Eric Huang, MD, PhD
Professor
Pathology
School of Medicine

Another major area of research in my lab focuses on the molecular mechanisms of neurodegenerative diseases, including frontotemporal dementia (FTD) and amyotrophic lateral sclerosis (ALS). In addition to our seminal work to investigate disease mechanisms in model organisms, we have made every effort to directly connect our research with human diseases. To this end, I have spearheaded the efforts to establish the UC Pediatric Neuropathology Consortium. The success of this endeavor is underscored by our extensive collaborations with colleagues within and outside UCSF, which lead to many important discoveries. Most importantly, these studies revealed previously unrecognized role of neural circuit dysfunctions in neurodevelopmental and neurodegenerative diseases, and re-define our approaches toward investigating the molecular and cellular pathways in these devastating diseases.

• Molecular and cellular dynamics of the developing human neocortex at single-cell resolution.
  bioRxiv : the preprint server for biology 2024 Wang L, Wang C, Moriano JA, Chen S, Zuo G, Cebrián-Silla A, Zhang S, Mukhtar T, Wang S, Song M, de Oliveira LG, Bi Q, Augustin JJ, Ge X, Paredes MF, Huang EJ, Alvarez-Buylla A, Duan X, Li J, …
• Comorbid neuropathology and atypical presentation of Alzheimer's disease.
  Alzheimer's & dementia (Amsterdam, Netherlands) 2024 Pina-Escudero SD, La Joie R, Spina S, Hwang JH, Miller ZA, Huang EJ, Grant H, Mundada NS, Boxer AL, Gorno-Tempini ML, Rosen HJ, Kramer JH, Miller BL, Seeley WW, Rabinovici GD, Grinberg LT
• Deconstructing Intratumoral Heterogeneity through Multiomic and Multiscale Analysis of Serial Sections.
  Cancers 2024 Schupp PG, Shelton SJ, Brody DJ, Eliscu R, Johnson BE, Mazor T, Kelley KW, Potts MB, McDermott MW, Huang EJ, Lim DA, Pieper RO, Berger MS, Costello JF, Phillips JJ, Oldham MC
• TMEM16F exacerbates tau pathology and mediates phosphatidylserine exposure in phospho-tau-burdened neurons.
  Proceedings of the National Academy of Sciences of the United States of America 2024 Zubia MV, Yong AJH, Holtz KM, Huang EJ, Jan YN, Jan LY
• Neuroinflammatory reactive astrocyte formation correlates with adverse outcomes in perinatal white matter injury.
  Glia 2024 Renz P, Steinfort M, Haesler V, Tscherrig V, Huang EJ, Chavali M, Liddelow S, Rowitch DH, Surbek D, Schoeberlein A, Brosius Lutz A
• Structural and molecular basis of choline uptake into the brain by FLVCR2.
  Nature 2024 Cater RJ, Mukherjee D, Gil-Iturbe E, Erramilli SK, Chen T, Koo K, Santander N, Reckers A, Kloss B, Gawda T, Choy BC, Zhang Z, Katewa A, Larpthaveesarp A, Huang EJ, Mooney SWJ, Clarke OB, Yee SW, …
• Deconstructing intratumoral heterogeneity through multiomic and multiscale analysis of serial sections.
  bioRxiv : the preprint server for biology 2024 Schupp PG, Shelton SJ, Brody DJ, Eliscu R, Johnson BE, Mazor T, Kelley KW, Potts MB, McDermott MW, Huang EJ, Lim DA, Pieper RO, Berger MS, Costello JF, Phillips JJ, Oldham MC
• Protracted neuronal recruitment in the temporal lobes of young children.
  Nature 2023 Nascimento MA, Biagiotti S, Herranz-Pérez V, Santiago S, Bueno R, Ye CJ, Abel TJ, Zhang Z, Rubio-Moll JS, Kriegstein AR, Yang Z, Garcia-Verdugo JM, Huang EJ, Alvarez-Buylla A, Sorrells SF
• NEMO reshapes the α-Synuclein aggregate interface and acts as an autophagy adapter by co-condensation with p62.
  Nature communications 2023 Furthmann N, Bader V, Angersbach L, Blusch A, Goel S, Sánchez-Vicente A, Krause LJ, Chaban SA, Grover P, Trinkaus VA, van Well EM, Jaugstetter M, Tschulik K, Damgaard RB, Saft C, Ellrichmann G, Gold R…
• Profiling human brain vascular cells using single-cell transcriptomics and organoids.
  Nature protocols 2023 Crouch EE, Diafos LN, Valenzuela EJ, Wedderburn-Pugh K, Birrueta JO, Caston J, Joseph T, Andrews MG, Bhaduri A, Huang EJ
• Non-synaptic function of the autism spectrum disorder-associated gene SYNGAP1 in cortical neurogenesis.
  Nature neuroscience 2023 Birtele M, Del Dosso A, Xu T, Nguyen T, Wilkinson B, Hosseini N, Nguyen S, Urenda JP, Knight G, Rojas C, Flores I, Atamian A, Moore R, Sharma R, Pirrotte P, Ashton RS, Huang EJ, Rumbaugh G, Coba MP, …
• Single-cell analysis of prenatal and postnatal human cortical development.
  Science (New York, N.Y.) 2023 Velmeshev D, Perez Y, Yan Z, Valencia JE, Castaneda-Castellanos DR, Wang L, Schirmer L, Mayer S, Wick B, Wang S, Nowakowski TJ, Paredes M, Huang EJ, Kriegstein AR
• A cross-species proteomic map reveals neoteny of human synapse development.
  Nature 2023 Wang L, Pang K, Zhou L, Cebrián-Silla A, González-Granero S, Wang S, Bi Q, White ML, Ho B, Li J, Li T, Perez Y, Huang EJ, Winkler EA, Paredes MF, Kovner R, Sestan N, Pollen AA, Liu P, Li J, Piao X, …
• Defining RNA oligonucleotides that reverse deleterious phase transitions of RNA-binding proteins with prion-like domains.
  bioRxiv : the preprint server for biology 2023 Guo L, Mann JR, Mauna JC, Copley KE, Wang H, Rubien JD, Odeh HM, Lin J, Lee BL, Ganser L, Robinson E, Kim KM, Murthy AC, Paul T, Portz B, Gleixner AM, Diaz Z, Carey JL, Smirnov A, Padilla G, Lavorando…
• Disentangling brain vasculature in neurogenesis and neurodegeneration using single-cell transcriptomics.
