ATAD3B is a human embryonic stem cell specific mitochondrial protein, re-expressed in cancer cells, that functions as dominant negative for the ubiquitous ATAD3A.
Here we report on the identification of a human pluripotent embryonic stem cell (hESC) specific mitochondrial protein that is re-expressed in cancer cells, ATAD3B. ATAD3B belongs to the AAA+ ATPase ATAD3 protein family of mitochondrial proteins specific to multicellular eukaryotes. Using loss- and gain-of-function approaches, we show that ATAD3B associates with the ubiquitous ATAD3A species, negatively regulates the interaction of ATAD3A with matrix nucleoid complexes and contributes to a mitochondria fragmentation phenotype. We conclude that ATAD3B is a negative regulator of ATAD3A and may function as an adaptor of mitochondrial homeostasis and metabolism in hESCs and cancer cells.
INSERM Unité 1038 and INSERM U873, 17 rue des Martyrs, 38054 Grenoble, France.
Nicolas
N
Merle
Olivier
O
Féraud
Benoit
B
Gilquin
Arnaud
A
Hubstenberger
Sylvie
S
Kieffer-Jacquinot
Nicole
N
Assard
Annelise
A
Bennaceur-Griscelli
Jérôme
J
Honnorat
Jacques
J
Baudier
D000074183:ATPases Associated with Diverse Cellular Activities; D000251:Adenosine Triphosphatases / Q000235:genetics / Q000378:metabolism*; D045744:Cell Line, Tumor; D053595:Embryonic Stem Cells / Q000502:physiology*; D006801:Humans; D008565:Membrane Proteins / Q000235:genetics / Q000378:metabolism*; D024101:Mitochondrial Proteins / Q000235:genetics / Q000378:metabolism*; D050505:Mutant Proteins / Q000096:biosynthesis / Q000235:genetics; D009154:Mutation; D009369:Neoplasms / Q000503:physiopathology*
C549686:ATAD3A protein, human; C519737:ATAD3B protein, human; D008565:Membrane Proteins; D024101:Mitochondrial Proteins; D050505:Mutant Proteins; D000251:Adenosine Triphosphatases; D000074183:ATPases Associated with Diverse Cellular Activities