9 research outputs found
Rapid degradation of mutant SLC25A46 by the ubiquitin-proteasome system results in MFN1/2-mediated hyperfusion of mitochondria
- Author
- Abrams AJ
- Ajay A. Vashisht
- Alexander C
- Ambivero CT
- Anton F
- Archer SL
- Bae S
- Bertholet AM
- Bohnert M
- Braschi E
- Brownell JE
- Campello S
- Carla M. Koehler
- Chen H
- Chen H
- Delettre C
- Detmer SA
- DeVay RM
- Emanuele MJ
- Haitina T
- Harner M
- Heo JM
- Hoppins S
- Hwang DK
- Ishihara N
- James A. Wohlschlegel
- Janer A
- Janos Steffen
- Jijun Wan
- Joanna C. Jen
- Karbowski M
- Karbowski M
- Kisselev AF
- Leboucher GP
- Ling Q
- Livnat-Levanon N
- Lokireddy S
- Magnaghi P
- Moore BS
- Nakamura N
- Narendra D
- Okuda-Shimizu Y
- Quiros PM
- Santel A
- Sesaki H
- Soucy TA
- Steven M. Claypool
- Tanaka A
- Thomas D. Fox
- Tsubuki S
- Verhoeven K
- Wan J
- Wong ED
- Yonashiro R
- Younger JM
- Yun J
- Zhang B
- ZĂĽchner S
- Publication venue
- 'American Society for Cell Biology (ASCB)'
- Publication date
- 01/03/2017
- Field of study
SCL25A46 is a mitochondrial carrier protein that surprisingly localizes to the outer membrane and is distantly related to Ugo1. Here we show that a subset of SLC25A46 interacts with mitochondrial dynamics components and the MICOS complex. Decreased expression of SLC25A46 results in increased stability and oligomerization of MFN1 and MFN2 on mitochondria, promoting mitochondrial hyperfusion. A mutation at L341P causes rapid degradation of SLC25A46, which manifests as a rare disease, pontocerebellar hypoplasia. The E3 ubiquitin ligases MULAN and MARCH5 coordinate ubiquitylation of SLC25A46 L341P, leading to degradation by organized activities of P97 and the proteasome. Whereas outer mitochondrial membrane–associated degradation is typically associated with apoptosis or a specialized type of autophagy termed mitophagy, SLC25A46 degradation operates independently of activation of outer membrane stress pathways. Thus SLC25A46 is a new component in mitochondrial dynamics that serves as a regulator for MFN1/2 oligomerization. Moreover, SLC25A46 is selectively degraded from the outer membrane independently of mitophagy and apoptosis, providing a framework for mechanistic studies in the proteolysis of outer membrane proteins
DEGRADATION OF CELL PROTEINS AND THE GENERATION OF MHC CLASS I-PRESENTED PEPTIDES
- Author
- Aki M
- Anton LC
- Benham A
- Benham AM
- Bergmann C
- Botbol V
- Cox JH
- DeMartino GN
- Deng Y
- Desautels M
- Dice J
- Dick LR
- Driscoll J
- Dubiel W
- Fruh K
- Gooding LR
- Goth S
- Gottesman S
- Grant E
- Gronostajski R
- Hammond SA
- Harding C
- Heinemeyer W
- Hershko A
- Hershko A
- Hilt W
- Kisselev AF
- Lee DH
- Luckey C
- Ma CP
- Michalek M
- Pickart CM
- Realini C
- Rechsteiner M
- Rock KL
- Rodriguez F
- Schmidtke G
- Shastri N
- Sijts AJ
- Sommer T
- Stern LJ
- Vinitsky A
- Wu Y
- Yellen-Shaw A
- Yellen-Shaw AJ
- Yewdell J
- Yewdell J
- Zaghouani H
- Zweerink HJ
- Publication venue
- 'Annual Reviews'
- Publication date
- Field of study
Making sense of mass destruction: quantitating MHC class I antigen presentation
- Author
- A Admon
- A Goldberg
- A Navon
- A Neisig
- A Neisig
- A Porgador
- A Turzynski
- AF Kisselev
- AM Benham
- B Drisaldi
- B Ortmann
- B Tomkinson
- CL Ward
- D Bromme
- D Fruci
- DB Williams
- DM Gakamsky
- DN Garboczi
- DN Wheatley
- DN Wheatley
- E De Plaen
- E Geier
- E Lammert
- E Macpherson
- E Reits
- EA Reits
- EA Reits
- F Esquivel
- F Momburg
- G Denkberg
- GC Turner
- GD Johnson
- H Schild
- HJ Zweerink
- I Bach
- IA York
- IA York
- J Beninga
- J Roelse
- JA Kenniston
- JJ Neefjes
- JJ Neefjes
- JO Koopmann
- JW Yewdell
- JW Yewdell
- JW Yewdell
- K Falk
- K Falk
