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Mitochondria are double-membrane-bound organelles that are essential for cellular energy production. A fundamental question in eukaryotic cell biology is how the biogenesis of mitochondria is achieved and regulated

线粒体是双膜结合的细胞器，对于细胞能量生产至关重要。

基本信息

批准号：
MC_PC_13029/2
负责人：
Antonella Spinazzola
金额：
$ 132.8万
依托单位：
University College London
依托单位国家：
英国
项目类别：
Intramural
财政年份：
2016
资助国家：
英国
起止时间：
2016 至无数据
项目状态：
已结题

来源：
https://gtr.ukri.org/projects?ref=MC_PC_13029%2F2
关键词：
Mitochondria double membrane bound organelles

项目摘要

Mitochondria are key parts of the cell whose central role is to produce energy in a suitable form for many biological processes. They are also involved in programmed cell death, and in maintaining appropriate levels of calcium in cells. These activities require mitochondria to communicate with the cell nucleus. Disruption of mitochondrial function can lead to a broad range of human diseases including diabetes, neurodegenerative disorders, obesity, cancer and premature ageing. Therefore, a full understanding of the basic processes in mitochondria is needed to identify the causes and consequences of mitochondrial malfunction and to enable us to design new therapies that compensate for or correct such faults. This programme will study how mitochondria are made and how their function responds to the changing requirements of the cell during growth and development.

线粒体是细胞的关键部分，其中心作用是以适合于许多生物过程的形式产生能量。它们还参与程序性细胞死亡，并维持细胞中适当的钙水平。这些活动需要线粒体与细胞核通讯。线粒体功能的破坏可导致广泛的人类疾病，包括糖尿病、神经退行性疾病、肥胖症、癌症和过早衰老。因此，需要充分了解线粒体的基本过程，以确定线粒体功能障碍的原因和后果，并使我们能够设计新的治疗方法来弥补或纠正这些缺陷。该计划将研究线粒体是如何产生的，以及它们的功能如何响应细胞在生长和发育过程中不断变化的要求。

项目成果

期刊论文数量（10）

专著数量（0）

科研奖励数量（0）

会议论文数量（0）

专利数量（0）

2 deoxy-D-glucose augments the mitochondrial respiratory chain in heart.

DOI：
10.1038/s41598-022-10168-1
发表时间：
2022-04-27
期刊：
SCIENTIFIC REPORTS
影响因子：
4.6
作者：
Aiestaran-Zelaia, Irati;Jesus Sanchez-Guisado, Maria;Villar-Fernandez, Marina;Azkargorta, Mikel;Fadon-Padilla, Lucia;Fernandez-Pelayo, Uxoa;Perez-Rodriguez, Diego;Ramos-Cabrer, Pedro;Spinazzola, Antonella;Elortza, Felix;Ruiz-Cabello, Jesus;Holt, Ian J.
通讯作者：
Holt, Ian J.

Reply to: "Mitochondrial Parkinsonism due to SPG7/Paraplegin variants with secondary mtDNA depletion"

回复：“SPG7/Paraplegin 变体继发性 mtDNA 缺失导致线粒体帕金森症”

DOI：
10.1002/mds.27899
发表时间：
2019
期刊：
Movement Disorders
影响因子：
8.6
作者：
De La Casa-Fages B
通讯作者：
De La Casa-Fages B

MPV17 Loss Causes Deoxynucleotide Insufficiency and Slow DNA Replication in Mitochondria.

DOI：
10.1371/journal.pgen.1005779
发表时间：
2016-01
期刊：
PLoS genetics
影响因子：
4.5
作者：
Dalla Rosa I;Cámara Y;Durigon R;Moss CF;Vidoni S;Akman G;Hunt L;Johnson MA;Grocott S;Wang L;Thorburn DR;Hirano M;Poulton J;Taylor RW;Elgar G;Martí R;Voshol P;Holt IJ;Spinazzola A
通讯作者：
Spinazzola A

Transcript availability dictates the balance between strand-asynchronous and strand-coupled mitochondrial DNA replication.

DOI：
10.1093/nar/gky852
发表时间：
2018-11-16
期刊：
Nucleic acids research
影响因子：
14.9
作者：
Cluett TJ;Akman G;Reyes A;Kazak L;Mitchell A;Wood SR;Spinazzola A;Spelbrink JN;Holt IJ
通讯作者：
Holt IJ

Clinicopathologic and molecular spectrum of RNASEH1-related mitochondrial disease.

