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Items: 1 to 20 of 33

1.
GLRX5-associated [Fe-S] cluster biogenesis disorder: further characterisation of the neurological phenotype and long-term outcome
Bindu Parayil Sankaran, Sachin Gupta, Michel Tchan, Beena Devanapalli, Yusof Rahman, Peter Procopis, Kaustuv Bhattacharya
Orphanet J Rare Dis. 2021; 16: 465. Published online 2021 Nov 3. doi: 10.1186/s13023-021-02073-z
PMCID:
PMC8565018
AbstractArticlePDF–1.4M
2.
Molecular Basis of Multiple Mitochondrial Dysfunctions Syndrome 2 Caused by CYS59TYR BOLA3 Mutation
Giovanni Saudino, Dafne Suraci, Veronica Nasta, Simone Ciofi-Baffoni, Lucia Banci
Int J Mol Sci. 2021 May; 22(9): 4848. Published online 2021 May 3. doi: 10.3390/ijms22094848
PMCID:
PMC8125686
AbstractArticlePDF–3.0M
3.
Glutaredoxin 5 deficiency causes sideroblastic anemia by specifically impairing heme biosynthesis and depleting cytosolic iron in human erythroblasts
Hong Ye, Suh Young Jeong, Manik C. Ghosh, Gennadiy Kovtunovych, Laura Silvestri, Danilo Ortillo, Naoya Uchida, John Tisdale, Clara Camaschella, Tracey A. Rouault
J Clin Invest. 2010 May 3; 120(5): 1749–1761. Published online 2010 Apr 1. doi: 10.1172/JCI40372
PMCID:
PMC2860907
AbstractArticlePDF–2.9M
4.
Cluster Exchange Reactivity of [2Fe-2S] Cluster-Bridged Complexes of BOLA3 with Monothiol Glutaredoxins
Sambuddha Sen, Brian Rao, Christine Wachnowsky, J. A. Cowan*
Metallomics. Author manuscript; available in PMC 2019 Sep 19.
Published in final edited form as: Metallomics. 2018 Sep 19; 10(9): 1282–1290. doi: 10.1039/c8mt00128f
PMCID:
PMC6146392
AbstractArticlePDF–1.1M
5.
Variant non ketotic hyperglycinemia is caused by mutations in LIAS, BOLA3 and the novel gene GLRX5
Peter R. Baker, II, Marisa W. Friederich, Michael A. Swanson, Tamim Shaikh, Kaustuv Bhattacharya, Gunter H. Scharer, Joseph Aicher, Geralyn Creadon-Swindell, Elizabeth Geiger, Kenneth N. MacLean, Wang-Tso Lee, Charu Deshpande, Mary-Louise Freckmann, Ling-Yu Shih, Melissa Wasserstein, Malene B. Rasmussen, Allan M. Lund, Peter Procopis, Jessie M. Cameron, Brian H. Robinson, Garry K. Brown, Ruth M. Brown, Alison G. Compton, Carol L. Dieckmann, Renata Collard, Curtis R. Coughlin, II, Elaine Spector, Michael F. Wempe, Johan L.K. Van Hove
Brain. 2014 Feb; 137(2): 366–379. Published online 2013 Dec 10. doi: 10.1093/brain/awt328
PMCID:
PMC3914472
AbstractArticlePDF–1.2M
6.
The mitochondrial Hsp70 chaperone Ssq1 facilitates Fe/S cluster transfer from Isu1 to Grx5 by complex formation
Marta A. Uzarska, Rafal Dutkiewicz, Sven-Andreas Freibert, Roland Lill, Ulrich Mühlenhoff
Mol Biol Cell. 2013 Jun 15; 24(12): 1830–1841. doi: 10.1091/mbc.E12-09-0644
PMCID:
PMC3681689
AbstractArticlePDF–1.6M
7.
Systematic Molecular Genetic Analysis of Congenital Sideroblastic Anemia: Evidence for Genetic Heterogeneity and Identification of Novel Mutations
Anke K. Bergmann, Dean R. Campagna, Erin M. McLoughlin, Suneet Agarwal, Mark D. Fleming, Sylvia S. Bottomley, Ellis J. Neufeld
Pediatr Blood Cancer. Author manuscript; available in PMC 2010 Mar 22.
