ATP Synthase c-Subunit Leak Causes Aberrant Cellular Metabolism in Fragile X Syndrome

Pawel Licznerski; Han A. Park; Harshvardhan Rolyan; Rongmin Chen; Nelli Mnatsakanyan; Paige Miranda; Morven Graham; Jing Wu; Nicole Cruz-Reyes; Nikita Mehta; Sana Sohail; Jorge Salcedo; Erin Song; Charles Effman; Samuel Effman; Lucas Brandao; Gulan N. Xu; Amber Braker; Valentin K. Gribkoff; Richard J. Levy; Elizabeth A. Jonas

doi:10.1016/j.cell.2020.07.008

ATP Synthase c-Subunit Leak Causes Aberrant Cellular Metabolism in Fragile X Syndrome

Pawel Licznerski, Han A. Park, Harshvardhan Rolyan, Rongmin Chen, Nelli Mnatsakanyan, Paige Miranda, Morven Graham, Jing Wu, Nicole Cruz-Reyes, Nikita Mehta, Sana Sohail, Jorge Salcedo, Erin Song, Charles Effman, Samuel Effman, Lucas Brandao, Gulan N. Xu, Amber Braker, Valentin K. Gribkoff, Richard J. LevyElizabeth A. Jonas

Department of Cellular and Molecular Physiology

Research output: Contribution to journal › Article › peer-review

64 Scopus citations

Abstract

Lack of FMRP in Fragile X neurons is associated with a leak in the ATP synthase, the blockade of which normalizes cellular and behavioral disease phenotypes.

Original language	English (US)
Pages (from-to)	1170-1185.e9
Journal	Cell
Volume	182
Issue number	5
DOIs	https://doi.org/10.1016/j.cell.2020.07.008
State	Published - Sep 3 2020

All Science Journal Classification (ASJC) codes

General Biochemistry, Genetics and Molecular Biology

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10.1016/j.cell.2020.07.008

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Licznerski, P., Park, H. A., Rolyan, H., Chen, R., Mnatsakanyan, N., Miranda, P., Graham, M., Wu, J., Cruz-Reyes, N., Mehta, N., Sohail, S., Salcedo, J., Song, E., Effman, C., Effman, S., Brandao, L., Xu, G. N., Braker, A., Gribkoff, V. K., ... Jonas, E. A. (2020). ATP Synthase c-Subunit Leak Causes Aberrant Cellular Metabolism in Fragile X Syndrome. Cell, 182(5), 1170-1185.e9. https://doi.org/10.1016/j.cell.2020.07.008

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title = "ATP Synthase c-Subunit Leak Causes Aberrant Cellular Metabolism in Fragile X Syndrome",

abstract = "Lack of FMRP in Fragile X neurons is associated with a leak in the ATP synthase, the blockade of which normalizes cellular and behavioral disease phenotypes.",

author = "Pawel Licznerski and Park, {Han A.} and Harshvardhan Rolyan and Rongmin Chen and Nelli Mnatsakanyan and Paige Miranda and Morven Graham and Jing Wu and Nicole Cruz-Reyes and Nikita Mehta and Sana Sohail and Jorge Salcedo and Erin Song and Charles Effman and Samuel Effman and Lucas Brandao and Xu, {Gulan N.} and Amber Braker and Gribkoff, {Valentin K.} and Levy, {Richard J.} and Jonas, {Elizabeth A.}",

note = "Publisher Copyright: {\textcopyright} 2020 Elsevier Inc.",

year = "2020",

month = sep,

day = "3",

doi = "10.1016/j.cell.2020.07.008",

language = "English (US)",

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Licznerski, P, Park, HA, Rolyan, H, Chen, R, Mnatsakanyan, N, Miranda, P, Graham, M, Wu, J, Cruz-Reyes, N, Mehta, N, Sohail, S, Salcedo, J, Song, E, Effman, C, Effman, S, Brandao, L, Xu, GN, Braker, A, Gribkoff, VK, Levy, RJ & Jonas, EA 2020, 'ATP Synthase c-Subunit Leak Causes Aberrant Cellular Metabolism in Fragile X Syndrome', Cell, vol. 182, no. 5, pp. 1170-1185.e9. https://doi.org/10.1016/j.cell.2020.07.008

TY - JOUR

T1 - ATP Synthase c-Subunit Leak Causes Aberrant Cellular Metabolism in Fragile X Syndrome

AU - Licznerski, Pawel

AU - Park, Han A.

AU - Rolyan, Harshvardhan

AU - Chen, Rongmin

AU - Mnatsakanyan, Nelli

AU - Miranda, Paige

AU - Graham, Morven

AU - Wu, Jing

AU - Cruz-Reyes, Nicole

AU - Mehta, Nikita

AU - Sohail, Sana

AU - Salcedo, Jorge

AU - Song, Erin

AU - Effman, Charles

AU - Effman, Samuel

AU - Brandao, Lucas

AU - Xu, Gulan N.

AU - Braker, Amber

AU - Gribkoff, Valentin K.

AU - Levy, Richard J.

AU - Jonas, Elizabeth A.

PY - 2020/9/3

Y1 - 2020/9/3

N2 - Lack of FMRP in Fragile X neurons is associated with a leak in the ATP synthase, the blockade of which normalizes cellular and behavioral disease phenotypes.

AB - Lack of FMRP in Fragile X neurons is associated with a leak in the ATP synthase, the blockade of which normalizes cellular and behavioral disease phenotypes.

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U2 - 10.1016/j.cell.2020.07.008

DO - 10.1016/j.cell.2020.07.008

M3 - Article

C2 - 32795412

AN - SCOPUS:85090000245

SN - 0092-8674

VL - 182

SP - 1170-1185.e9

JO - Cell

JF - Cell

IS - 5

ER -

ATP Synthase c-Subunit Leak Causes Aberrant Cellular Metabolism in Fragile X Syndrome

Abstract

All Science Journal Classification (ASJC) codes

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