TY  - JOUR
AB  - Beta-propeller protein-associated neurodegeneration (BPAN) is a rare neurodegenerative disease associated with severe cognitive and motor deficits. BPAN pathophysiology and phenotypic spectrum are still emerging due to the fact that mutations in the WDR45 (WD repeat domain 45) gene, a regulator of macroautophagy/autophagy, were only identified a decade ago. In the first international symposium dedicated to BPAN, which was held in Lyon, France, a panel of international speakers, including several researchers from the autophagy community, presented their work on human patients, cellular and animal models, carrying WDR45 mutations and their homologs. Autophagy researchers found an opportunity to explore the defective function of autophagy mechanisms associated with WDR45 mutations, which underlie neuronal dysfunction and early death. Importantly, BPAN is one of the few human monogenic neurological diseases targeting a regulator of autophagy, which raises the possibility that it is a relevant model to directly assess the roles of autophagy in neurodegeneration and to develop autophagy restorative therapeutic strategies for more common disorders.Abbreviations: ATG: autophagy related; BPAN: beta-propeller protein-associated neurodegeneration; ER: endoplasmic reticulum; KO: knockout; NBIA: neurodegeneration with brain iron accumulation; PtdIns3P: phosphatidylinositol-3-phosphate; ULK1: unc-51 like autophagy activating kinase 1; WDR45: WD repeat domain 45; WIPI: WD repeat domain, phosphoinositide interacting.
AU  - Mollereau, B.*
AU  - Hayflick, S.J.*
AU  - Escalante, R.*
AU  - Mauthe, M.*
AU  - Papandreou, A.*
AU  - Iuso, A.
AU  - Celle, M.*
AU  - Aniorte, S.*
AU  - Issa, A.R.*
AU  - Lasserre, J.P.*
AU  - Lesca, G.*
AU  - Thobois, S.*
AU  - Burger, P.*
AU  - Walter, L.*
C1  - 68006
C2  - 54484
CY  - 530 Walnut Street, Ste 850, Philadelphia, Pa 19106 Usa
SP  - 3234-3239
TI  - A burning question from the first international BPAN symposium: Is restoration of autophagy a promising therapeutic strategy for BPAN?
JO  - Autophagy
VL  - 19
IS  - 12
PB  - Taylor & Francis Inc
PY  - 2023
SN  - 1554-8627
ER  - 

TY  - JOUR
AB  - MicroRNAs (miRNAs) are post-transcriptional regulators of gene expression which act by guiding AGO (argonaute) proteins to target RNA transcripts in the RNA-induced silencing complex (RISC). This macromolecular complex includes multiple additional components (e.g., TNRC6A) that allow for interaction with enzymes mediating inhibition of translation or RNA decay. However, miRNAs also reside in low-molecular weight complexes without being engaged in target repression, and their function in this context is largely unknown. Our recent findings show that endothelial cells exposed to protective high-shear stress or MTORC inhibition activate the macroautophagy/autophagy machinery to sustain viability by promoting differential trafficking ofMIR126strands and by enabling unconventional features ofMIR126-5p. WhereasMIR126-3pis degraded upon autophagy activation,MIR126-5pinteracts with the RNA-binding protein MEX3A to form a ternary complex with AGO2. This complex forms on the autophagosomal surface and facilitates its nuclear localization. Once in the nucleus,MIR126-5pdissociates from AGO2 and establishes aptamer-like interactions with the effector CASP3 (caspase 3). The binding toMIR126-5pprevents dimerization and proper active site formation of CASP3, thus inhibiting proteolytic activity and limiting apoptosis. Disrupting this pathwayin vivoby genetic deletion ofMex3aor by specific deficiency of endothelial autophagy aggravates endothelial apoptosis and exacerbates the progression of atherosclerosis. The direct inhibition of CASP3 byMIR126-5preveals a non-canonical mechanism by which miRNAs can modulate protein function and mediate the autophagy-apoptosis crosstalk.
AU  - Santovito, D.*
AU  - Egea, V.*
AU  - Bidzhekov, K.*
AU  - Natarelli, L.*
AU  - Mourao, A.
AU  - Blanchet, X.*
AU  - Wichapong, K.*
AU  - Aslani, M.*
AU  - Brunßen, C.*
AU  - Horckmans, M.*
AU  - Hristov, M.*
AU  - Geerlof, A.
AU  - Lutgens, E.*
AU  - Daemen, M.J.A.P.*
AU  - Hackeng, T.*
AU  - Ries, C.*
AU  - Chavakis, T.*
AU  - Morawietz, H.*
AU  - Naumann, R.*
AU  - Hundelshausen, P.V.*
AU  - Steffens, S.*
AU  - Duchêne, J.*
AU  - Megens, R.T.A.*
AU  - Sattler, M.
AU  - Weber, C.*
C1  - 60333
C2  - 49252
CY  - 530 Walnut Street, Ste 850, Philadelphia, Pa 19106 Usa
SP  - 2294-2296
TI  - Autophagy unleashes noncanonical microRNA functions.