  Trends in neurosciences 2023 Crouch EE, Joseph T, Marsan E, Huang EJ
• Granulin loss of function in human mature brain organoids implicates astrocytes in TDP-43 pathology.
  Stem cell reports 2023 de Majo M, Koontz M, Marsan E, Salinas N, Ramsey A, Kuo YM, Seo K, Li H, Dräger N, Leng K, Gonzales SL, Kurnellas M, Miyaoka Y, Klim JR, Kampmann M, Ward ME, Huang EJ, Ullian EM
• Plastin 3 rescues BDNF-TrkB signaling in spinal muscular atrophy.
  The Journal of cell biology 2023 Ramsey A, Huang EJ
• Clinical and Neurophysiologic Phenotypes in Neonates With BRAT1 Encephalopathy.
  Neurology 2023 Carapancea E, Cornet MC, Milh M, De Cosmo L, Huang EJ, Granata T, Striano P, Ceulemans B, Stein A, Morris-Rosendahl D, Conti G, Mitra N, Raymond FL, Rowitch DH, Solazzi R, Vercellino F, De Liso P, D'…
• Meta-Analysis of the Test-Retest Repeatability of [18F]-Fluorodeoxyglucose Standardized Uptake Values: Implications for Assessment of Tumor Response.
  Clinical cancer research : an official journal of the American Association for Cancer Research 2023 Shankar LK, Huang E, Litiere S, Hoekstra OS, Schwartz L, Collette S, Boellaard R, Bogaerts J, Seymour L, deVries EGE
• Acid sphingomyelinase inhibition improves motor behavioral deficits and neuronal loss in an amyotrophic lateral sclerosis mouse model.
  BMB reports 2022 Choi BJ, Park KH, Park MH, Huang EJ, Kim SH, Bae JS, Jin HK
• Developmental dynamics of RNA translation in the human brain.
  Nature neuroscience 2022 Duffy EE, Finander B, Choi G, Carter AC, Pritisanac I, Alam A, Luria V, Karger A, Phu W, Sherman MA, Assad EG, Pajarillo N, Khitun A, Crouch EE, Ganesh S, Chen J, Berger B, Sestan N, O'Donnell-Luria A…
• Endosomal recycling defects link Huntington's disease with McLeod syndrome.
  The Journal of cell biology 2022 Marsan E, Huang EJ
• Secretory autophagy maintains proteostasis upon lysosome inhibition.
  The Journal of cell biology 2022 Solvik TA, Nguyen TA, Tony Lin YH, Marsh T, Huang EJ, Wiita AP, Debnath J, Leidal AM
• Comment on "Impact of neurodegenerative diseases on human adult hippocampal neurogenesis".
  Science (New York, N.Y.) 2022 Alvarez-Buylla A, Cebrian-Silla A, Sorrells SF, Nascimento MA, Paredes MF, Garcia-Verdugo JM, Yang Z, Huang EJ
• Progranulin deficiency promotes neuroinflammation and neuron loss following toxin-induced injury.
  The Journal of clinical investigation 2022 Martens LH, Zhang J, Barmada SJ, Zhou P, Kamiya S, Sun B, Min SW, Gan L, Finkbeiner S, Huang EJ, Jr RVF
• Atypical Young-onset Dementia in Cerebral Thromboangiitis Obliterans: A Case Report.
  Alzheimer disease and associated disorders 2021 Kim EJ, Jung NY, Lee MJ, Pak K, Lee JH, Lee YM, Shin JH, Ko JK, Lee JM, Yoon JA, Hwang C, Choi KU, Huang EJ, Huh GY
• Comorbid neuropathological diagnoses in early versus late-onset Alzheimer's disease.
  Brain : a journal of neurology 2021 Spina S, La Joie R, Petersen C, Nolan AL, Cuevas D, Cosme C, Hepker M, Hwang JH, Miller ZA, Huang EJ, Karydas AM, Grant H, Boxer AL, Gorno-Tempini ML, Rosen HJ, Kramer JH, Miller BL, Seeley WW, …
• Processing of progranulin into granulins involves multiple lysosomal proteases and is affected in frontotemporal lobar degeneration.
  Molecular neurodegeneration 2021 Mohan S, Sampognaro PJ, Argouarch AR, Maynard JC, Welch M, Patwardhan A, Courtney EC, Zhang J, Mason A, Li KH, Huang EJ, Seeley WW, Miller BL, Burlingame A, Jacobson MP, Kao AW
• Label-retention expansion microscopy.
  The Journal of cell biology 2021 Shi X, Li Q, Dai Z, Tran AA, Feng S, Ramirez AD, Lin Z, Wang X, Chow TT, Chen J, Kumar D, McColloch AR, Reiter JF, Huang EJ, Seiple IB, Huang B
• ALS-associated mutation FUS-R521C causes DNA damage and RNA splicing defects.
  The Journal of clinical investigation 2021 Qiu H, Lee S, Shang Y, Wang WY, Au KF, Kamiya S, Barmada SJ, Finkbeiner S, Lui H, Carlton CE, Tang AA, Oldham MC, Wang H, Shorter J, Filiano AJ, Roberson ED, Tourtellotte WG, Chen B, Tsai LH, Huang EJ
• Positive Controls in Adults and Children Support That Very Few, If Any, New Neurons Are Born in the Adult Human Hippocampus.
  The Journal of neuroscience : the official journal of the Society for Neuroscience 2021 Sorrells SF, Paredes MF, Zhang Z, Kang G, Pastor-Alonso O, Biagiotti S, Page CE, Sandoval K, Knox A, Connolly A, Huang EJ, Garcia-Verdugo JM, Oldham MC, Yang Z, Alvarez-Buylla A
• Wnt-Dependent Oligodendroglial-Endothelial Interactions Regulate White Matter Vascularization and Attenuate Injury.
  Neuron 2020 Chavali M, Ulloa-Navas MJ, Pérez-Borredá P, Garcia-Verdugo JM, McQuillen PS, Huang EJ, Rowitch DH
• Reciprocal Interaction between Vascular Filopodia and Neural Stem Cells Shapes Neurogenesis in the Ventral Telencephalon.
