-induced injury.
The Journal of clinical investigation
Creation of an open-access, mutation-defined fibroblast resource for neurological disease research.
PloS one
Methylene blue modulates huntingtin aggregation intermediates and is protective in Huntington's disease models.
The Journal of neuroscience : the official journal of the Society for Neuroscience
Mutant induced pluripotent stem cell lines recapitulate aspects of TDP-43 proteinopathies and reveal cell-specific vulnerability.
Proceedings of the National Academy of Sciences of the United States of America
Identification of novel potentially toxic oligomers formed in vitro from mammalian-derived expanded huntingtin exon-1 protein.
The Journal of biological chemistry
Disease-associated polyglutamine stretches in monomeric huntingtin adopt a compact structure.
Journal of molecular biology
High-throughput screening in primary neurons.
Methods in enzymology
Protein aggregates in Huntington's disease.
Experimental neurology
Identifying polyglutamine protein species in situ that best predict neurodegeneration.
Nature chemical biology
Arc in synaptic plasticity: from gene to behavior.
Trends in neurosciences
Drug discovery in Parkinson's disease-Update and developments in the use of cellular models.
International journal of high throughput screening
Huntington's Disease.
Cold Spring Harbor perspectives in biology
Direct membrane association drives mitochondrial fission by the Parkinson disease-associated protein alpha-synuclein.
The Journal of biological chemistry
A compact beta model of huntingtin toxicity.
The Journal of biological chemistry
Bringing SOD1 into the fold.
Nature neuroscience
Arc regulates spine morphology and maintains network stability in vivo.
Proceedings of the National Academy of Sciences of the United States of America
A small-molecule scaffold induces autophagy in primary neurons and protects against toxicity in a Huntington disease model.
Proceedings of the National Academy of Sciences of the United States of America
Tau reduction prevents Abeta-induced defects in axonal transport.
Science (New York, N.Y.)
Quantitative relationships between huntingtin levels, polyglutamine length, inclusion body formation, and neuronal death provide novel insight into huntington's disease molecular pathogenesis.
The Journal of neuroscience : the official journal of the Society for Neuroscience
Conformation sensors that distinguish monomeric proteins from oligomers in live cells.
Chemistry & biology
Proteolysis of mutant huntingtin produces an exon 1 fragment that accumulates as an aggregated protein in neuronal nuclei in Huntington disease.
The Journal of biological chemistry
Cytoplasmic mislocalization of TDP-43 is toxic to neurons and enhanced by a mutation associated with familial amyotrophic lateral sclerosis.
The Journal of neuroscience : the official journal of the Society for Neuroscience
Identical oligomeric and fibrillar structures captured from the brains of R6/2 and knock-in mouse models of Huntington's disease.
Human molecular genetics
Pathogenic TARDBP mutations in amyotrophic lateral sclerosis and frontotemporal dementia: disease-associated pathways.
Reviews in the neurosciences
IKK phosphorylates Huntingtin and targets it for degradation by the proteasome and lysosome.
The Journal of cell biology
High-content screening of primary neurons: ready for prime time.
Current opinion in neurobiology
Monoclonal antibodies recognize distinct conformational epitopes formed by polyglutamine in a mutant huntingtin fragment.
The Journal of biological chemistry
Cytoplasmic retention of polyglutamine-expanded androgen receptor ameliorates disease via autophagy in a mouse model of spinal and bulbar muscular atrophy.
Human molecular genetics
The serum response factor and a putative novel transcription factor regulate expression of the immediate-early gene Arc/Arg3.1 in neurons.
The Journal of neuroscience : the official journal of the Society for Neuroscience
Single neuron ubiquitin-proteasome dynamics accompanying inclusion body formation in huntington disease.
The Journal of biological chemistry
The ubiquitin-proteasome pathway in Huntington's disease.
TheScientificWorldJournal
RNA decay back in play.
Nature neuroscience
NMDA and AMPA receptors: old channels, new tricks.
Trends in neurosciences
Disease-modifying pathways in neurodegeneration.
The Journal of neuroscience : the official journal of the Society for Neuroscience
Common threads in neurodegenerative disorders of aging.
Alzheimer's & dementia : the journal of the Alzheimer's Association
AMPA receptors regulate transcription of the plasticity-related immediate-early gene Arc.
Nature neuroscience
Splice variants of the NR1 subunit differentially induce NMDA receptor-dependent gene expression.
The Journal of neuroscience : the official journal of the Society for Neuroscience
Crystallization and diffraction properties of the Fab fragment of 3B5H10, an antibody specific for disease-causing polyglutamine stretches.
Acta crystallographica. Section F, Structural biology and crystallization communications
Automated microscope system for determining factors that predict neuronal fate.
Proceedings of the National Academy of Sciences of the United States of America
Using antibodies to analyze polyglutamine stretches.
Methods in molecular biology (Clifton, N.J.)
An evaluation of specificity in activity-dependent gene expression in neurons.
Progress in neurobiology
The IGF-1/Akt pathway is neuroprotective in Huntington's disease and involves Huntingtin phosphorylation by Akt.
Developmental cell
New roles for introns: sites of combinatorial regulation of Ca2+- and cyclic AMP-dependent gene transcription.
Science's STKE : signal transduction knowledge environment
Calcium regulation of the brain-derived neurotrophic factor gene.
Cellular and molecular life sciences : CMLS
Sending signals from the synapse to the nucleus: possible roles for CaMK, Ras/ERK, and SAPK pathways in the regulation of synaptic plasticity and neuronal growth.
Journal of neuroscience research
Ca2+ channel-regulated neuronal gene expression.
Journal of neurobiology
To fear or not to fear: what was the question? A potential role for Ras-GRF in memory.
BioEssays : news and reviews in molecular, cellular and developmental biology
Ca2+ influx regulates BDNF transcription by a CREB family transcription factor-dependent mechanism.
Neuron
Spatial features of calcium-regulated gene expression.
BioEssays : news and reviews in molecular, cellular and developmental biology
Ca(2+)-dependent routes to Ras: mechanisms for neuronal survival, differentiation, and plasticity?
Neuron
Glial calcium.
Glia
Calcium excitability and oscillations in suprachiasmatic nucleus neurons and glia in vitro.
The Journal of neuroscience : the official journal of the Society for Neuroscience
Ciprofloxacin interaction with sodium warfarin: a potentially dangerous side effect.
The American journal of emergency medicine
Endothelin induces a sustained rise in intracellular calcium in hippocampal astrocytes.
Neuroscience letters
Ca2+ waves in astrocytes.
Cell calcium
Glutamate induces calcium waves in cultured astrocytes: long-range glial signaling.
Science (New York, N.Y.)
Synthesis and characterization of a series of diarylguanidines that are noncompetitive N-methyl-D-aspartate receptor antagonists with neuroprotective properties.
Proceedings of the National Academy of Sciences of the United States of America
Therapeutic use of magnesium sulfate in selected cases of cerebral ischemia and seizure.
The New England journal of medicine
Applications of quantitative measurements for assessing glutamate neurotoxicity.
Proceedings of the National Academy of Sciences of the United States of America