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F. Ebstein · SciRah

Researcher profile · Semantic Scholar

F. Ebstein

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F. Ebstein — researcher profile on SciRah

h-index: 30
Works: 94
Citations: 3,959

Most-cited papers

Superior antigen cross-presentation and XCR1 expression define human CD11c+CD141+ cells as homologues of mouse CD8+ dendritic cells
A. Bachem, S. Güttler, Evelyn Hartung, F. Ebstein +10 more
2010Journal of Experimental Medicine863 citations Open access
Semantic Scholar
Immunoproteasomes preserve protein homeostasis upon interferon-induced oxidative stress.
U. Seifert, L. Biały, F. Ebstein, Dawadschargal Bech‐Otschir +10 more
2010Cell574 citations Open access
Semantic Scholar
The Ubiquitin–Proteasome System in Immune Cells
Gonca Çetin, Sandro Klafack, Maja Studencka-Turski, Elke Krüger +1 more
2021Biomolecules191 citations Open access
Semantic Scholar
GestaltMatcher: Overcoming the limits of rare disease matching using facial phenotypic descriptors
Tzung-Chien Hsieh, Aviram Bar-Haim, Shahida Moosa, Nadja Ehmke +43 more
2022Nature Genetics185 citations Open access
Semantic Scholar
Heterozygous Truncating Variants in POMP Escape Nonsense-Mediated Decay and Cause a Unique Immune Dysregulatory Syndrome.
M. Poli, F. Ebstein, S. Nicholas, M. De Guzman +23 more
2018American Journal of Human Genetics151 citations Open access
Semantic Scholar
Emerging roles of immunoproteasomes beyond MHC class I antigen processing
F. Ebstein, P. Kloetzel, Elke Krüger, U. Seifert
2012Cellular and Molecular Life Sciences144 citations Open access
Semantic Scholar
Proteasomes generate spliced epitopes by two different mechanisms and as efficiently as non-spliced epitopes
F. Ebstein, K. Textoris-Taube, C. Keller, R. Golnik +14 more
2016Scientific Reports96 citations Open access
Semantic Scholar
Contribution of the Unfolded Protein Response (UPR) to the Pathogenesis of Proteasome-Associated Autoinflammatory Syndromes (PRAAS)
F. Ebstein, M. C. Poli Harlowe, Maja Studencka-Turski, Elke Krüger
2019Frontiers in Immunology77 citations Open access
Semantic Scholar
Role of Proteasomes in Inflammation
Carl Christoph Goetzke, F. Ebstein, T. Kallinich
2021Journal of Clinical Medicine76 citations Open access
Semantic Scholar
De Novo Disruption of the Proteasome Regulatory Subunit PSMD12 Causes a Syndromic Neurodevelopmental Disorder.
S. Küry, T. Besnard, F. Ebstein, Tahir N. Khan +57 more
2017American Journal of Human Genetics76 citations Open access
Semantic Scholar
Maturation of human dendritic cells is accompanied by functional remodelling of the ubiquitin-proteasome system.
F. Ebstein, N. Lange, S. Urban, U. Seifert +2 more
2009International Journal of Biochemistry and Cell Biology73 citations
Semantic Scholar
T lymphocytes export proteasomes by way of microparticles: a possible mechanism for generation of extracellular proteasomes
Isabel Bochmann, F. Ebstein, Andrea Lehmann, J. Wohlschlaeger +3 more
2013Journal of Cellular and Molecular Medicine71 citations Open access
Semantic Scholar
Proteasome subunit PSMC3 variants cause neurosensory syndrome combining deafness and cataract due to proteotoxic stress
Ariane Kröll-Hermi, F. Ebstein, C. Stoetzel, Véronique Geoffroy +25 more
2019bioRxiv62 citations Open access
Semantic Scholar
Biallelic Variants in OTUD6B Cause an Intellectual Disability Syndrome Associated with Seizures and Dysmorphic Features.
Teresa Santiago-Sim, Lindsay C. Burrage, F. Ebstein, M. Tokita +63 more
2017American Journal of Human Genetics62 citations Open access
Semantic Scholar
Cross-Presentation of Synthetic Long Peptides by Human Dendritic Cells: A Process Dependent on ERAD Component p97/VCP but Not sec61 and/or Derlin-1
J. Ménager, F. Ebstein, R. Oger, P. Hulin +6 more
2014PLoS ONE61 citations Open access
Semantic Scholar
Protein kinase R is an innate immune sensor of proteotoxic stress via accumulation of cytoplasmic IL-24
Sophia Davidson, Chien-Hsiung Yu, A. Steiner, F. Ebstein +22 more
2022Science immunology55 citations Open access
Semantic Scholar
The proteasome immunosubunits, PA28 and ER‐aminopeptidase 1 protect melanoma cells from efficient MART‐126‐35‐specific T‐cell recognition