  Cell reports 2020 Di Marco B, Crouch EE, Shah B, Duman C, Paredes MF, Ruiz de Almodovar C, Huang EJ, Alfonso J
• A Developmental Analysis of Juxtavascular Microglia Dynamics and Interactions with the Vasculature.
  The Journal of neuroscience : the official journal of the Society for Neuroscience 2020 Mondo E, Becker SC, Kautzman AG, Schifferer M, Baer CE, Chen J, Huang EJ, Simons M, Schafer DP
• Inhibition of sphingolipid synthesis improves outcomes and survival in GARP mutant wobbler mice, a model of motor neuron degeneration.
  Proceedings of the National Academy of Sciences of the United States of America 2020 Petit CS, Lee JJ, Boland S, Swarup S, Christiano R, Lai ZW, Mejhert N, Elliott SD, McFall D, Haque S, Huang EJ, Bronson RT, Harper JW, Farese RV, Walther TC
• Frontotemporal dementia non-sense mutation of progranulin rescued by aminoglycosides.
  Human molecular genetics 2020 Kuang L, Hashimoto K, Huang EJ, Gentry MS, Zhu H
• The LC3-conjugation machinery specifies the loading of RNA-binding proteins into extracellular vesicles.
  Nature cell biology 2020 Leidal AM, Huang HH, Marsh T, Solvik T, Zhang D, Ye J, Kai F, Goldsmith J, Liu JY, Huang YH, Monkkonen T, Vlahakis A, Huang EJ, Goodarzi H, Yu L, Wiita AP, Debnath J
• Outer Radial Glia-like Cancer Stem Cells Contribute to Heterogeneity of Glioblastoma.
  Cell stem cell 2020 Bhaduri A, Di Lullo E, Jung D, Müller S, Crouch EE, Espinosa CS, Ozawa T, Alvarado B, Spatazza J, Cadwell CR, Wilkins G, Velmeshev D, Liu SJ, Malatesta M, Andrews MG, Mostajo-Radji MA, Huang EJ, …
• Loss of HIPK2 Protects Neurons from Mitochondrial Toxins by Regulating Parkin Protein Turnover.
  The Journal of neuroscience : the official journal of the Society for Neuroscience 2019 Zhang J, Shang Y, Kamiya S, Kotowski SJ, Nakamura K, Huang EJ
• Cortical developmental abnormalities in logopenic variant primary progressive aphasia with dyslexia.
  Brain communications 2019 Miller ZA, Spina S, Pakvasa M, Rosenberg L, Watson C, Mandelli ML, Paredes MF, Joie R, Rabinovici GD, Rosen HJ, Grinberg LT, Huang EJ, Miller BL, Seeley WW, Gorno-Tempini ML
• C9orf72-specific phenomena associated with frontotemporal dementia and gastrointestinal symptoms in the absence of TDP-43 aggregation.
  Acta neuropathologica 2019 Sampognaro PJ, Vatsavayai SC, Cosme CG, Hwang JL, Nolan A, Huang EJ, Seeley WW, De May MG
• Novel and lethal case of cardiac involvement in DNM1L mitochondrial encephalopathy.
  American journal of medical genetics. Part A 2019 Vandeleur D, Chen CV, Huang EJ, Connolly AJ, Sanchez H, Moon-Grady AJ
• Neuropathological correlates of structural and functional imaging biomarkers in 4-repeat tauopathies.
  Brain : a journal of neurology 2019 Spina S, Brown JA, Deng J, Gardner RC, Nana AL, Hwang JL, Gaus SE, Huang EJ, Kramer JH, Rosen HJ, Kornak J, Neuhaus J, Miller BL, Grinberg LT, Boxer AL, Seeley WW
• Immature excitatory neurons develop during adolescence in the human amygdala.
  Nature communications 2019 Sorrells SF, Paredes MF, Velmeshev D, Herranz-Pérez V, Sandoval K, Mayer S, Chang EF, Insausti R, Kriegstein AR, Rubenstein JL, Manuel Garcia-Verdugo J, Huang EJ, Alvarez-Buylla A
• Impaired αVβ8 and TGFβ signaling lead to microglial dysmaturation and neuromotor dysfunction.
  The Journal of experimental medicine 2019 Arnold TD, Lizama CO, Cautivo KM, Santander N, Lin L, Qiu H, Huang EJ, Liu C, Mukouyama YS, Reichardt LF, Zovein AC, Sheppard D
• Does Adult Neurogenesis Persist in the Human Hippocampus?
  Cell stem cell 2018 Paredes MF, Sorrells SF, Cebrian-Silla A, Sandoval K, Qi D, Kelley KW, James D, Mayer S, Chang J, Auguste KI, Chang EF, Gutierrez Martin AJ, Kriegstein AR, Mathern GW, Oldham MC, Huang EJ, Garcia-…
• Neurons selectively targeted in frontotemporal dementia reveal early stage TDP-43 pathobiology.
  Acta neuropathologica 2018 Nana AL, Sidhu M, Gaus SE, Hwang JL, Li L, Park Y, Kim EJ, Pasquini L, Allen IE, Rankin KP, Toller G, Kramer JH, Geschwind DH, Coppola G, Huang EJ, Grinberg LT, Miller BL, Seeley WW
• Progranulin in the hematopoietic compartment protects mice from atherosclerosis.
  Atherosclerosis 2018 Nguyen AD, Nguyen TA, Singh RK, Eberlé D, Zhang J, Abate JP, Robles A, Koliwad S, Huang EJ, Maxfield FR, Walther TC, Farese RV
• HIPK2-Mediated Transcriptional Control of NMDA Receptor Subunit Expression Regulates Neuronal Survival and Cell Death.
  The Journal of neuroscience : the official journal of the Society for Neuroscience 2018 Shang Y, Zhang J, Huang EJ
• Human hippocampal neurogenesis drops sharply in children to undetectable levels in adults.
  Nature 2018 Sorrells SF, Paredes MF, Cebrian-Silla A, Sandoval K, Qi D, Kelley KW, James D, Mayer S, Chang J, Auguste KI, Chang EF, Gutierrez AJ, Kriegstein AR, Mathern GW, Oldham MC, Huang EJ, Garcia-Verdugo JM,…
• Murine knockin model for progranulin-deficient frontotemporal dementia with nonsense-mediated mRNA decay.
