Jonathan P Bernardini
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Explore the profile of Jonathan P Bernardini including associated specialties, affiliations and a list of published articles.
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13
Citations
507
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Recent Articles
1.
Baker H, Bernardini J, Csizmok V, Madero A, Kamat S, Eng H, et al.
J Cell Sci
. 2024 Dec;
138(1.
PMID: 39618332
Mutations can disrupt the native function of protein by causing misfolding, which is generally handled by an intricate protein quality control network. To better understand the triaging mechanisms for misfolded...
2.
Cotton T, Cobbold S, Bernardini J, Richardson L, Wang X, Lechtenberg B
Mol Cell
. 2022 Jan;
82(3):598-615.e8.
PMID: 34998453
An increasing number of genetic diseases are linked to deregulation of E3 ubiquitin ligases. Loss-of-function mutations in the RING-between-RING (RBR) family E3 ligase RNF216 (TRIAD3) cause Gordon-Holmes syndrome (GHS) and...
3.
Sandow J, Tan I, Huang A, Masaldan S, Bernardini J, Wardak A, et al.
EMBO J
. 2021 Sep;
40(20):e107237.
PMID: 34523147
BAK and BAX, the effectors of intrinsic apoptosis, each undergo major reconfiguration to an activated conformer that self-associates to damage mitochondria and cause cell death. However, the dynamic structural mechanisms...
4.
Baker H, Bernardini J
Biochem Soc Trans
. 2021 Feb;
49(1):365-377.
PMID: 33634825
The accumulation of misfolded proteins is associated with numerous degenerative conditions, cancers and genetic diseases. These pathological imbalances in protein homeostasis (termed proteostasis), result from the improper triage and disposal...
5.
Cowan A, Smith N, Sandow J, Kapp E, Rustam Y, Murphy J, et al.
Nat Struct Mol Biol
. 2020 Sep;
27(11):1024-1031.
PMID: 32929280
BAK and BAX are essential mediators of apoptosis that oligomerize in response to death cues, thereby causing permeabilization of the mitochondrial outer membrane. Their transition from quiescent monomers to pore-forming...
6.
Klemm T, Ebert G, Calleja D, Allison C, Richardson L, Bernardini J, et al.
EMBO J
. 2020 Aug;
39(18):e106275.
PMID: 32845033
The SARS-CoV-2 coronavirus encodes an essential papain-like protease domain as part of its non-structural protein (nsp)-3, namely SARS2 PLpro, that cleaves the viral polyprotein, but also removes ubiquitin-like ISG15 protein...
7.
Kuechler E, Budzynska P, Bernardini J, Gsponer J, Mayor T
J Mol Biol
. 2020 Feb;
432(7):2349-2368.
PMID: 32105731
Recently generated proteomic data provides unprecedented insight into stress granule composition and stands as fruitful ground for further analysis. Stress granules are stress-induced biological assemblies that are of keen interest...
8.
Bolten M, Bernardini J, Mayor T
J Biol Chem
. 2019 Nov;
294(45):16525-16526.
PMID: 31704773
Cellular processes accompanying protein aggregation are diverse and entangled, making it difficult to investigate the underlying molecular processes in a time-resolved way. Gottlieb, Thompson, and colleagues address this shortcoming using...
9.
Bernardini J, Brouwer J, Tan I, Sandow J, Huang S, Stafford C, et al.
EMBO J
. 2018 Dec;
38(2).
PMID: 30573668
The E3 ubiquitin ligase Parkin is a key effector of the removal of damaged mitochondria by mitophagy. Parkin determines cell fate in response to mitochondrial damage, with its loss promoting...
10.
Aubrey B, Janic A, Chen Y, Chang C, Lieschke E, Diepstraten S, et al.
Genes Dev
. 2018 Oct;
32(21-22):1420-1429.
PMID: 30366906
Mutations in , prevalent in human cancer, are reported to drive tumorigenesis through dominant-negative effects (DNEs) over wild-type TRP53 function as well as neomorphic gain-of-function (GOF) activity. We show that...