M. Keller, F. Ebstein, Elke Bürger, K. Textoris-Taube +16 more
2015European Journal of Immunology54 citations Open access
Semantic Scholar
De Novo Disruption of the Proteasome Regulatory Subunit PSMD12 Causes a Syndromic Neurodevelopmental Disorder.
S. Küry, T. Besnard, F. Ebstein, Tahir N. Khan +57 more
2017American Journal of Human Genetics52 citations Open access
Semantic Scholar
Immunoproteasomes are important for proteostasis in immune responses.
F. Ebstein, A. Voigt, N. Lange, A. Warnatsch +7 more
2013Cell48 citations Open access
Semantic Scholar
The FAT10- and ubiquitin-dependent degradation machineries exhibit common and distinct requirements for MHC class I antigen presentation
F. Ebstein, Andrea Lehmann, P. Kloetzel
2012Cellular and Molecular Life Sciences48 citations Open access
Semantic Scholar
Neurodevelopmental Disorders (NDD) Caused by Genomic Alterations of the Ubiquitin-Proteasome System (UPS): the Possible Contribution of Immune Dysregulation to Disease Pathogenesis
F. Ebstein, S. Küry, J. J. Papendorf, Elke Krüger
2021Frontiers in Molecular Neuroscience43 citations Open access
Semantic Scholar
Molecular Insight Into the IRE1α-Mediated Type I Interferon Response Induced by Proteasome Impairment in Myeloid Cells of the Brain
Maja Studencka-Turski, Gonca Çetin, H. Junker, F. Ebstein +1 more
2019Frontiers in Immunology43 citations Open access
Semantic Scholar
Increased expression of inducible HSP70 in apoptotic cells is correlated with their efficacy for antitumor vaccine therapy
D. Massé, F. Ebstein, G. Bougras, J. Harb +2 more
2004International Journal of Cancer41 citations Open access
Semantic Scholar
Biallelic variants in PSMB1 encoding the proteasome subunit β6 cause impairment of proteasome function, microcephaly, intellectual disability, developmental delay and short stature.
Muhammad Ansar, F. Ebstein, Hayriye Özkoc, S. A. Paracha +10 more
2020Human Molecular Genetics40 citations
Semantic Scholar
Culture Medium and Protein Supplementation in the Generation and Maturation of Dendritic Cells
P. Royer, Séverine Tanguy-Royer, F. Ebstein, C. Sapede +4 more
2006Scandinavian Journal of Immunology40 citations Open access
Semantic Scholar
PSMC3 proteasome subunit variants are associated with neurodevelopmental delay and type I interferon production
F. Ebstein, S. Küry, V. Most, C. Rosenfelt +70 more
2023Science Translational Medicine34 citations Open access
Semantic Scholar
GestaltMatcher Database - A global reference for the facial phenotypic variability of rare human diseases
H. Lesmann, Alexander Hustinx, Shahida Moosa, E. Marchi +79 more
2023medRxiv31 citations Open access
Semantic Scholar
Identification of eight novel proteasome variants in five unrelated cases of proteasome-associated autoinflammatory syndromes (PRAAS)
J. J. Papendorf, F. Ebstein, S. Alehashemi, D. Piotto +26 more
2023Frontiers in Immunology31 citations Open access
Semantic Scholar
Cytotoxic T cell responses against mesothelioma by apoptotic cell-pulsed dendritic cells.
F. Ebstein, C. Sapede, Pierre-Joseph Royer, M. Marcq +5 more
2004American Journal of Respiratory and Critical Care Medicine31 citations
Semantic Scholar
Transient exposure of dendritic cells to maturation stimuli is sufficient to induce complete phenotypic maturation while preserving their capacity to respond to subsequent restimulation
R. Špíšek, G. Bougras, F. Ebstein, D. Massé +3 more
2003Cancer Immunology and Immunotherapy31 citations
Semantic Scholar
A new carboxamide compound exerts immuno‐suppressive activity by inhibiting dendritic cell maturation
D. Carbonnelle, F. Ebstein, Catherine Rabu, J. Petit +2 more
2005European Journal of Immunology30 citations
Semantic Scholar
Antigen‐presentation capacity of dendritic cells is impaired in ongoing enterovirus myocarditis
A. Rahnefeld, F. Ebstein, N. Albrecht, E. Opitz +5 more
2011European Journal of Immunology28 citations Open access
Semantic Scholar
Proteostasis Perturbations and Their Roles in Causing Sterile Inflammation and Autoinflammatory Diseases
J. J. Papendorf, Elke Krüger, F. Ebstein
2022Cells27 citations Open access
Semantic Scholar