  Proceedings of the National Academy of Sciences of the United States of America 2018 Nguyen AD, Nguyen TA, Zhang J, Devireddy S, Zhou P, Karydas AM, Xu X, Miller BL, Rigo F, Ferguson SM, Huang EJ, Walther TC, Farese RV
• Early vs late age at onset frontotemporal dementia and frontotemporal lobar degeneration.
  Neurology 2018 Seo SW, Thibodeau MP, Perry DC, Hua A, Sidhu M, Sible I, Vargas JNS, Gaus SE, Rabinovici GD, Rankin KD, Boxer AL, Kramer JH, Rosen HJ, Gorno-Tempini ML, Grinberg LT, Huang EJ, DeArmond SJ, Trojanowski…
• Clinicopathological correlations in behavioural variant frontotemporal dementia.
  Brain : a journal of neurology 2017 Perry DC, Brown JA, Possin KL, Datta S, Trujillo A, Radke A, Karydas A, Kornak J, Sias AC, Rabinovici GD, Gorno-Tempini ML, Boxer AL, De May M, Rankin KP, Sturm VE, Lee SE, Matthews BR, Kao AW, Vossel…
• Fibrinogen Activates BMP Signaling in Oligodendrocyte Progenitor Cells and Inhibits Remyelination after Vascular Damage.
  Neuron 2017 Petersen MA, Ryu JK, Chang KJ, Etxeberria A, Bardehle S, Mendiola AS, Kamau-Devers W, Fancy SPJ, Thor A, Bushong EA, Baeza-Raja B, Syme CA, Wu MD, Rios Coronado PE, Meyer-Franke A, Yahn S, Pous L, Lee…
• Self-Organized Cerebral Organoids with Human-Specific Features Predict Effective Drugs to Combat Zika Virus Infection.
  Cell reports 2017 Watanabe M, Buth JE, Vishlaghi N, de la Torre-Ubieta L, Taxidis J, Khakh BS, Coppola G, Pearson CA, Yamauchi K, Gong D, Dai X, Damoiseaux R, Aliyari R, Liebscher S, Schenke-Layland K, Caneda C, Huang …
• An RNA interference screen identifies druggable regulators of MeCP2 stability.
  Science translational medicine 2017 Lombardi LM, Zaghlula M, Sztainberg Y, Baker SA, Klisch TJ, Tang AA, Huang EJ, Zoghbi HY
• Loss of dual leucine zipper kinase signaling is protective in animal models of neurodegenerative disease.
  Science translational medicine 2017 Le Pichon CE, Meilandt WJ, Dominguez S, Solanoy H, Lin H, Ngu H, Gogineni A, Sengupta Ghosh A, Jiang Z, Lee SH, Maloney J, Gandham VD, Pozniak CD, Wang B, Lee S, Siu M, Patel S, Modrusan Z, Liu X, …
• Afadin controls cell polarization and mitotic spindle orientation in developing cortical radial glia.
  Neural development 2017 Rakotomamonjy J, Brunner M, Jüschke C, Zang K, Huang EJ, Reichardt LF, Chenn A
• Precipitous Deterioration of Motor Function, Cognition, and Behavior.
  JAMA neurology 2017 Fernández-Fournier M, Perry DC, Tartaglia MC, de May M, Boxer A, Coppola G, Christine CW, Huang EJ, Seeley WW, Miller BL, DeArmond SJ, Grinberg LT, Geschwind MD
• Long-term oral kinetin does not protect against α-synuclein-induced neurodegeneration in rodent models of Parkinson's disease.
  Neurochemistry international 2017 Orr AL, Rutaganira FU, de Roulet D, Huang EJ, Hertz NT, Shokat KM, Nakamura K
• Individuals with progranulin haploinsufficiency exhibit features of neuronal ceroid lipofuscinosis.
  Science translational medicine 2017 Ward ME, Chen R, Huang HY, Ludwig C, Telpoukhovskaia M, Taubes A, Boudin H, Minami SS, Reichert M, Albrecht P, Gelfand JM, Cruz-Herranz A, Cordano C, Alavi MV, Leslie S, Seeley WW, Miller BL, Bigio E,…
• Typical and atypical pathology in primary progressive aphasia variants.
  Annals of neurology 2017 Spinelli EG, Mandelli ML, Miller ZA, Santos-Santos MA, Wilson SM, Agosta F, Grinberg LT, Huang EJ, Trojanowski JQ, Meyer M, Henry ML, Comi G, Rabinovici G, Rosen HJ, Filippi M, Miller BL, Seeley WW, …
• Testing the Amyloid Hypothesis with a Humanized AD Mouse Model.
  Neuron 2017 Cahill MK, Huang EJ
• Suppression of C9orf72 RNA repeat-induced neurotoxicity by the ALS-associated RNA-binding protein Zfp106.
  eLife 2017 Celona B, Dollen JV, Vatsavayai SC, Kashima R, Johnson JR, Tang AA, Hata A, Miller BL, Huang EJ, Krogan NJ, Seeley WW, Black BL
• TGF-β Signaling in Dopaminergic Neurons Regulates Dendritic Growth, Excitatory-Inhibitory Synaptic Balance, and Reversal Learning.
  Cell reports 2016 Luo SX, Timbang L, Kim JI, Shang Y, Sandoval K, Tang AA, Whistler JL, Ding JB, Huang EJ
• Extensive migration of young neurons into the infant human frontal lobe.
  Science (New York, N.Y.) 2016 Paredes MF, James D, Gil-Perotin S, Kim H, Cotter JA, Ng C, Sandoval K, Rowitch DH, Xu D, McQuillen PS, Garcia-Verdugo JM, Huang EJ, Alvarez-Buylla A
• Quantitative MRI radiomics in the prediction of molecular classifications of breast cancer subtypes in the TCGA/TCIA data set.
  NPJ breast cancer 2016 Li H, Zhu Y, Burnside ES, Huang E, Drukker K, Hoadley KA, Fan C, Conzen SD, Zuley M, Net JM, Sutton E, Whitman GJ, Morris E, Perou CM, Ji Y, Giger ML
• MR Imaging Radiomics Signatures for Predicting the Risk of Breast Cancer Recurrence as Given by Research Versions of MammaPrint, Oncotype DX, and PAM50 Gene Assays.