Published in final edited form as: Pediatr Blood Cancer. 2010 Feb; 54(2): 273–278. doi: 10.1002/pbc.22244
PMCID:
PMC2843911
AbstractArticlePDF–420K
8.
BOLA1 Is an Aerobic Protein That Prevents Mitochondrial Morphology Changes Induced by Glutathione Depletion
Peter Willems, Bas F.J. Wanschers, John Esseling, Radek Szklarczyk, Urszula Kudla, Isabel Duarte, Marleen Forkink, Marco Nooteboom, Herman Swarts, Jolein Gloerich, Leo Nijtmans, Werner Koopman, Martijn A. Huynen
Antioxid Redox Signal. 2013 Jan 10; 18(2): 129–138. doi: 10.1089/ars.2011.4253
PMCID:
PMC3513987
AbstractArticlePDF–672K
9.
A Glutaredoxin·BolA Complex Serves as an Iron-Sulfur Cluster Chaperone for the Cytosolic Cluster Assembly Machinery
Avery G. Frey, Daniel J. Palenchar, Justin D. Wildemann, Caroline C. Philpott
J Biol Chem. 2016 Oct 21; 291(43): 22344–22356. Published online 2016 Aug 12. doi: 10.1074/jbc.M116.744946
PMCID:
PMC5077177
AbstractArticlePDF–2.7M
10.
Mitochondrial Bol1 and Bol3 function as assembly factors for specific iron-sulfur proteins
Marta A Uzarska, Veronica Nasta, Benjamin D Weiler, Farah Spantgar, Simone Ciofi-Baffoni, Maria Rosaria Saviello, Leonardo Gonnelli, Ulrich Mühlenhoff, Lucia Banci, Roland Lill
eLife. 2016; 5: e16673. Published online 2016 Aug 17. doi: 10.7554/eLife.16673
PMCID:
PMC5014550
AbstractArticlePDF–2.3M
11.
Cochaperone binding to LYR motifs confers specificity of iron sulfur cluster delivery
Nunziata Maio, Anamika Singh, Helge Uhrigshardt, Neetu Saxena, Wing-Hang Tong, Tracey A. Rouault
Cell Metab. Author manuscript; available in PMC 2019 Jun 5.
Published in final edited form as: Cell Metab. 2014 Mar 4; 19(3): 445–457. doi: 10.1016/j.cmet.2014.01.015
PMCID:
PMC6550293
AbstractArticlePDF–2.6M
12.
Role of Nfu1 and Bol3 in iron-sulfur cluster transfer to mitochondrial clients
Andrew Melber, Un Na, Ajay Vashisht, Benjamin D Weiler, Roland Lill, James A Wohlschlegel, Dennis R Winge
eLife. 2016; 5: e15991. Published online 2016 Aug 17. doi: 10.7554/eLife.15991
PMCID:
PMC5014551
AbstractArticlePDF–2.2M
13.
Mammalian BCAS3 and C16orf70 associate with the phagophore assembly site in response to selective and non-selective autophagy
Waka Kojima, Koji Yamano, Hidetaka Kosako, Kenichiro Imai, Reika Kikuchi, Keiji Tanaka, Noriyuki Matsuda
Autophagy. 2021; 17(8): 2011–2036. Published online 2021 Jan 26. doi: 10.1080/15548627.2021.1874133
PMCID:
PMC8386740
AbstractArticlePDF–13M
14.
Mitochondrial ClpP-mediated proteolysis induces selective cancer cell lethality
Jo Ishizawa, Sarah F. Zarabi, R. Eric Davis, Ondrej Halgas, Takenobu Nii, Yulia Jitkova, Ran Zhao, Jonathan St-Germain, Lauren E. Heese, Grace Egan, Vivian R. Ruvolo, Samir H. Barghout, Yuki Nishida, Rose Hurren, Wencai Ma, Marcela Gronda, Todd Link, Keith Wong, Mark Mabanglo, Kensuke Kojima, Gautam Borthakur, Neil MacLean, John Man Chun Ma, Andrew B. Leber, Mark D. Minden, Walid Houry, Hagop Kantarjian, Martin Stogniew, Brian Raught, Emil F. Pai, Aaron D. Schimmer, Michael Andreeff
Cancer Cell. Author manuscript; available in PMC 2020 May 13.
Published in final edited form as: Cancer Cell. 2019 May 13; 35(5): 721–737.e9. Published online 2019 May 2. doi: 10.1016/j.ccell.2019.03.014
PMCID:
PMC6620028
AbstractArticlePDF–2.9M
15.
