TY - JOUR
T1 - Eight human OPA1 isoforms, long and short
T2 - What are they for?
AU - Del Dotto, Valentina
AU - Fogazza, Mario
AU - Carelli, Valerio
AU - Rugolo, Michela
AU - Zanna, Claudia
N1 - Ricercatore distaccato presso IRCCS a seguito Convenzione esclusiva con Università di Bologna (Carelli Valerio)
PY - 2018/4/1
Y1 - 2018/4/1
N2 - OPA1 is a dynamin-related GTPase that controls mitochondrial dynamics, cristae integrity, energetics and mtDNA maintenance. The exceptional complexity of this protein is determined by the presence, in humans, of eight different isoforms that, in turn, are proteolytically cleaved into combinations of membrane-anchored long forms and soluble short forms. Recent advances highlight how each OPA1 isoform is able to fulfill “essential” mitochondrial functions, whereas only some variants carry out “specialized” features. Long forms determine fusion, long or short forms alone build cristae, whereas long and short forms together tune mitochondrial morphology. These findings offer novel challenging therapeutic potential to gene therapy.
AB - OPA1 is a dynamin-related GTPase that controls mitochondrial dynamics, cristae integrity, energetics and mtDNA maintenance. The exceptional complexity of this protein is determined by the presence, in humans, of eight different isoforms that, in turn, are proteolytically cleaved into combinations of membrane-anchored long forms and soluble short forms. Recent advances highlight how each OPA1 isoform is able to fulfill “essential” mitochondrial functions, whereas only some variants carry out “specialized” features. Long forms determine fusion, long or short forms alone build cristae, whereas long and short forms together tune mitochondrial morphology. These findings offer novel challenging therapeutic potential to gene therapy.
KW - Cristae
KW - Energetics
KW - Long and short OPA1 forms
KW - Mitochondrial network dynamics
KW - mtDNA
KW - OPA1
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U2 - 10.1016/j.bbabio.2018.01.005
DO - 10.1016/j.bbabio.2018.01.005
M3 - Review article
C2 - 29382469
AN - SCOPUS:85041623765
VL - 1859
SP - 263
EP - 269
JO - Biochimica et Biophysica Acta - Bioenergetics
JF - Biochimica et Biophysica Acta - Bioenergetics
SN - 0005-2728
IS - 4
ER -