Detalhe da pesquisa
1.
Coordination between aminoacylation and editing to protect against proteotoxicity.
Nucleic Acids Res
; 51(19): 10606-10618, 2023 10 27.
Artigo
em Inglês
| MEDLINE | ID: mdl-37742077
2.
Heterozygous MAP3K20 variants cause ectodermal dysplasia, craniosynostosis, sensorineural hearing loss, and limb anomalies.
Hum Genet
; 143(3): 279-291, 2024 Mar.
Artigo
em Inglês
| MEDLINE | ID: mdl-38451290
3.
Bi-allelic variants in OGDHL cause a neurodevelopmental spectrum disease featuring epilepsy, hearing loss, visual impairment, and ataxia.
Am J Hum Genet
; 108(12): 2368-2384, 2021 12 02.
Artigo
em Inglês
| MEDLINE | ID: mdl-34800363
4.
Biallelic variants in OGDH encoding oxoglutarate dehydrogenase lead to a neurodevelopmental disorder characterized by global developmental delay, movement disorder, and metabolic abnormalities.
Genet Med
; 25(2): 100332, 2023 02.
Artigo
em Inglês
| MEDLINE | ID: mdl-36520152
5.
Atomic structure of the Leishmania spp. Hsp100 N-domain.
Proteins
; 90(6): 1242-1246, 2022 06.
Artigo
em Inglês
| MEDLINE | ID: mdl-35122310
6.
Deciphering the mechanism and function of Hsp100 unfoldases from protein structure.
Biochem Soc Trans
; 50(6): 1725-1736, 2022 12 16.
Artigo
em Inglês
| MEDLINE | ID: mdl-36454589
7.
A biallelic pathogenic variant in the OGDH gene results in a neurological disorder with features of a mitochondrial disease.
J Inherit Metab Dis
; 44(2): 388-400, 2021 03.
Artigo
em Inglês
| MEDLINE | ID: mdl-32383294
8.
Mitochondrial Hsp90 is a ligand-activated molecular chaperone coupling ATP binding to dimer closure through a coiled-coil intermediate.
Proc Natl Acad Sci U S A
; 113(11): 2952-7, 2016 Mar 15.
Artigo
em Inglês
| MEDLINE | ID: mdl-26929380
9.
Structural insights into the oligomerization of FtsH periplasmic domain from Thermotoga maritima.
Biochem Biophys Res Commun
; 495(1): 1201-1207, 2018 01 01.
Artigo
em Inglês
| MEDLINE | ID: mdl-29180014
10.
An intersubunit signaling network coordinates ATP hydrolysis by m-AAA proteases.
Mol Cell
; 35(5): 574-85, 2009 Sep 11.
Artigo
em Inglês
| MEDLINE | ID: mdl-19748354
11.
Heat shock protein (Hsp) 70 is an activator of the Hsp104 motor.
Proc Natl Acad Sci U S A
; 110(21): 8513-8, 2013 May 21.
Artigo
em Inglês
| MEDLINE | ID: mdl-23650362
12.
Structural basis for intersubunit signaling in a protein disaggregating machine.
Proc Natl Acad Sci U S A
; 109(31): 12515-20, 2012 Jul 31.
Artigo
em Inglês
| MEDLINE | ID: mdl-22802670
13.
CryoEM structure of Hsp104 and its mechanistic implication for protein disaggregation.
Proc Natl Acad Sci U S A
; 107(18): 8135-40, 2010 May 04.
Artigo
em Inglês
| MEDLINE | ID: mdl-20404203
14.
Structural basis of impaired disaggregase function in the oxidation-sensitive SKD3 mutant causing 3-methylglutaconic aciduria.
Nat Commun
; 14(1): 2028, 2023 04 11.
Artigo
em Inglês
| MEDLINE | ID: mdl-37041140
15.
Enniatin A inhibits the chaperone Hsp90 and unleashes the immune system against triple-negative breast cancer.
iScience
; 26(12): 108308, 2023 Dec 15.
Artigo
em Inglês
| MEDLINE | ID: mdl-38025772
16.
Functional analysis of conserved cis- and trans-elements in the Hsp104 protein disaggregating machine.
J Struct Biol
; 179(2): 172-80, 2012 Aug.
Artigo
em Inglês
| MEDLINE | ID: mdl-22634726
17.
Electron cryomicroscopy structure of a membrane-anchored mitochondrial AAA protease.
J Biol Chem
; 286(6): 4404-11, 2011 Feb 11.
Artigo
em Inglês
| MEDLINE | ID: mdl-21147776
18.
Differential relationship between metabolic syndrome score and severity of coronary atherosclerosis as assessed by angiography in a non-diabetic and diabetic Korean population.
J Korean Med Sci
; 26(7): 900-5, 2011 Jul.
Artigo
em Inglês
| MEDLINE | ID: mdl-21738343
19.
Macrocyclic Immunoproteasome Inhibitors as a Potential Therapy for Alzheimer's Disease.
J Med Chem
; 64(15): 10934-10950, 2021 08 12.
Artigo
em Inglês
| MEDLINE | ID: mdl-34309393
20.
The Mitochondrial Protease LonP1 Promotes Proteasome Inhibitor Resistance in Multiple Myeloma.
Cancers (Basel)
; 13(4)2021 Feb 17.
Artigo
em Inglês
| MEDLINE | ID: mdl-33671345