DOI：
10.1212/nxg.0000000000000149
发表时间：
2017-06
期刊：
Neurology. Genetics
影响因子：
0
作者：
Bugiardini E;Poole OV;Manole A;Pittman AM;Horga A;Hargreaves I;Woodward CE;Sweeney MG;Holton JL;Taanman JW;Plant GT;Poulton J;Zeviani M;Ghezzi D;Taylor J;Smith C;Fratter C;Kanikannan MA;Paramasivam A;Thangaraj K;Spinazzola A;Holt IJ;Houlden H;Hanna MG;Pitceathly RDS
通讯作者：
Pitceathly RDS

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Antonella Spinazzola其他文献

Proceedings of the Frontiers of Retrovirology Conference 2016

DOI：
10.1186/s12977-016-0294-5
发表时间：
2016-09-26
期刊：
Retrovirology
影响因子：
3.900
作者：
Irena Zurnic;Sylvia Hütter;Ute Lehmann;Nicole Stanke;Juliane Reh;Tobias Kern;Fabian Lindel;Gesche Gerresheim;Martin Hamann;Erik Müllers;Paul Lesbats;Peter Cherepanov;Erik Serrao;Alan Engelman;Dirk Lindemann;Claire Da Silva Santos;Kevin Tartour;Andrea Cimarelli;Rya Burdick;Jianbo Chen;Jaya Sastri;Wei-Shau Hu;Vinay Pathak;Oliver T. Keppler;Karine Pradeau;Sylvia Eiler;Nicolas Levy;Sarah Lennon;Sarah Cianferani;Stéphane Emiliani;Marc Ruff;Vincent Parissi;Sylvie Rato;Antonio Rausell;Miguel Munoz;Amalio Telenti;Angela Ciuffi;Alexander Zhyvoloup;Anat Melamed;Ian Anderson;Delphine Planas;Janos Kriston-Vizi;Robin Ketteler;Chen- Hsuin Lee;Andy Merritt;Petronela Ancuta;Charles Bangham;Ariberto Fassati;Anthony Rodari;Benoit Van Driessche;Mathilde Galais;Nadége Delacourt;Sylvain Fauquenoy;Caroline Vanhulle;Anna Kula;Arsène Burny;Olivier Rohr;Carine Van Lint;Thijs van Montfort;Renee van der Sluis;Dave Speijer;Ben Berkhout;Bo Meng;Andrzej Rutkowski;Neil Berry;Lars Dölken;Andrew Lever;Thomas Schuster;Benedikt Asbach;Ralf Wagner;Christine Gross;Veit Wiesmann;Martina Kalmer;Thomas Wittenberg;Jan Gettemans;Andrea K. Thoma-Kress;Minghua Li;Eric O. Freed;Shan-Lu Liu;Janis Müller;Jan Münch;Xaver Sewald;Pradeep Uchil;Mark Ladinsky;Jagadish Beloor;Ruoxi Pi;Christin Herrmann;Nasim Motamedi;Thomas Murooka;Michael Brehm;Dale Greiner;Thorsten Mempel;Pamela Bjorkman;Priti Kumar;Walther Mothes;Simone Joas;Erica Parrish;Clement Wesley Gnanadurai;Edina Lump;Christina M. Stürzel;Nicholas F. Parrish;Ulrike Sauermann;Katharina Töpfer;Tina Schultheiss;Steven Bosinger;Guido Silvestri;Cristian Apetrei;Nicholas Huot;Michaela Müller-Trutwin;Daniel Sauter;Beatrice H. Hahn;Christiane Stahl-Hennig;Frank Kirchhoff;Gerald Schumann;Sabine Jung-Klawitter;Nina V. Fuchs;Kyle R. Upton;Martin Muñoz-Lopez;Ruchi Shukla;Jichang Wang;Marta Garcia-Canadas;Cesar Lopez-Ruiz;Daniel J. Gerhardt;Attila Sebe;Ivana Grabundzija;Patricia Gerdes;Sylvia Merkert;Andres Pulgarin;Anja Bock;Ulrike Held;Anett Witthuhn;Alexandra Haase;Ernst J. Wolvetang;Ulrich Martin;Zoltán Ivics;Zsuzsanna Izsvák;J. Garcia-Perez;Geoffrey J. Faulkner;Tara Hurst;Aris Katzourakis;Gkikas Magiorkinis;Kerstin Schott;Rita Derua;Janna Seifried;Andreas Reuter;Heike Schmitz;Christiane Tondera;Alberto Brandariz-Nuñez;Felipe