- KC Garcia
- KL Rock
- L Notterpek
- L Stoltze
- LC Anton
- M Gromme
- M Houde
- MF Princiotta
- MF Princiotta
- MH Glickman
- MJ Kleijmeer
- ML Wei
- MS Villanueva
- N Benaroudj
- N Garbi
- NF Bence
- NP Dantuma
- P Cascio
- P Guermonprez
- P Spee
- PK Jackson
- PK Jackson
- PM Kloetzel
- PM van Endert
- R Glas
- R Verma
- RT Schimke
- S Ben-Shahar
- S Khan
- S Pareek
- S Siffroi-Fernandez
- SR Schwab
- T Ogura
- T Saric
- T Saric
- T Serwold
- TJ Jensen
- TP Dick
- TS Jardetzky
- U Schubert
- U Seifert
- UE Petaja-Repo
- V Thulasiraman
- Y Yedidia
- Publication venue
- 'Springer Science and Business Media LLC'
- Publication date
- Field of study
CMS TriDAS project: Technical Design Report, Volume 1: The Trigger Systems
- Author
- Aarnio Pertti A
- Abadjiev K
- Abbaneo D
- Abbrescia M
- Abdullin S
- Abramov A
- Abramov V
- Acharya B S
- Acosta D
- Adam W
- Adams M R
- Adams N
- Adzic P
- Afanasiev S V
- Aftab Z
- Ageron M
- Aguilar-BenĂtez M
- Ahmad J R
- Ahmad M A
- Ahn S
- Akchurin N
- Akgun U
- Alam J J
- Albajar C
- Alberdi J
- Albergo S
- Albert A
- Alemany-Fernandez R
- Almeida C
- Almeida N
- Altieri S
- Alverson G
- Amaglobeli N S
- Amapane N
- Amsler Claude
- An Q
- Anderhub H
- Anderson E W
- Andriyash A
- Anfreville M G
- Angarano M M
- Angelini F
- Anguelov T
- Anisimov I
- Annenkov A N
- Antchev G
- Antunovic Z
- Apollinari G
- Apollonio M
- Arce P
- Arcelli S
- Arisaka K
- Arneodo M
- Asimidis A
- Aspell P
- Atac Muzaffer
- Atamanchuk A G
- Atanasov I H
- Atoyan G S
- Attal A
- Auffray Etiennette
- Augusto J
- Avery P
- Avezov A
- Awan I U
- Axer M
- Ayan A S
- Azhgirey L S
- Aziz S
- Aziz T
- Azzi P
- Azzurri P
- Babucci E
- Baccaro S
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- Baden D
- Badertscher A
- Badier J
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- Bagliesi G
- Bagoly Z
- Bahk S Y
- Bailey D S
- Baillon Paul
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- Bala S B
- Ball A
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- Bandurin D V
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- Pauss Felicitas
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- Perrotta A
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- Petrov V
- Petrunin A
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- Pfeiffer A
- Pieri M
- Pierschel G
- Pietarinen E
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- Pikalov V A
- Pimiä Martti
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- Piotto E
- Pirollet B
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- Pishchalnikov Yu M
- Placci Alfredo
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- Platner E D
- Podrasky V
- Poilleux P
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- Pordes R
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- Potapov V
- Potashov S Yu
- Potenza R
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- Poukhov A
- Pozniak Krzysztof T
- Presland A D
- Priano C
- Principe R
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- Prokofiev O E
- Proskouriakov A
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- Prosper H B
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- Pruys H S
- Puccini M
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- Pugliese G
- Puljak I
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- Quadt A
- Rabbetts P D
- Radicci V
- Raffaelli F
- Rahal G
- Raics P
- Raidal Martti
- Raja R
- Rakhmatov N
- Ramond P
- Ramos S
- Rander J
- Ranieri A
- Rapidis P A
- Rasmislovich V
- Ratti S P
- Raupach F
- Raven G
- Raykov P
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- Razis P A
- Razmyslovich B V
- Rebecchi P