JO  - Autophagy
VL  - 16
IS  - 12
PB  - Taylor & Francis Inc
PY  - 2020
SN  - 1554-8627
ER  - 

TY  - JOUR
AB  - Research in autophagy continues to accelerate,(1) and as a result many new scientists are entering the field. Accordingly, it is important to establish a standard set of criteria for monitoring macroautophagy in different organisms. Recent reviews have described the range of assays that have been used for this purpose.(2,3) There are many useful and convenient methods that can be used to monitor macroautophagy in yeast, but relatively few in other model systems, and there is much confusion regarding acceptable methods to measure macroautophagy in higher eukaryotes. A key point that needs to be emphasized is that there is a difference between measurements that monitor the numbers of autophagosomes versus those that measure flux through the autophagy pathway; thus, a block in macroautophagy that results in autophagosome accumulation needs to be differentiated from fully functional autophagy that includes delivery to, and degradation within, lysosomes (in most higher eukaryotes) or the vacuole (in plants and fungi). Here, we present a set of guidelines for the selection and interpretation of the methods that can be used by investigators who are attempting to examine macroautophagy and related processes, as well as by reviewers who need to provide realistic and reasonable critiques of papers that investigate these processes. This set of guidelines is not meant to be a formulaic set of rules, because the appropriate assays depend in part on the question being asked and the system being used. In addition, we emphasize that no individual assay is guaranteed to be the most appropriate one in every situation, and we strongly recommend the use of multiple assays to verify an autophagic response.
AU  - Klionsky, D.J.*
AU  - Abeliovich, H.*
AU  - Agostinis, P.*
AU  - Agrawal, D.K.*
AU  - Aliev, G.*
AU  - Askew, D.S.*
AU  - Baba, M.*
AU  - Baehrecke, E.H.*
AU  - Bahr, BA.*
AU  - Ballabio, A.*
AU  - Bamber, BA.*
AU  - Bassham, D.C.*
AU  - Bergamini, E.*
AU  - Bi, X.*
AU  - Biard-Piechaczyk, M.*
AU  - Blum, J.S.*
AU  - Bredesen, D.E.*
AU  - Brodsky, J.L.*
AU  - Brumell, J.H.*
AU  - Brunk, U.T.*
AU  - Bursch, W.*
AU  - Camougrand, N.*
AU  - Cebollero, E.*
AU  - Cecconi, F.*
AU  - Chen, Y.*
AU  - Chin, L.S.*
AU  - Choi, A.*
AU  - Chu, C.T.*
AU  - Chung, J.*
AU  - Clarke, P.G.*
AU  - Clark, R.S.*
AU  - Clarke, S.G.*
AU  - Clavé, C.*
AU  - Cleveland, J.L.*
AU  - Codogno, P.*
AU  - Colombo, M.I.*
AU  - Coto-Montes, A.*
AU  - Cregg, J.M.*
AU  - Cuervo, A.M.
AU  - Debnath, J.*
AU  - Demarchi, F.*
AU  - Dennis, P.B.*
AU  - Dennis, P.A.*
AU  - Deretic, V.*
AU  - Devenish, R.J.*
AU  - Di, Sano, F.*
AU  - Dice, J.F.*
AU  - Difiglia, M.*
AU  - Dinesh-Kumar, S.*
AU  - Distelhorst, C.W.*
AU  - Djavaheri-Mergny, M.*
AU  - Dorsey, F.C.*
AU  - Dröge, W.*
AU  - Dron, M.*
AU  - Dunn, W.A, Jr.*
AU  - Duszenko, M.*
AU  - Eissa, N.T.*
AU  - Elazar, Z.*
AU  - Esclatine, A.*
AU  - Eskelinen, E.L.*
AU  - Fésüs, L.*
AU  - Finley, KD.*
AU  - Fuentes, J.M.*
AU  - Fueyo, J.*
AU  - Fujisaki, K.*
AU  - Galliot, B.*
AU  - Gao, F.B.*
AU  - Gewirtz, D.A.*
AU  - Gibson, SB.*
AU  - Gohla, A.*
AU  - Goldberg, A.L.*
AU  - González, R.*
AU  - González-Estévez, C.*
AU  - Gorski, S.*
AU  - Gottlieb, R.A.*
AU  - Häussinger, D.*
AU  - He, Y.W.*
AU  - Heidenreich, K.*
AU  - Hill, J.A.*
AU  - Høyer-Hansen, M.*
AU  - Hu, X.*
AU  - Huang, W.P.*
AU  - Iwasaki, A.*
AU  - Jäättelä, M.*
AU  - Jackson, W.T.*
AU  - Jiang, X.*
AU  - Jin, S.*
AU  - Johansen, T.*
AU  - Jung, J.U.*
AU  - Kadowaki, M.*
AU  - Kang, C.*
AU  - Kelekar, A.*
AU  - Kessel, D.H.*
AU  - Kiel, J.A.*
AU  - Kim, H.P.*
AU  - Kimchi, A.*
AU  - Kinsella, T.J.*
AU  - Kiselyov, K.*
AU  - Kitamoto, K.*
AU  - Knecht, E.*
AU  - Komatsu, M.*
AU  - Kominami, E.*
AU  - Kondo, S.*
AU  - Kovács, A.L.*
AU  - Kroemer, G.*
AU  - Kuan, C.Y.*
AU  - Kumar, R.*
AU  - Kundu, M.*
AU  - Landry, J.*
AU  - Laporte, M.*
AU  - Le, W.*
AU  - Lei, H.Y.*
AU  - Lenardo, M.J.*
AU  - Levine, B.*
AU  - Lieberman, A.*
AU  - Lim, K.L.*
AU  - Lin, F.C.*
AU  - Liou, W.*
AU  - Liu, L.F.*
AU  - Lopez-Berestein, G.*
AU  - López-Otín, C.*
AU  - Lu, B.*
AU  - Macleod, K.F.*
AU  - Malorni, W.*
AU  - Martinet, W.*
AU  - Matsuoka, K.*
AU  - Mautner, J.