Diaz-Griffero;Veerle Janssens;Renate König;Hanna-Mari Baldauf;Lena Stegmann;Sarah-Marie Schwarz;Maud Trotard;Margarethe Martin;Gina Lenzi;Manja Burggraf;Xiaoyu Pan;Oliver I. Fregoso;Efrem S. Lim;Libin Abraham;Elina Erikson;Laura Nguyen;Ina Ambiel;Frank Rutsch;Baek Kim;Michael Emerman;Oliver T. Fackler;Sabine Wittmann;Rayk Behrendt;Bianca Volkmann;Kristin Eissmann;Thomas Gramberg;Sebastian Bolduan;Herwig Koppensteiner;Stefanie Regensburg;Ruth Brack-Werner;Rika Draenert;Michael Schindler;Aurélie Ducroux;Shuting Xu;Aparna Ponnurangam;Sergej Franz;Angelina Malassa;Ellen Ewald;Christine Goffinet;Sin-Yee Fung;Ching-Ping Chan;Chun-Kit Yuen;Kin-Hang Kok;Chin-Ping Chan;Dong-Yan Jin;Ulf Dittmer;Dorota Kmiec;Shilpa Iyer;Christina Stürzel;Beatrice Hahn;Yasuo Ariumi;Mariko Yasuda-Inoue;Koudai Kawano;Satoshi Tateishi;Priscilla Turelli;Alex Compton;Nicolas Roy;Françoise Porrot;Anne Billet;Nicoletta Casartelli;Jacob Yount;Chen Liang;Oliver Schwartz;Carsten Magnus;Lucia Reh;Penny Moore;Therese Uhr;Jacqueline Weber;Lynn Morris;Alexandra Trkola;Rashel V. Grindberg;Erika Schlaepfer;Gideon Schreiber;Viviana Simon;Roberto F. Speck;Zeger Debyser;Lenard Vranckx;Jonas Demeulemeester;Suha Saleh;Eric Verdin;Anna Cereseto;Frauke Christ;Rik Gijsbers;Gang Wang;Na Zhao;Atze T. Das;Josef Köstler;Beatriz Perdiguero;Mariano Esteban;Bertram L. Jacobs;David C. Montefiori;Celia C. LaBranche;Nicole L. Yates;Georgia D. Tomaras;Guido Ferrari;Kathryn E. Foulds;Mario Roederer;Gary Landucci;Donald N. Forthal;Michael S. Seaman;Natalie Hawkins;Steven G. Self;Sanjay Phogat;James Tartaglia;Susan W. Barnett;Brian Burke;Anthony D. Cristillo;Song Ding;Jonathan L. Heeney;Giuseppe Pantaleo;Viktoria Stab;Armin Ensser;Bettina Tippler;Dennis Burton;Matthias Tenbusch;Klaus Überla;Galit Alter;Giuseppe Lofano;Anne-Sophie Dugast;Viraj Kulkarni;Todd Suscovich;Tatiana Opazo;Felipe Barraza;Diego Herrera;Andrea Garces;Tomas Schwenke;Diego Tapia;Jorge Cancino;Gloria Arriagada;Christina Haußner;Dominik Damm;Anette Rohrhofer;Barbara Schmidt;Jutta Eichler;Rebecca Midgley;James Wheeldon;Vincent Piguet;Priyanka Khopkar;Megha Rohamare;Smita Kulkarni;Ana Godinho-Santos;Allan Hance;Joao Goncalves;Fabrizio Mammano;Romain Gasser;Meriem Hamoudi;Martina Pellicciotta;Zhicheng Zhou;Clara Visdeloup;Philippe Colin;Martine Braibant;Bernard Lagane;Matteo Negroni;Jula Wamara;Norbert Bannert;Thibault Mesplede;Nathan Osman;Kaitlin Anstett;Jiaming Calvin Liang;Hanh Thi Pham;Mark Wainberg;Wei Shao;Jigui Shan;Mary Kearney;Xiaolin Wu;Frank Maldarelli;John Mellors;Brian Luke;John Coffin;Stephen Hughes;Thomas Fricke;Silvana Opp;Caitlin Shepard;Dmitri Ivanov;Jose Valle-Casuso;Marine Kanja;Pierre Cappy;Matteo Negroni;Daniela Lener;Ekaterina Knyazhanskaya;Andrey Anisenko;Timofey Zatsepin;Marina Gottikh;Alexander Komkov;Anastasia Minervina;Gaiaz Nugmanov;Vadim Nazarov;Konstantin