  Radiology 2016 Li H, Zhu Y, Burnside ES, Drukker K, Hoadley KA, Fan C, Conzen SD, Whitman GJ, Sutton EJ, Net JM, Ganott M, Huang E, Morris EA, Perou CM, Ji Y, Giger ML
• Mechanisms of FUS mutations in familial amyotrophic lateral sclerosis.
  Brain research 2016 Shang Y, Huang EJ
• Selective Frontoinsular von Economo Neuron and Fork Cell Loss in Early Behavioral Variant Frontotemporal Dementia.
  Cerebral cortex (New York, N.Y. : 1991) 2016 Kim EJ, Sidhu M, Gaus SE, Huang EJ, Hof PR, Miller BL, DeArmond SJ, Seeley WW
• Dopaminergic Neurons and Brain Reward Pathways: From Neurogenesis to Circuit Assembly.
  The American journal of pathology 2015 Luo SX, Huang EJ
• Ventral midbrain dopaminergic neurons: From neurogenesis to neurodegeneration.
  FEBS letters 2015 Huang EJ
• Modeling ALS and FTD with iPSC-derived neurons.
  Brain research 2015 Lee S, Huang EJ
• Aldehyde dehydrogenase 1a1 mediates a GABA synthesis pathway in midbrain dopaminergic neurons.
  Science (New York, N.Y.) 2015 Kim JI, Ganesan S, Luo SX, Wu YW, Park E, Huang EJ, Chen L, Ding JB
• Myeloid expression of the AP-1 transcription factor JUNB modulates outcomes of type 1 and type 2 parasitic infections.
  Parasite immunology 2015 Fontana MF, Baccarella A, Kellar D, Oniskey TK, Terinate P, Rosenberg SD, Huang EJ, Herbert DR, Kim CC
• Evaluating and treating neurobehavioral symptoms in professional American football players: Lessons from a case series.
  Neurology. Clinical practice 2015 Gardner RC, Possin KL, Hess CP, Huang EJ, Grinberg LT, Nolan AL, Cohn-Sheehy BI, Ghosh PM, Lanata S, Merrilees J, Kramer JH, Berger MS, Miller BL, Yaffe K, Rabinovici GD
• The behavioural/dysexecutive variant of Alzheimer's disease: clinical, neuroimaging and pathological features.
  Brain : a journal of neurology 2015 Ossenkoppele R, Pijnenburg YA, Perry DC, Cohn-Sheehy BI, Scheltens NM, Vogel JW, Kramer JH, van der Vlies AE, La Joie R, Rosen HJ, van der Flier WM, Grinberg LT, Rozemuller AJ, Huang EJ, van Berckel …
• Existing Pittsburgh Compound-B positron emission tomography thresholds are too high: statistical and pathological evaluation.
  Brain : a journal of neurology 2015 Villeneuve S, Rabinovici GD, Cohn-Sheehy BI, Madison C, Ayakta N, Ghosh PM, La Joie R, Arthur-Bentil SK, Vogel JW, Marks SM, Lehmann M, Rosen HJ, Reed B, Olichney J, Boxer AL, Miller BL, Borys E, Jin …
• ALS-causative mutations in FUS/TLS confer gain and loss of function by altered association with SMN and U1-snRNP.
  Nature communications 2015 Sun S, Ling SC, Qiu J, Albuquerque CP, Zhou Y, Tokunaga S, Li H, Qiu H, Bui A, Yeo GW, Huang EJ, Eggan K, Zhou H, Fu XD, Lagier-Tourenne C, Cleveland DW
• Loss of mitochondrial fission depletes axonal mitochondria in midbrain dopamine neurons.
  The Journal of neuroscience : the official journal of the Society for Neuroscience 2014 Berthet A, Margolis EB, Zhang J, Hsieh I, Zhang J, Hnasko TS, Ahmad J, Edwards RH, Sesaki H, Huang EJ, Nakamura K
• Activity-dependent FUS dysregulation disrupts synaptic homeostasis.
  Proceedings of the National Academy of Sciences of the United States of America 2014 Sephton CF, Tang AA, Kulkarni A, West J, Brooks M, Stubblefield JJ, Liu Y, Zhang MQ, Green CB, Huber KM, Huang EJ, Herz J, Yu G
• Massive CNS monocytic infiltration at autopsy in an alemtuzumab-treated patient with NMO.
  Neurology(R) neuroimmunology & neuroinflammation 2014 Gelfand JM, Cotter J, Klingman J, Huang EJ, Cree BA
• Deletion of Rbpj from postnatal endothelium leads to abnormal arteriovenous shunting in mice.
  Development (Cambridge, England) 2014 Nielsen CM, Cuervo H, Ding VW, Kong Y, Huang EJ, Wang RA
• CCR2 deficiency impairs macrophage infiltration and improves cognitive function after traumatic brain injury.
  Journal of neurotrauma 2014 Hsieh CL, Niemi EC, Wang SH, Lee CC, Bingham D, Zhang J, Cozen ML, Charo I, Huang EJ, Liu J, Nakamura MC
• Parallel states of pathological Wnt signaling in neonatal brain injury and colon cancer.
  Nature neuroscience 2014 Fancy SP, Harrington EP, Baranzini SE, Silbereis JC, Shiow LR, Yuen TJ, Huang EJ, Lomvardas S, Rowitch DH
• Axons take a dive: Specialized contacts of serotonergic axons with cells in the walls of the lateral ventricles in mice and humans.
  Neurogenesis (Austin, Tex.) 2014 Tong CK, Cebrián-Silla A, Paredes MF, Huang EJ, García-Verdugo JM, Alvarez-Buylla A
• In vivo signatures of nonfluent/agrammatic primary progressive aphasia caused by FTLD pathology.
  Neurology 2013 Caso F, Mandelli ML, Henry M, Gesierich B, Bettcher BM, Ogar J, Filippi M, Comi G, Magnani G, Sidhu M, Trojanowski JQ, Huang EJ, Grinberg LT, Miller BL, Dronkers N, Seeley WW, Gorno-Tempini ML
• Practical utility of amyloid and FDG-PET in an academic dementia center.
  Neurology 2013 Sánchez-Juan P, Ghosh PM, Hagen J, Gesierich B, Henry M, Grinberg LT, O'Neil JP, Janabi M, Huang EJ, Trojanowski JQ, Vinters HV, Gorno-Tempini M, Seeley WW, Boxer AL, Rosen HJ, Kramer JH, Miller BL, …
• Interaction of FUS and HDAC1 regulates DNA damage response and repair in neurons.