Interaction of LATS1 with SMAC links the MST2/Hippo pathway with apoptosis in an IAP-dependent manner
Lucía García-Gutiérrez, Emma Fallahi, Nourhan Aboud, Niall Quinn, David Matallanas
Cell Death Dis. 2022 Aug; 13(8): 692. Published online 2022 Aug 8. doi: 10.1038/s41419-022-05147-3
PMCID:
PMC9360443
AbstractArticlePDF–5.8M
16.
Mitochondrial protein interaction mapping identifies new regulators of respiratory chain function
Brendan J. Floyd, Emily M. Wilkerson, Mike T. Veling, Catie E. Minogue, Chuanwu Xia, Emily T. Beebe, Russell L. Wrobel, Holly Cho, Laura S. Kremer, Charlotte L. Alston, Katarzyna A. Gromek, Brendan K. Dolan, Arne Ulbrich, Jonathan A. Stefely, Sarah L. Bohl, Kelly M. Werner, Adam Jochem, Michael S. Westphall, Jarred W. Rensvold, Robert W. Taylor, Holger Prokisch, Jung-Ja P. Kim, Joshua J. Coon, David J. Pagliarini
Mol Cell. Author manuscript; available in PMC 2017 Aug 18.
Published in final edited form as: Mol Cell. 2016 Aug 18; 63(4): 621–632. Published online 2016 Aug 4. doi: 10.1016/j.molcel.2016.06.033
PMCID:
PMC4992456
AbstractArticlePDF–1.7M
17.
Novel Genetic Loci Identified for the Pathophysiology of Childhood Obesity in the Hispanic Population
Anthony G. Comuzzie, Shelley A. Cole, Sandra L. Laston, V. Saroja Voruganti, Karin Haack, Richard A. Gibbs, Nancy F. Butte
PLoS One. 2012; 7(12): e51954. Published online 2012 Dec 14. doi: 10.1371/journal.pone.0051954
PMCID:
PMC3522587
AbstractArticlePDF–130K
18.
Rewiring of the Human Mitochondrial Interactome during Neuronal Reprogramming Reveals Regulators of the Respirasome and Neurogenesis
Mohamed Taha Moutaoufik, Ramy Malty, Shahreen Amin, Qingzhou Zhang, Sadhna Phanse, Alla Gagarinova, Mara Zilocchi, Larissa Hoell, Zoran Minic, Maria Gagarinova, Hiroyuki Aoki, Jocelyn Stockwell, Matthew Jessulat, Florian Goebels, Kirsten Broderick, Nichollas E. Scott, James Vlasblom, Gabriel Musso, Bhanu Prasad, Eleonora Lamantea, Barbara Garavaglia, Alex Rajput, Kei Murayama, Yasushi Okazaki, Leonard J. Foster, Gary D. Bader, Francisco S. Cayabyab, Mohan Babu
iScience. 2019 Sep 27; 19: 1114–1132. Published online 2019 Sep 4. doi: 10.1016/j.isci.2019.08.057
PMCID:
PMC6831851
AbstractArticlePDF–9.8M
19.
Genetic Variants in Nuclear-Encoded Mitochondrial Genes Influence AIDS Progression
Sher L. Hendrickson, James A. Lautenberger, Leslie Wei Chinn, Michael Malasky, Efe Sezgin, Lawrence A. Kingsley, James J. Goedert, Gregory D. Kirk, Edward D. Gomperts, Susan P. Buchbinder, Jennifer L. Troyer, Stephen J. O'Brien
PLoS One. 2010; 5(9): e12862. Published online 2010 Sep 21. doi: 10.1371/journal.pone.0012862
PMCID:
PMC2943476
AbstractArticlePDF–461K
20.
BAP1 regulation of the key adaptor protein NCoR1 is critical for γ-globin gene repression
Lei Yu, Natee Jearawiriyapaisarn, Mary P. Lee, Tomonori Hosoya, Qingqing Wu, Greggory Myers, Kim-Chew Lim, Ryo Kurita, Yukio Nakamura, Anne B. Vojtek, Jean-François Rual, James Douglas Engel
Genes Dev. 2018 Dec 1; 32(23-24): 1537–1549. doi: 10.1101/gad.318436.118
PMCID:
PMC6295165
AbstractArticlePDF–3.5M

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