[HTML][HTML] Mitochondrial dysfunction and oxidative stress in heart disease

…, A Saraf, N Ghazal, TT Pham, JQ Kwong - … & molecular medicine, 2019 - nature.com
Beyond their role as a cellular powerhouse, mitochondria are emerging as integral players
in molecular signaling and cell fate determination through reactive oxygen species (ROS). …

[PDF][PDF] Physiological and pathological roles of the mitochondrial permeability transition pore in the heart

JQ Kwong, JD Molkentin - Cell metabolism, 2015 - cell.com
Prolonged mitochondrial permeability transition pore (MPTP) opening results in mitochondrial
energetic dysfunction, organelle swelling, rupture, and typically a type of necrotic cell …

Rescue of a deficiency in ATP synthesis by transfer of MTATP6, a mitochondrial DNA-encoded gene, to the nucleus

G Manfredi, J Fu, J Ojaimi, JE Sadlock, JQ Kwong… - Nature …, 2002 - nature.com
AT→ G transversion at nt 8993 in mitochondrial DNA of MTATP6 (encoding ATPase 6 of
complex V of the respiratory chain) causes impaired mitochondrial ATP synthesis in two related …

The mitochondrial respiratory chain is a modulator of apoptosis

JQ Kwong, MS Henning, AA Starkov… - The Journal of cell …, 2007 - rupress.org
Mitochondrial dysfunction and dysregulation of apoptosis are implicated in many diseases
such as cancer and neurodegeneration. We investigate here the role of respiratory chain (RC…

[PDF][PDF] The mitochondrial calcium uniporter selectively matches metabolic output to acute contractile stress in the heart

JQ Kwong, X Lu, RN Correll, JA Schwanekamp… - Cell reports, 2015 - cell.com
In the heart, augmented Ca 2+ fluxing drives contractility and ATP generation through
mitochondrial Ca 2+ loading. Pathologic mitochondrial Ca 2+ overload with ischemic injury …

Positive contribution of pathogenic mutations in the mitochondrial genome to the promotion of cancer by prevention from apoptosis

…, K Yamagata, T Kanamori, K Nakano, JQ Kwong… - Cancer research, 2005 - AACR
The role of mitochondrial dysfunction in cancer has been a subject of great interest and
much ongoing investigation. Although most cancer cells harbor somatic mutations in …

Bax and Bak function as the outer membrane component of the mitochondrial permeability pore in regulating necrotic cell death in mice

J Karch, JQ Kwong, AR Burr, MA Sargent, JW Elrod… - elife, 2013 - elifesciences.org
10.7554/eLife.00772.001 A critical event in ischemia-based cell death is the opening of the
mitochondrial permeability transition pore (MPTP). However, the molecular identity of the …

The role of mitochondria in inherited neurodegenerative diseases

JQ Kwong, MF Beal, G Manfredi - Journal of neurochemistry, 2006 - Wiley Online Library
In the past decade, the genetic causes underlying familial forms of many neurodegenerative
disorders, such as Huntington's disease, Parkinson's disease, Alzheimer's disease, …

Individual cardiac mitochondria undergo rare transient permeability transition pore openings

X Lu, JQ Kwong, JD Molkentin, DM Bers - Circulation research, 2016 - Am Heart Assoc
Rationale: Mitochondria produce ATP, especially critical for survival of highly aerobic cells,
such as cardiac myocytes. Conversely, opening of mitochondrial high-conductance and long-…

[HTML][HTML] The mammalian phosphate carrier SLC25A3 is a mitochondrial copper transporter required for cytochrome c oxidase biogenesis

…, SE Cole, X Zhu, CB Phillips, JQ Kwong… - Journal of Biological …, 2018 - ASBMB
Copper is required for the activity of cytochrome c oxidase (COX), the terminal electron-accepting
complex of the mitochondrial respiratory chain. The likely source of copper used for …