Khodosevich;Ilgar Mamedov;Yuri Lebedev;Marta Colomer-Lluch;Ruth Serra-Moreno;Ambra Sarracino;Lavina Gharu;Alexander Pasternak;Alessandro Marcello;Ann Marie McCartin;Anurag Kulkarni;Valentin Le Douce;Virginie Gautier;Ann Baeyens;Evelien Naessens;Anouk Van Nuffel;Karin Weening;Anne- Marie Reilly;Eva Claeys;Wim Trypsteen;Linos Vandekerckhove;Sven Eyckerman;Kris Gevaert;Bruno Verhasselt;Hoi Ping Mok;Nicholas Norton;Axel Fun;Jack Hirst;Mark Wills;Dalibor Miklik;Filip Senigl;Jiri Hejnar;Jun-ichi Sakuragi;Sayuri Sakuragi;Masaru Yokoyama;Tatsuo Shioda;Hironori Sato;Jochen Bodem;Rebecca Moschall;Sarah Denk;Steffen Erkelenz;Christian Schenk;Heiner Schaal;Norbert Donhauser;Ellen Socher;Sebastian Millen;Heinrich Sticht;Christine Gross;Melanie Mann;Guochao Wei;Matthew J. Betts;Yang Liu;Timo Kehl;Robert B. Russell;Martin Löchelt;Oliver Hohn;Saeed Mostafa;Kirsten Hanke;Stephen Norley;Chia-Yen Chen;Masashi Shingai;Pedro Borrego;Nuno Taveira;Klaus Strebel;Chris Hellmund;Bo Meng;Melanie Friedrich;Friedrich Hahn;Christian Setz;Pia Rauch;Kirsten Fraedrich;Alina Matthaei;Petra Henklein;Maximilian Traxdorf;Torgils Fossen;Ulrich Schubert;Aya Khwaja;Meytal Galilee;Akram Alian;Birco Schwalbe;Heiko Hauser;Michael Schreiber;Mirte Scherpenisse;Young-Keol Cho;Jungeun Kim;Daeun Jeong;Katerina Trejbalova;Martina Benesova;Dana Kucerova;Zdenka Vernerova;Rachel Amouroux;Petra Hajkova;Daniel Elleder;Tomas Hron;Helena Farkasova;Abinash Padhi;Jan Paces;Henan Zhu;Robert Gifford;Pablo Murcia;Maria Luisa Carrozza;Anna-Maria Niewiadomska;Maurizio Mazzei;Mounir Abi-Said;Joseph Hughes;Stéphane Hué;Robert Gifford;Adetayo Obasa;Graeme Jacobs;Susan Engelbrecht;Katharina Mack;Kathrin Starz;Matthias Geyer;Frederic Bibollet-Ruche;Christina Stürzel;Marie Leoz;Jean Christophe Plantier;Ayele Argaw-Denboba;Emanuela Balestrieri;Annalucia Serafino;Ilaria Bucci;Chiara Cipriani;Corrado Spadafora;Paolo Sinibaldi-Vallebona;Claudia Matteucci;S. Nandi Jayashree;Ujjwal Neogi;Anil K. Chhangani;Shravan Sing Rathore;Bajrang R. J. Mathur;Adeyemi Abati;B. Taylan Koç;Tuba Çiğdem Oğuzoğlu;Takatoshi Shimauchi;Stephan Caucheteux;Jocelyn Turpin;Katja Finsterbusch;Yoshiki Tokura;Shanti Souriant;Luciana Balboa;Karine Pingris;Denise Kviatcowsky;Brigitte Raynaud-Messina;Céline Cougoule;Ingrid Mercier;Marcelo Kuroda;Pablo González-Montaner;Sandra Inwentarz;Eduardo Jose Moraña;Maria del Carmen Sasiain;Olivier Neyrolles;Isabelle Maridonneau-Parini;Geanncarlo Lugo-Villarino;Christel Vérollet;Alexandra Herrmann;Dominique Thomas;Nerea Ferreirós Bouzas;Xavier Lahaye;Anvita Bhargava;Takeshi Satoh;Matteo Gentili;Silvia Cerboni;Aymeric Silvin;Cécile Conrad;Hakim Ahmed-Belkacem;Elisa C. Rodriguez;Jean-François Guichou;Nathalie Bosquet;Matthieu Piel;Roger Le Grand;Megan King;Jean-Michel Pawlotsky;Nicolas Manel;Henning Hofmann;Benedicte Vanwalscappel;Nicolin Bloch;Nathaniel Landau;Stanislav