  Nature neuroscience 2013 Wang WY, Pan L, Su SC, Quinn EJ, Sasaki M, Jimenez JC, Mackenzie IR, Huang EJ, Tsai LH
• A dramatic increase of C1q protein in the CNS during normal aging.
  The Journal of neuroscience : the official journal of the Society for Neuroscience 2013 Stephan AH, Madison DV, Mateos JM, Fraser DA, Lovelett EA, Coutellier L, Kim L, Tsai HH, Huang EJ, Rowitch DH, Berns DS, Tenner AJ, Shamloo M, Barres BA
• Overexpression of vascular endothelial growth factor in the germinal matrix induces neurovascular proteases and intraventricular hemorrhage.
  Science translational medicine 2013 Yang D, Baumann JM, Sun YY, Tang M, Dunn RS, Akeson AL, Kernie SG, Kallapur S, Lindquist DM, Huang EJ, Potter SS, Liang HC, Kuan CY
• Foxc1 is required by pericytes during fetal brain angiogenesis.
  Biology open 2013 Siegenthaler JA, Choe Y, Patterson KP, Hsieh I, Li D, Jaminet SC, Daneman R, Kume T, Huang EJ, Pleasure SJ
• Lamin B1 mediates cell-autonomous neuropathology in a leukodystrophy mouse model.
  The Journal of clinical investigation 2013 Heng MY, Lin ST, Verret L, Huang Y, Kamiya S, Padiath QS, Tong Y, Palop JJ, Huang EJ, Ptácek LJ, Fu YH
• Temporal and spatial requirements of Smoothened in ventral midbrain neuronal development.
  Neural development 2013 Tang M, Luo SX, Tang V, Huang EJ
• Systematic analysis of challenge-driven improvements in molecular prognostic models for breast cancer.
  Science translational medicine 2013 Margolin AA, Bilal E, Huang E, Norman TC, Ottestad L, Mecham BH, Sauerwine B, Kellen MR, Mangravite LM, Furia MD, Vollan HK, Rueda OM, Guinney J, Deflaux NA, Hoff B, Schildwachter X, Russnes HG, Park …
• Transcriptional corepressors HIPK1 and HIPK2 control angiogenesis via TGF-β-TAK1-dependent mechanism.
  PLoS biology 2013 Shang Y, Doan CN, Arnold TD, Lee S, Tang AA, Reichardt LF, Huang EJ
• Dissociation of frontotemporal dementia-related deficits and neuroinflammation in progranulin haploinsufficient mice.
  The Journal of neuroscience : the official journal of the Society for Neuroscience 2013 Filiano AJ, Martens LH, Young AH, Warmus BA, Zhou P, Diaz-Ramirez G, Jiao J, Zhang Z, Huang EJ, Gao FB, Farese RV, Roberson ED
• Conceptual developments in the causes of Cockayne syndrome.
  Mechanisms of ageing and development 2013 Cleaver JE, Bezrookove V, Revet I, Huang EJ
• Argyrophilic grain disease differs from other tauopathies by lacking tau acetylation.
  Acta neuropathologica 2013 Grinberg LT, Wang X, Wang C, Sohn PD, Theofilas P, Sidhu M, Arevalo JB, Heinsen H, Huang EJ, Rosen H, Miller BL, Gan L, Seeley WW
• Nonfluent/agrammatic PPA with in-vivo cortical amyloidosis and Pick's disease pathology.
  Behavioural neurology 2013 Caso F, Gesierich B, Henry M, Sidhu M, LaMarre A, Babiak M, Miller BL, Rabinovici GD, Huang EJ, Magnani G, Filippi M, Comi G, Seeley WW, Gorno-Tempini ML
• STAT3-mediated astrogliosis protects myelin development in neonatal brain injury.
  Annals of neurology 2012 Nobuta H, Ghiani CA, Paez PM, Spreuer V, Dong H, Korsak RA, Manukyan A, Li J, Vinters HV, Huang EJ, Rowitch DH, Sofroniew MV, Campagnoni AT, de Vellis J, Waschek JA
• Species-dependent posttranscriptional regulation of NOS1 by FMRP in the developing cerebral cortex.
  Cell 2012 Kwan KY, Lam MM, Johnson MB, Dube U, Shim S, Rašin MR, Sousa AM, Fertuzinhos S, Chen JG, Arellano JI, Chan DW, Pletikos M, Vasung L, Rowitch DH, Huang EJ, Schwartz ML, Willemsen R, Oostra BA, Rakic P,…
• ApoE and TDP-43 neuropathology in two siblings with familial FTLD-motor neuron disease.
  Neurocase 2012 Vossel KA, Bien-Ly N, Bernardo A, Rascovsky K, Karydas A, Rabinovici GD, Sidhu M, Huang EJ, Miller BL, Huang Y, Seeley WW
• Replication of hepatitis C virus RNA on autophagosomal membranes.
  The Journal of biological chemistry 2012 Sir D, Kuo CF, Tian Y, Liu HM, Huang EJ, Jung JU, Machida K, Ou JH
• Dysmyelination not demyelination causes neurological symptoms in preweaned mice in a murine model of Cockayne syndrome.
  Proceedings of the National Academy of Sciences of the United States of America 2012 Revet I, Feeney L, Tang AA, Huang EJ, Cleaver JE
• Defective retinal vascular endothelial cell development as a consequence of impaired integrin αVβ8-mediated activation of transforming growth factor-β.
  The Journal of neuroscience : the official journal of the Society for Neuroscience 2012 Arnold TD, Ferrero GM, Qiu H, Phan IT, Akhurst RJ, Huang EJ, Reichardt LF
• Amyloid vs FDG-PET in the differential diagnosis of AD and FTLD.
  Neurology 2011 Rabinovici GD, Rosen HJ, Alkalay A, Kornak J, Furst AJ, Agarwal N, Mormino EC, O'Neil JP, Janabi M, Karydas A, Growdon ME, Jang JY, Huang EJ, Dearmond SJ, Trojanowski JQ, Grinberg LT, Gorno-Tempini ML…
• Expression of A20 by dendritic cells preserves immune homeostasis and prevents colitis and spondyloarthritis.