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- Regenfus C
- Reichanadter M
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- Rodrigues-Simoes-Moreira P
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- Rohringer Herbert
- Roinishvili N N
- Roinishvili V N
- Rolandi Luigi
- Romano F
- Romanteau T
- Romero A
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- Ronchese P
- Rondeaux F
- Ronzhin A
- Rosca A
- Rosenson L
- Rosowsky A
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- Rusack R W
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- Russo V
- Rustamov I
- Ruuskanen P V
- Rykaczewski H
- Rykalin V I
- Rácz A
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- Sadovskii A
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- Sakhelashvili T M
- Sakulin H
- Sakumoto W
- Salicio J
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- Sannikov A V
- Santiard Jean-Claude
- Santinelli R
- Santocchia A
- Santos M
- Sanzeni C
- Sarycheva L I
- Savage P J
- Savina M V
- Schael S
- Schleifer W
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- Schmitz D
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- Schnetzer S
- Schnyder R
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- Schultz von Dratzig A
- Schwick C
- Schöning A
- SciabĂ A
- Sciortino S
- Scorzoni A
- Scurlock B
- Seberini M
- Sedov A
- Seez Christopher J
- Segneri G
- Sehgal V
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- Shmatkov V
- Shmatov S V
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- Staiano A
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- Staveris-Polykalas A
- Stavitski I
- Stefanescu J
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- Taurok Anton
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- Teixeira I
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- Tudoric-Ghemo J
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- Udo Fred
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- Uzhinskii V V
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- Veltchev I
- Vemuri N
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- Ventura Sandro
- Venturi A
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- Verma P
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- Vesztergombi G
- Vidal R
- Viertel Gert M
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- Vila I
- Vintache D
- Virdee Tejinder S
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- Wallraff W
- Walsham P
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- Weiler Thomas J
- Weingarten Wolfgang
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- Wertelaers Piet
- Weseler S
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- Willers Ian Malcolm
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- Wittmer B
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- Wrochna G
- Wu Wei Min
- Wu J
- Wu S
- Wulz Claudia Elisabeth
- Wynhoff S
- Xia L
- Xie X
- Xie Z
- Xu Rong Sheng
- Xue Z L
- Yagil A
- Yang M
- Yarba V A
- Yasuda T
- Ye Shu Wei
- Ye Yan Lin
- Yelton J
- Yepes P
- Yildiz H D
- Yuan H B
- Yukaev A I
- Yuldashev B S
- Zach F
- Zachariadou K
- Zaichenko A A
- Zalessky V B
- Zalewski Piotr
- Zalyubovskij I I
- Zamiatin N I
- Zander A
- Zanet A
- Zarubin A V
- Zarubin P I
- Zavodthikov S
- Zeb J
- Zelepoukine S
- Zer-Zion D
- Zeyrek M T
- Zghiche A
- Zhang Bing Yun
- Zhang J W
- Zhang S Q
- Zhang Z P
- Zhao Wei Ren
- Zhao Z
- Zheng Jian Ping
- Zhu G Y
- Zhu R Y
- Zhu Y S
- Zhukov V
- Zilizi G
- Zito G
- Zotto P L
- Zsenei M
- Zubarev E V
- Zullo A
- Zumerle G
- Zupan M
- Zuyeuski R
- Zych P
- Zálán P
- Äystö J
- Ă“dor G
- Ă–nel Y M
- Publication venue
- Publication date
- 22/01/2004
- Field of study