AU  - Meijer, A.J.*
AU  - Meléndez, A.*
AU  - Michels, P.*
AU  - Miotto, G.*
AU  - Mistiaen, W.P.*
AU  - Mizushima, N.*
AU  - Mograbi, B.*
AU  - Monastyrska, I.*
AU  - Moore, M.N.*
AU  - Moreira, P.I.*
AU  - Moriyasu, Y.*
AU  - Motyl, T.*
AU  - Münz, C.*
AU  - Murphy, L.O.*
AU  - Naqvi, N.I.*
AU  - Neufeld, T.P.*
AU  - Nishino, I.*
AU  - Nixon, R.A.*
AU  - Noda, T.*
AU  - Nürnberg, B.*
AU  - Ogawa, M.*
AU  - Oleinick, NL.*
AU  - Olsen, LJ.*
AU  - Ozpolat, B.*
AU  - Paglin, S.*
AU  - Palmer, G.E.*
AU  - Papassideri, I.*
AU  - Parkes, M.*
AU  - Perlmutter, D.H.*
AU  - Perry, G.*
AU  - Piacentini, M.*
AU  - Pinkas-Kramarski, R.*
AU  - Prescott, M.*
AU  - Proikas-Cezanne, T.*
AU  - Raben, N.*
AU  - Rami, A.*
AU  - Reggiori, F.*
AU  - Rohrer, B.*
AU  - Rubinsztein, D.C.*
AU  - Ryan, K.M.*
AU  - Sadoshima, J.*
AU  - Sakagami, H.*
AU  - Sakai, Y.*
AU  - Sandri, M.*
AU  - Sasakawa, C.*
AU  - Sass, M.*
AU  - Schneider, C.*
AU  - Seglen, P.O.*
AU  - Seiliez, I.*
AU  - Seleverstov, O.*
AU  - Settleman, J.*
AU  - Shacka, J.J.*
AU  - Shapiro, I.M.*
AU  - Sibirny, A.*
AU  - Silva-Zacarin, E.C.*
AU  - Simon, H.U.*
AU  - Simone, C.*
AU  - Simonsen, A.*
AU  - Smith, M.A.*
AU  - Spanel-Borowski, K.*
AU  - Srinivas, V.*
AU  - Steeves, M.*
AU  - Stenmark, H.*
AU  - Stromhaug, P.E.*
AU  - Subauste, C.S.*
AU  - Sugimoto, S.*
AU  - Sulzer, D.*
AU  - Suzuki, T.*
AU  - Swanson, M.S.*
AU  - Tabas, I.*
AU  - Takeshita, F.*
AU  - Talbot, N.J.*
AU  - Tallóczy, Z.*
AU  - Tanaka, K.*
AU  - Tanida, I.*
AU  - Taylor, G.S.*
AU  - Taylor, J.P.*
AU  - Terman, A.
AU  - Tettamanti, G.*
AU  - Thompson, C.B.*
AU  - Thumm, M.*
AU  - Tolkovsky, A.M.*
AU  - Tooze, S.A.*
AU  - Truant, R.*
AU  - Tumanovska, L.V.*
AU  - Uchiyama, Y.*
AU  - Ueno, T.*
AU  - Uzcátegui, N.L.*
AU  - van der Klei, I.*
AU  - Vaquero, E.C.*
AU  - Vellai, T.*
AU  - Vogel, M.W.*
AU  - Wang, H.G.
AU  - Webster, P.*
AU  - Wiley, J.W.*
AU  - Xi, Z.*
AU  - Xiao, G.*
AU  - Yahalom, J.*
AU  - Yang, J.M.*
AU  - Yap, G.*
AU  - Yin, X.M.*
AU  - Yoshimori, T.*
AU  - Yu, L.*
AU  - Yue, Z.*
AU  - Yuzaki, M.*
AU  - Zabirnyk, O.*
AU  - Zheng, X.*
AU  - Zhu, X.*
AU  - Deter, R.L.*
C1  - 163
C2  - 26024
SP  - 151-175
TI  - Guidelines for the use and interpretation of assays for monitoring autophagy in higher eukaryotes.
JO  - Autophagy
VL  - 4
IS  - 2
PB  - Landes Bioscience
PY  - 2008
SN  - 1554-8627
ER  -