Novel UBA domain mutations of SQSTM1 in Paget's disease of bone: genotype phenotype correlation, functional analysis, and structural consequences LJ Hocking, GJA Lucas, A Daroszewska, T Cundy, GC Nicholson, ... Journal of Bone and Mineral Research 19 (7), 1122-1127, 2004 | 177 | 2004 |
Structure of the ubiquitin-associated domain of p62 (SQSTM1) and implications for mutations that cause Paget's disease of bone B Ciani, R Layfield, JR Cavey, PW Sheppard, MS Searle Journal of Biological Chemistry 278 (39), 37409-37412, 2003 | 165 | 2003 |
Stabilization of β-hairpin peptides by salt bridges: Role of preorganization in the energetic contribution of weak interactions B Ciani, M Jourdan, MS Searle Journal of the American Chemical Society 125 (30), 9038-9047, 2003 | 144 | 2003 |
Loss of Ubiquitin‐Binding Associated With Paget's Disease of Bone p62 (SQSTM1) Mutations JR Cavey, SH Ralston, LJ Hocking, PW Sheppard, B Ciani, MS Searle, ... Journal of Bone and Mineral Research 20 (4), 619-624, 2005 | 131 | 2005 |
Design of β-sheet systems for understanding the thermodynamics and kinetics of protein folding MS Searle, B Ciani Current opinion in structural biology 14 (4), 458-464, 2004 | 127 | 2004 |
A designed system for assessing how sequence affects α to β conformational transitions in proteins B Ciani, EG Hutchinson, RB Sessions, DN Woolfson Journal of Biological Chemistry 277 (12), 10150-10155, 2002 | 125 | 2002 |
Transglutaminase 1 Mutations in Lamellar Ichthyosis LOSS OF ACTIVITY DUE TO FAILURE OF ACTIVATION BY PROTEOLYTIC PROCESSING E Candi, G Melino, A Lahm, R Ceci, A Rossi, IG Kim, B Ciani, PM Steinert Journal of Biological Chemistry 273 (22), 13693-13702, 1998 | 117 | 1998 |
Loss of Ubiquitin Binding Is a Unifying Mechanism by Which Mutations of SQSTM1 Cause Paget’s Disease of Bone JR Cavey, SH Ralston, PW Sheppard, B Ciani, TRA Gallagher, JE Long, ... Calcified tissue international 78, 271-277, 2006 | 92 | 2006 |
Molecular basis of coiled-coil oligomerization-state specificity B Ciani, S Bjelić, S Honnappa, H Jawhari, R Jaussi, A Payapilly, T Jowitt, ... Proceedings of the National Academy of Sciences 107 (46), 19850-19855, 2010 | 81 | 2010 |
Transglutaminase crosslinking and structural studies of the human small proline rich 3 protein PM Steinert, E Candi, E Tarcsa, LN Marekov, M Sette, M Paci, B Ciani, ... Cell Death & Differentiation 6 (9), 916-930, 1999 | 75 | 1999 |
ESCRT-III is necessary for the integrity of the nuclear envelope in micronuclei but is aberrant at ruptured micronuclear envelopes generating damage J Willan, AJ Cleasby, N Flores-Rodriguez, F Stefani, C Rinaldo, ... Oncogenesis 8 (5), 29, 2019 | 70 | 2019 |
Structure of the extracellular domain of Tie receptor tyrosine kinases and localization of the angiopoietin-binding epitope PR Macdonald, P Progias, B Ciani, S Patel, U Mayer, MO Steinmetz, ... Journal of Biological Chemistry 281 (38), 28408-28414, 2006 | 69 | 2006 |
Electrostatic contributions to the stability of the GCN4 leucine zipper structure WM Matousek, B Ciani, CA Fitch, B Garcia-Moreno E, RA Kammerer, ... Journal of molecular biology 374 (1), 206-219, 2007 | 67 | 2007 |
Direct binding of ESCRT protein Chm7 to phosphatidic acid–rich membranes at nuclear envelope herniations DJ Thaller, D Tong, CJ Marklew, NR Ader, PJ Mannino, S Borah, MC King, ... Journal of Cell Biology 220 (3), e202004222, 2021 | 50 | 2021 |
Atomic Models of De Novo Designed ccβ-Met Amyloid-Like Fibrils MO Steinmetz, Z Gattin, R Verel, B Ciani, T Stromer, JM Green, P Tittmann, ... Journal of molecular biology 376 (3), 898-912, 2008 | 42 | 2008 |
Structural and functional studies of mutations affecting the UBA domain of SQSTM1 (p62) which cause Paget's disease of bone R Layfield, B Ciani, SH Ralston, LJ Hocking, PW Sheppard, MS Searle, ... Biochemical Society Transactions 32 (5), 728-730, 2004 | 42 | 2004 |
Nature's lessons in design: nanomachines to scaffold, remodel and shape membrane compartments PA Beales, B Ciani, AJ Cleasby Physical Chemistry Chemical Physics 17 (24), 15489-15507, 2015 | 36 | 2015 |
C5orf30 is a negative regulator of tissue damage in rheumatoid arthritis M Muthana, S Hawtree, A Wilshaw, E Linehan, H Roberts, S Khetan, ... Proceedings of the National Academy of Sciences 112 (37), 11618-11623, 2015 | 31 | 2015 |
In Vitro Membrane Remodeling by ESCRT is Regulated by Negative Feedback from Membrane Tension A Booth, CJ Marklew, B Ciani, PA Beales iScience 15, 173-184, 2019 | 29 | 2019 |
The cornified envelope: a model of cell death in the skin G Melino, V De Laurenzi, MV Catani, A Terrinoni, B Ciani, E Candi, ... Apoptosis: Mechanisms and Role in Disease, 175-212, 1998 | 28 | 1998 |