Indik;Benedikt Hagen;José Carlos Valle-Casuso;Awatef Allouch;Annie David;Françoise Barré-Sinoussi;Monsef Benkirane;Gianfranco Pancino;Asier Saez-Cirion;Wing-Yiu Lee;Richard Sloan;Bianca Schulte;Silvana Opp;Jonas Blomberg;Luana Vargiu;Patricia Rodriguez-Tomé;Enzo Tramontano;Göran Sperber;Namita Kumari;Tatiana Ammosova;Sharmeen Diaz;Patricia Oneal;Sergei Nekhai;Audrey Fahrny;Gustavo Gers-Huber;Annette Audigé;Anitha Jayaprakash;Ravi Sachidanandam;Matt Hernandez;Marsha Dillon-White;Shanti Souriant;Karine Pingris;Brigitte Raynaud-Messina;Céline Cougoule;Ingrid Mercier;Olivier Neyrolles;Isabelle Maridonneau-Parini;Geanncarlo Lugo-Villarino;Emmanuel Maze;Claire Ham;Neil Almond;Greg Towers;Robert Belshaw;Patrícia de Sousa-Pereira;Joana Abrantes;Massimo Pizzato;Pedro J. Esteves;Tanja Kahle;Sven Schmitt;Laura Merkel;Nina Reuter;Thomas Stamminger;Ilaria Dalla Rosa;Kate Bishop;Antonella Spinazzola;Harriet Groom;Gabrielle Vieyres;Mathias Müsken;Thomas Zillinger;Veit Hornung;Winfried Barchet;Susanne Häussler;Thomas Pietschmann;Aneela Javed;Nicole Leuchte;Gabriela Salinas;Lennart Opitz;Sieghart Sopper;Christiane Mummert;Christian Hofmann;Angela G. Hückelhoven;Silke Bergmann;Sandra M. Müller-Schmucker;Ellen G. Harrer;Jan Dörrie;Niels Schaft;Thomas Harrer;Laure Cardinaux;M.- L. Zahno;H.- R. Vogt;R. Zanoni;G. Bertoni;Maximilian Muenchhoff;Philip Goulder;Oliver Keppler;Stephanie Rebensburg;Markus Helfer;Yuwei Zhang;Huicheng Chen;Annie Bernier;Annie Gosselin;Jean- Pierre Routy;Birgitta Wöhrl;Anna Schneider;Angela Corona;Imke Spöring;Mareike Jordan;Bernd Buchholz;Elias Maccioni;Roberto Di Santo;Kristian Schweimer;Christian Schölz;Brian Weinert;Sebastian Wagner;Petra Beli;Yasuyuki Miyake;Jun Qi;Lars Jensen;Werner Streicher;Anna McCarthy;Nicholas Westwood;Sonia Lain;Jürgen Cox;Patrick Matthias;Matthias Mann;James Bradner;Chunaram Choudhary;Marcel Stern;Elena Valletta;Caterina Frezza;Francesca Marino-Merlo;Sandro Grelli;Anna Lucia Serafino;Antonio Mastino;Beatrice Macchi;Meike Kaulfuß;Sonja Windmann;Wibke Bayer;Sello Mikasi;Graeme Jacobs;Rebecca Heß;Michael Storcksdieck gen. Bonsmann;Carsten Kirschning;Bernd Lepenies;Anne Kolenbrander;Vladimir Temchura;Kenta Iijima;Junya Kobayashi;Yukihito Ishizaka
通讯作者：
Yukihito Ishizaka

Mitochondrial DNA competition: starving out the mutant genome

线粒体DNA竞争：“饿死”突变基因组

DOI：
10.1016/j.tips.2024.01.011
发表时间：
2024-03-01
期刊：
TRENDS IN PHARMACOLOGICAL SCIENCES
影响因子：
19.900
作者：
Antonella Spinazzola;Diego Perez-Rodriguez;Jan Ježek;Ian J. Holt
通讯作者：
Ian J. Holt

64 Systematic identification of human mitochondrial disease genes through integrative genomics

DOI：
10.1016/j.mito.2007.08.068
发表时间：
2007-12-01
期刊：
Conference abstract
影响因子：
作者：
Sarah Calvo;Mohit Jain;Xiaohui Xie;Sunil A. Sheth;Betty Chang;Olga A. Goldberger;Antonella Spinazzola;Massimo Zeviani;Steven A. Carr;Vamsi K. Mootha
通讯作者：
Vamsi K. Mootha