Transcriptional activation of LON Gene by a new form of mitochondrial stress: A role for the nuclear respiratory factor 2 in StAR overload response (SOR)

Molecular and Cellular Endocrinology
Assaf BahatJ Orly

Abstract

High output of steroid hormone synthesis in steroidogenic cells of the adrenal cortex and the gonads requires the expression of the steroidogenic acute regulatory protein (StAR) that facilitates cholesterol mobilization to the mitochondrial inner membrane where the CYP11A1/P450scc enzyme complex converts the sterol to the first steroid. Earlier studies have shown that StAR is active while pausing on the cytosolic face of the outer mitochondrial membrane while subsequent import of the protein into the matrix terminates the cholesterol mobilization activity. Consequently, during repeated activity cycles, high level of post-active StAR accumulates in the mitochondrial matrix. To prevent functional damage due to such protein overload effect, StAR is degraded by a sequence of three to four ATP-dependent proteases of the mitochondria protein quality control system, including LON and the m-AAA membranous proteases AFG3L2 and SPG7/paraplegin. Furthermore, StAR expression in both peri-ovulatory ovarian cells, or under ectopic expression in cell line models, results in up to 3-fold enrichment of the mitochondrial proteases and their transcripts. We named this novel form of mitochondrial stress as StAR overload response (SOR). To better u...Continue Reading

References

Jul 1, 1983·Canadian Journal of Biochemistry and Cell Biology = Revue Canadienne De Biochimie Et Biologie Cellulaire·E R Simpson, M R Waterman
Dec 1, 1993·Proceedings of the National Academy of Sciences of the United States of America·N WangM R Maurizi
Jun 1, 1996·Endocrine Reviews·D M Stocco, B J Clark
Nov 1, 1996·The Journal of Clinical Endocrinology and Metabolism·M KiriakidouJ F Strauss
Nov 26, 1996·Proceedings of the National Academy of Sciences of the United States of America·F ArakaneJ F Strauss
Dec 19, 1996·The New England Journal of Medicine·H S BoseUNKNOWN International Congenital Lipoid Adrenal Hyperplasia Consortium
Jun 1, 1997·Journal of Cellular Biochemistry·D CurcićB H Sells
May 3, 2002·Nature·Himangshu S BoseWalter L Miller
Aug 3, 2002·Nature Reviews. Molecular Cell Biology·Walter Neupert, Michael Brunner
Aug 29, 2002·The EMBO Journal·Quan ZhaoNicholas J Hoogenraad
Aug 29, 2002·Nature Cell Biology·Daniela A Bota, Kelvin J A Davies
Oct 31, 2003·Molecular and Cellular Biology·Morten SunesenJean-Pierre Changeux
Jan 15, 2004·The Journal of Clinical Investigation·Fatima FerreirinhaElena I Rugarli
Mar 16, 2004·The European Journal of Neuroscience·Shou Jing YangMargaret T T Wong-Riley
Mar 24, 2004·Journal of Structural Biology·Axel MogkBernd Bukau
Sep 30, 2004·Cell·Robert T SauerTania A Baker
Nov 24, 2004·The Journal of Biological Chemistry·Steffen AugustinThomas Langer
Feb 3, 2005·Free Radical Biology & Medicine·Daniela A BotaKelvin J A Davies
May 5, 2005·The Journal of Biological Chemistry·Gabriela OndrovicováCarolyn K Suzuki
Aug 2, 2005·Cell Metabolism·Jiandie LinBruce M Spiegelman
Jan 16, 2007·Molecular and Cellular Endocrinology·Zvi GranotJoseph Orly
Jan 18, 2007·Annual Review of Biochemistry·Michael T Ryan, Nicholas J Hoogenraad

❮ Previous
Next ❯

Citations

Mar 10, 2016·Nature Reviews. Molecular Cell Biology·Pedro M QuirósJohan Auwerx
Sep 14, 2015·Cellular and Molecular Life Sciences : CMLS·Marcello PintiAndrea Cossarizza
Aug 16, 2016·Molecular and Cellular Endocrinology·Jinwoo LeeColin Jefcoate
Oct 30, 2016·Frontiers in Cell and Developmental Biology·Iryna Bohovych, Oleh Khalimonchuk
Feb 13, 2020·Biomolecules·Wolfgang Voos, Karen Pollecker
May 4, 2018·Archives of Toxicology·Jieyeqi YangXiao Li Shen
Jun 20, 2019·Biochemistry. Biokhimii︠a︡·K V Tugaeva, N N Sluchanko

❮ Previous
Next ❯

Related Concepts

Related Feeds

Aortic Aneurysm

An aortic aneurysm is the weakening and bulging of the blood vessel wall in the aorta. This causes dilatation of the aorta, which is usually asymptomatic but carries the risk of rupture and hemorrhage. Find the latest research on aortic aneurysms here.

Aneurysm

Aneurysms are outward distensions or bulges that occurs in a weakened wall of blood vessels. Discover the latest research on aneurysms here.