  Nature immunology 2011 Hammer GE, Turer EE, Taylor KE, Fang CJ, Advincula R, Oshima S, Barrera J, Huang EJ, Hou B, Malynn BA, Reizis B, DeFranco A, Criswell LA, Nakamura MC, Ma A
• Transgenic expression of entire hepatitis B virus in mice induces hepatocarcinogenesis independent of chronic liver injury.
  PloS one 2011 Na B, Huang Z, Wang Q, Qi Z, Tian Y, Lu CC, Yu J, Hanes MA, Kakar S, Huang EJ, Ou JH, Liu L, Yen TS
• Corridors of migrating neurons in the human brain and their decline during infancy.
  Nature 2011 Sanai N, Nguyen T, Ihrie RA, Mirzadeh Z, Tsai HH, Wong M, Gupta N, Berger MS, Huang E, Garcia-Verdugo JM, Rowitch DH, Alvarez-Buylla A
• Homeodomain interacting protein kinase 2 regulates postnatal development of enteric dopaminergic neurons and glia via BMP signaling.
  The Journal of neuroscience : the official journal of the Society for Neuroscience 2011 Chalazonitis A, Tang AA, Shang Y, Pham TD, Hsieh I, Setlik W, Gershon MD, Huang EJ
• Clinicopathological correlations in corticobasal degeneration.
  Annals of neurology 2011 Lee SE, Rabinovici GD, Mayo MC, Wilson SM, Seeley WW, DeArmond SJ, Huang EJ, Trojanowski JQ, Growdon ME, Jang JY, Sidhu M, See TM, Karydas AM, Gorno-Tempini ML, Boxer AL, Weiner MW, Geschwind MD, …
• Hipk2 cooperates with p53 to suppress γ-ray radiation-induced mouse thymic lymphoma.
  Oncogene 2011 Mao JH, Wu D, Kim IJ, Kang HC, Wei G, Climent J, Kumar A, Pelorosso FG, DelRosario R, Huang EJ, Balmain A
• Comparative healing of human cutaneous surgical incisions created by the PEAK PlasmaBlade, conventional electrosurgery, and a standard scalpel.
  Plastic and reconstructive surgery 2011 Ruidiaz ME, Messmer D, Atmodjo DY, Vose JG, Huang EJ, Kummel AC, Rosenberg HL, Gurtner GC
• Krüppel-like factor 15 activates hepatitis B virus gene expression and replication.
  Hepatology (Baltimore, Md.) 2011 Zhou J, Tan T, Tian Y, Zheng B, Ou JH, Huang EJ, Yen TS
• Axin2 as regulatory and therapeutic target in newborn brain injury and remyelination.
  Nature neuroscience 2011 Fancy SP, Harrington EP, Yuen TJ, Silbereis JC, Zhao C, Baranzini SE, Bruce CC, Otero JJ, Huang EJ, Nusse R, Franklin RJ, Rowitch DH
• Selective frontoinsular von Economo neuron and fork cell loss in early behavioral variant frontotemporal dementia.
  Cerebral cortex (New York, N.Y. : 1991) 2011 Kim EJ, Sidhu M, Gaus SE, Huang EJ, Hof PR, Miller BL, DeArmond SJ, Seeley WW
• Loss of nuclear factor E2-related factor 1 in the brain leads to dysregulation of proteasome gene expression and neurodegeneration.
  Proceedings of the National Academy of Sciences of the United States of America 2011 Lee CS, Lee C, Hu T, Nguyen JM, Zhang J, Martin MV, Vawter MP, Huang EJ, Chan JY
• Towards improved animal models of neonatal white matter injury associated with cerebral palsy.
  Disease models & mechanisms 2010 Silbereis JC, Huang EJ, Back SA, Rowitch DH
• Acetylation of tau inhibits its degradation and contributes to tauopathy.
  Neuron 2010 Min SW, Cho SH, Zhou Y, Schroeder S, Haroutunian V, Seeley WW, Huang EJ, Shen Y, Masliah E, Mukherjee C, Meyers D, Cole PA, Ott M, Gan L
• Blocking the mitochondrial apoptotic pathway preserves motor neuron viability and function in a mouse model of amyotrophic lateral sclerosis.
  The Journal of clinical investigation 2010 Reyes NA, Fisher JK, Austgen K, VandenBerg S, Huang EJ, Oakes SA
• Interactions of Wnt/beta-catenin signaling and sonic hedgehog regulate the neurogenesis of ventral midbrain dopamine neurons.
  The Journal of neuroscience : the official journal of the Society for Neuroscience 2010 Tang M, Villaescusa JC, Luo SX, Guitarte C, Lei S, Miyamoto Y, Taketo MM, Arenas E, Huang EJ
• Extensive FUS-immunoreactive pathology in juvenile amyotrophic lateral sclerosis with basophilic inclusions.
  Brain pathology (Zurich, Switzerland) 2010 Huang EJ, Zhang J, Geser F, Trojanowski JQ, Strober JB, Dickson DW, Brown RH, Shapiro BE, Lomen-Hoerth C
• Two genetic variants of CD38 in subjects with autism spectrum disorder and controls.
  Neuroscience research 2010 Munesue T, Yokoyama S, Nakamura K, Anitha A, Yamada K, Hayashi K, Asaka T, Liu HX, Jin D, Koizumi K, Islam MS, Huang JJ, Ma WJ, Kim UH, Kim SJ, Park K, Kim D, Kikuchi M, Ono Y, Nakatani H, Suda S, …
• Safety evaluation of AAV2-GDNF gene transfer into the dopaminergic nigrostriatal pathway in aged and parkinsonian rhesus monkeys.
  Human gene therapy 2009 Su X, Kells AP, Huang EJ, Lee HS, Hadaczek P, Beyer J, Bringas J, Pivirotto P, Penticuff J, Eberling J, Federoff HJ, Forsayeth J, Bankiewicz KS
• Sporadic corticobasal syndrome due to FTLD-TDP.
  Acta neuropathologica 2009 Tartaglia MC, Sidhu M, Laluz V, Racine C, Rabinovici GD, Creighton K, Karydas A, Rademakers R, Huang EJ, Miller BL, DeArmond SJ, Seeley WW
• Multiple roles of beta-catenin in controlling the neurogenic niche for midbrain dopamine neurons.
