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Sofie V Nielsen
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DVC1 (C1orf124) is a DNA damage–targeting p97 adaptor that promotes ubiquitin-dependent responses to replication blocks
A Mosbech, I Gibbs-Seymour, K Kagias, T Thorslund, P Beli, L Povlsen, ...
Nature structural & molecular biology 19 (11), 1084-1092, 2012
2152012
Molecular basis and regulation of OTULIN-LUBAC interaction
PR Elliott, SV Nielsen, P Marco-Casanova, BK Fiil, K Keusekotten, ...
Molecular cell 54 (3), 335-348, 2014
2002014
Predicting the impact of Lynch syndrome-causing missense mutations from structural calculations
SV Nielsen, A Stein, AB Dinitzen, E Papaleo, MH Tatham, EG Poulsen, ...
PLoS genetics 13 (4), e1006739, 2017
922017
Understanding the origins of loss of protein function by analyzing the effects of thousands of variants on activity and abundance
M Cagiada, KE Johansson, A Valanciute, SV Nielsen, ...
Molecular Biology and Evolution 38 (8), 3235-3246, 2021
702021
A chaperone-assisted degradation pathway targets kinetochore proteins to ensure genome stability
F Kriegenburg, V Jakopec, EG Poulsen, SV Nielsen, A Roguev, N Krogan, ...
PLoS genetics 10 (1), e1004140, 2014
662014
Towards mechanistic models for genotype‐phenotype correlations in phenylketonuria using protein stability calculations
R Scheller, A Stein, SV Nielsen, FI Marin, AM Gerdes, MD Marco, ...
Human Mutation, 2019
602019
Computational and cellular studies reveal structural destabilization and degradation of MLH1 variants in Lynch syndrome
AB Abildgaard, A Stein, SV Nielsen, K Schultz-Knudsen, E Papaleo, ...
Elife 8, e49138, 2019
482019
Protein quality control in the nucleus
SV Nielsen, EG Poulsen, CA Rebula, R Hartmann-Petersen
Biomolecules 4 (3), 646-661, 2014
442014
Co-chaperones in targeting and delivery of misfolded proteins to the 26S proteasome
AB Abildgaard, SK Gersing, S Larsen-Ledet, SV Nielsen, A Stein, ...
Biomolecules 10 (8), 1141, 2020
362020
Bioinformatics analysis identifies several intrinsically disordered human E3 ubiquitin-protein ligases
W Boomsma, SV Nielsen, K Lindorff-Larsen, R Hartmann-Petersen, ...
PeerJ 4, e1725, 2016
302016
Folliculin variants linked to Birt-Hogg-Dubé syndrome are targeted for proteasomal degradation
L Clausen, A Stein, M Grønbæk-Thygesen, L Nygaard, CL Søltoft, ...
PLoS genetics 16 (11), e1009187, 2020
242020
Lynch syndrome, molecular mechanisms and variant classification
AB Abildgaard, SV Nielsen, I Bernstein, A Stein, K Lindorff-Larsen, ...
British journal of cancer 128 (5), 726-734, 2023
232023
Blocking protein quality control to counter hereditary cancers
C Kampmeyer, SV Nielsen, L Clausen, A Stein, AM Gerdes, ...
Genes, Chromosomes and Cancer 56 (12), 823-831, 2017
232017
Disease-linked mutations cause exposure of a protein quality control degron
C Kampmeyer, S Larsen-Ledet, MR Wagnkilde, M Michelsen, ...
Structure 30 (9), 1245-1253. e5, 2022
172022
Human ASPL/TUG interacts with p97 and complements the proteasome mislocalization of a yeast ubx4 mutant, but not the ER-associated degradation defect
L Madsen, K Molbæk, IB Larsen, SV Nielsen, EG Poulsen, PS Walmod, ...
BMC cell biology 15, 1-10, 2014
132014
Multiplexed assays reveal effects of missense variants in MSH2 and cancer predisposition
SV Nielsen, R Hartmann-Petersen, A Stein, K Lindorff-Larsen
PLoS Genetics 17 (4), e1009496, 2021
122021
Protein destabilization and degradation as a mechanism for hereditary disease
SV Nielsen, SM Schenstrøm, CE Christensen, A Stein, K Lindorff-Larsen, ...
Protein homeostasis diseases, 111-125, 2020
32020
High-Throughput siRNA Screening Applied to the Ubiquitin–Proteasome System
EG Poulsen, SV Nielsen, EJ Pietras, JV Johansen, C Steinhauer, ...
Proteostasis: Methods and Protocols, 421-439, 2016
32016
Disease-linked mutations trigger exposure of a protein quality control degron in the DHFR protein
C Kampmeyer, S Larsen-Ledet, MR Wagnkilde, M Michelsen, ...
bioRxiv, 2021.11. 04.467226, 2021
22021
Structural destabilization and chaperone-assisted proteasomal degradation of MLH1 as a mechanism for Lynch syndrome
AB Abildgaard, A Stein, K Schultz-Knudsen, SV Nielsen, E Papaleo, ...
BioRxiv, 622266, 2019
12019
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