Proteasomal adaptations underlying carfilzomib-resistance in human bone marrow plasma cells
E. S. Woodle, Simon Tremblay, P. Brailey, A. Girnita +9 more
2019American Journal of Transplantation26 citations Open access
Semantic Scholar
Exposure to Melan-A/MART-126-35 tumor epitope specific CD8+T cells reveals immune escape by affecting the ubiquitin-proteasome system (UPS)
F. Ebstein, M. Keller, A. Paschen, P. Walden +6 more
2016Scientific Reports26 citations Open access
Semantic Scholar
The Efficiency of Human Cytomegalovirus pp65495–503 CD8+ T Cell Epitope Generation Is Determined by the Balanced Activities of Cytosolic and Endoplasmic Reticulum-Resident Peptidases
S. Urban, K. Textoris-Taube, B. Reimann, Katharina Janek +15 more
2012Journal of Immunology26 citations
Semantic Scholar
HSCT corrects primary immunodeficiency and immune dysregulation in patients with POMP-related auto-inflammatory disease.
C. Martinez, F. Ebstein, S. Nicholas, M. De Guzman +9 more
2021Blood25 citations Open access
Semantic Scholar
Report of the first patient with a homozygous OTUD7A variant responsible for epileptic encephalopathy and related proteasome dysfunction
Philippine Garret, F. Ebstein, Geoffroy Delplancq, B. Dozières-Puyravel +15 more
2020Clinical Genetics25 citations
Semantic Scholar
Rare germline heterozygous missense variants in BRCA1-associated protein 1, BAP1, cause a syndromic neurodevelopmental disorder.
S. Küry, F. Ebstein, Alice Mollé, T. Besnard +54 more
2022American Journal of Human Genetics24 citations Open access
Semantic Scholar
Immunoproteasomes control activation of innate immune signaling and microglial function
Gonca Çetin, Maja Studencka-Turski, S. Venz, Eileen Schormann +5 more
2022Frontiers in Immunology24 citations Open access
Semantic Scholar
Immunoproteasome Function in Normal and Malignant Hematopoiesis
Nuria Tubío-Santamaría, F. Ebstein, F. Heidel, Elke Krüger
2021Cells24 citations Open access
Semantic Scholar
Efficient monocyte-derived dendritic cell generation in patients with acute myeloid leukemia after chemotherapy treatment: application to active immunotherapy.
Pierre-Joseph Royer, G. Bougras, F. Ebstein, L. Leveque +6 more
2008Experimental Hematology22 citations Open access
Semantic Scholar
The Proteasome Activator PA200/PSME4: An Emerging New Player in Health and Disease
A. Yazgili, F. Ebstein, S. Meiners
2022Biomolecules21 citations Open access
Semantic Scholar
Hematopoietic stem cell transplantation in a patient with proteasome-associated auto-inflammatory syndrome (PRAAS).
Dorit Verhoeven, D. Schonenberg-Meinema, F. Ebstein, J. J. Papendorf +11 more
2021Journal of Allergy and Clinical Immunology20 citations Open access
Semantic Scholar
Stankiewicz-Isidor syndrome: expanding the clinical and molecular phenotype.
B. Isidor, F. Ebstein, A. Hurst, M. Vincent +47 more
2021Genetics in Medicine18 citations Open access
Semantic Scholar
SIGLEC1 (CD169) as a potential diagnostical screening marker for monogenic interferonopathies
B. Orak, G. Ngoumou, F. Ebstein, B. Zieba +10 more
2020Pediatric Allergy and Immunology18 citations
Semantic Scholar
Major Histocompatibility Complex (MHC) Class I Processing of the NY-ESO-1 Antigen Is Regulated by Rpn10 and Rpn13 Proteins and Immunoproteasomes following Non-lysine Ubiquitination*
R. Golnik, Andrea Lehmann, P. Kloetzel, F. Ebstein
2016Journal of Biological Chemistry18 citations Open access
Semantic Scholar
Loss-of-function variants in CUL3 cause a syndromic neurodevelopmental disorder
P. Blackburn, F. Ebstein, Tzung-Chien Hsieh, M. Motta +75 more
2024Annals of Neurology17 citations Open access
Semantic Scholar
Understanding neurodevelopmental proteasomopathies as new rare disease entities: A review of current concepts, molecular biomarkers, and perspectives
S. Cuinat, S. Bézieau, W. Deb, Sandra Mercier +4 more
2023Genes and Diseases16 citations Open access
Semantic Scholar
Immunoproteasome function maintains oncogenic gene expression in KMT2A-complex driven leukemia
Nuria Tubío-Santamaría, A. Jayavelu, T. Schnoeder, Theresa Eifert +22 more
2023Molecular Cancer15 citations Open access
Semantic Scholar

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