  Development (Cambridge, England) 2009 Tang M, Miyamoto Y, Huang EJ
• A gamma-secretase inhibitor and quinacrine reduce prions and prevent dendritic degeneration in murine brains.
  Proceedings of the National Academy of Sciences of the United States of America 2008 Spilman P, Lessard P, Sattavat M, Bush C, Tousseyn T, Huang EJ, Giles K, Golde T, Das P, Fauq A, Prusiner SB, Dearmond SJ
• Increased apoptosis, p53 up-regulation, and cerebellar neuronal degeneration in repair-deficient Cockayne syndrome mice.
  Proceedings of the National Academy of Sciences of the United States of America 2007 Laposa RR, Huang EJ, Cleaver JE
• Dynamic expression of neurotrophic factor receptors in postnatal spinal motoneurons and in mouse model of ALS.
  Journal of neurobiology 2006 Zhang J, Huang EJ
• Direct phosphorylation and regulation of poly(ADP-ribose) polymerase-1 by extracellular signal-regulated kinases 1/2.
  Proceedings of the National Academy of Sciences of the United States of America 2006 Kauppinen TM, Chan WY, Suh SW, Wiggins AK, Huang EJ, Swanson RA
• Selective neuronal vulnerability and inadequate stress response in superoxide dismutase mutant mice.
  Free radical biology & medicine 2005 Lynn S, Huang EJ, Elchuri S, Naeemuddin M, Nishinaka Y, Yodoi J, Ferriero DM, Epstein CJ, Huang TT
• Notch-1 activation and dendritic atrophy in prion disease.
  Proceedings of the National Academy of Sciences of the United States of America 2005 Ishikura N, Clever JL, Bouzamondo-Bernstein E, Samayoa E, Prusiner SB, Huang EJ, DeArmond SJ
• Targeted deletion of numb and numblike in sensory neurons reveals their essential functions in axon arborization.
  Genes & development 2004 Huang EJ, Li H, Tang AA, Wiggins AK, Neve RL, Zhong W, Jan LY, Jan YN
• Interaction of Brn3a and HIPK2 mediates transcriptional repression of sensory neuron survival.
  The Journal of cell biology 2004 Wiggins AK, Wei G, Doxakis E, Wong C, Tang AA, Zang K, Luo EJ, Neve RL, Reichardt LF, Huang EJ
• Homeodomain-interacting protein kinase-2 regulates apoptosis in developing sensory and sympathetic neurons.
  Current biology : CB 2004 Doxakis E, Huang EJ, Davies AM
• Expression and activation of signal regulatory protein alpha on astrocytomas.
  Cancer research 2004 Chen TT, Brown EJ, Huang EJ, Seaman WE
• Trk receptors: roles in neuronal signal transduction.
  Annual review of biochemistry 2003 Huang EJ, Reichardt LF
• Brn3a is a transcriptional regulator of soma size, target field innervation and axon pathfinding of inner ear sensory neurons.
  Development (Cambridge, England) 2001 Huang EJ, Liu W, Fritzsch B, Bianchi LM, Reichardt LF, Xiang M
• NeuroD-null mice are deaf due to a severe loss of the inner ear sensory neurons during development.
  Development (Cambridge, England) 2001 Kim WY, Fritzsch B, Serls A, Bakel LA, Huang EJ, Reichardt LF, Barth DS, Lee JE
• Neurotrophins: roles in neuronal development and function.
  Annual review of neuroscience 2001 Huang EJ, Reichardt LF
• Formation of a full complement of cranial proprioceptors requires multiple neurotrophins.
  Developmental dynamics : an official publication of the American Association of Anatomists 2000 Fan G, Copray S, Huang EJ, Jones K, Yan Q, Walro J, Jaenisch R, Kucera J
• POU domain factor Brn-3a controls the differentiation and survival of trigeminal neurons by regulating Trk receptor expression.
  Development (Cambridge, England) 1999 Huang EJ, Zang K, Schmidt A, Saulys A, Xiang M, Reichardt LF
• Expression of Trk receptors in the developing mouse trigeminal ganglion: in vivo evidence for NT-3 activation of TrkA and TrkB in addition to TrkC.
  Development (Cambridge, England) 1999 Huang EJ, Wilkinson GA, Fariñas I, Backus C, Zang K, Wong SL, Reichardt LF
• Role of dimerization of the membrane-associated growth factor kit ligand in juxtacrine signaling: the Sl17H mutation affects dimerization and stability-phenotypes in hematopoiesis.
  The Journal of experimental medicine 1998 Tajima Y, Huang EJ, Vosseller K, Ono M, Moore MA, Besmer P
• Hepatitis B and C coinfections and persistent hepatitis B infections: clinical outcome and liver pathology after transplantation.
  Hepatology (Baltimore, Md.) 1996 Huang EJ, Wright TL, Lake JR, Combs C, Ferrell LD
• The expression pattern of the c-kit ligand in gonads of mice supports a role for the c-kit receptor in oocyte growth and in proliferation of spermatogonia.
  Developmental biology 1993 Manova K, Huang EJ, Angeles M, De Leon V, Sanchez S, Pronovost SM, Besmer P, Bachvarova RF
• The murine steel panda mutation affects kit ligand expression and growth of early ovarian follicles.
  Developmental biology 1993 Huang EJ, Manova K, Packer AI, Sanchez S, Bachvarova RF, Besmer P
• The kit-ligand (steel factor) and its receptor c-kit/W: pleiotropic roles in gametogenesis and melanogenesis.
  Development (Cambridge, England). Supplement 1993 Besmer P, Manova K, Duttlinger R, Huang EJ, Packer A, Gyssler C, Bachvarova RF
• c-kit receptor and ligand expression in postnatal development of the mouse cerebellum suggests a function for c-kit in inhibitory interneurons.
  The Journal of neuroscience : the official journal of the Society for Neuroscience 1992 Manova K, Bachvarova RF, Huang EJ, Sanchez S, Pronovost SM, Velazquez E, McGuire B, Besmer P
• Cleavage of membrane-anchored growth factors involves distinct protease activities regulated through common mechanisms.
  The Journal of biological chemistry 1992 Pandiella A, Bosenberg MW, Huang EJ, Besmer P, Massagué J
• Differential expression and processing of two cell associated forms of the kit-ligand: KL-1 and KL-2.
  Molecular biology of the cell 1992 Huang EJ, Nocka KH, Buck J, Besmer P