Results 11 to 20 of about 2,342 (176)

Dual role of Mic10 in mitochondrial cristae organization and ATP synthase-linked metabolic adaptation and respiratory growth

Cell Reports, 2022
Summary: Invaginations of the mitochondrial inner membrane, termed cristae, are hubs for oxidative phosphorylation. The mitochondrial contact site and cristae organizing system (MICOS) and the dimeric F1Fo-ATP synthase play important roles in controlling
Heike Rampelt, Florian Wollweber, Mariya Licheva, Rinse de Boer, Inge Perschil, Liesa Steidle, Thomas Becker, Maria Bohnert, Ida van der Klei, Claudine Kraft, Martin van der Laan, Nikolaus Pfanner   +11 more
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CARD19 Interacts with Mitochondrial Contact Site and Cristae Organizing System Constituent Proteins and Regulates Cristae Morphology

Cells, 2022
CARD19 is a mitochondrial protein of unknown function. While CARD19 was originally reported to regulate TCR-dependent NF-κB activation via interaction with BCL10, this function is not recapitulated ex vivo in primary murine CD8+ T cells.
Kariana E. Rios, Ming Zhou, Nathaniel M. Lott, Chelsi R. Beauregard, Dennis P. McDaniel, Thomas P. Conrads, Brian C. Schaefer   +6 more
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MICOS coordinates with respiratory complexes and lipids to establish mitochondrial inner membrane architecture

eLife, 2015
The conserved MICOS complex functions as a primary determinant of mitochondrial inner membrane structure. We address the organization and functional roles of MICOS and identify two independent MICOS subcomplexes: Mic27/Mic10/Mic12, whose assembly is ...
Jonathan R Friedman, Arnaud Mourier, Justin Yamada, J Michael McCaffery, Jodi Nunnari   +4 more
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QIL1 mutation causes MICOS disassembly and early onset fatal mitochondrial encephalopathy with liver disease

eLife, 2016
Previously, we identified QIL1 as a subunit of mitochondrial contact site (MICOS) complex and demonstrated a role for QIL1 in MICOS assembly, mitochondrial respiration, and cristae formation critical for mitochondrial architecture (Guarani et al., 2015).
Virginia Guarani, Claude Jardel, Dominique Chrétien, Anne Lombès, Paule Bénit, Clémence Labasse, Emmanuelle Lacène, Agnès Bourillon, Apolline Imbard, Jean-François Benoist, Imen Dorboz, Mylène Gilleron, Eric S Goetzman, Pauline Gaignard, Abdelhamid Slama, Monique Elmaleh-Bergès, Norma B Romero, Pierre Rustin, Hélène Ogier de Baulny, Joao A Paulo, J Wade Harper, Manuel Schiff   +21 more
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CHCHD2 maintains mitochondrial contact site and cristae organizing system stability and protects against mitochondrial dysfunction in an experimental model of Parkinson's disease

Chinese Medical Journal, 2022
Background:. Parkinson's disease (PD) is the second most common neurodegenerative disease after Alzheimer's dementia. Mitochondrial dysfunction is involved in the pathology of PD.
Lin Lu, Hengxu Mao, Miaomiao Zhou, Yuwan Lin, Wei Dai, Jiewen Qiu, Yousheng Xiao, Mingshu Mo, Xiaoqin Zhu, Zhuohua Wu, Zhong Pei, Wenyuan Guo, Pingyi Xu, Xiang Chen, Yuanyuan Ji   +14 more
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QIL1 is a novel mitochondrial protein required for MICOS complex stability and cristae morphology

eLife, 2015
The mitochondrial contact site and cristae junction (CJ) organizing system (MICOS) dynamically regulate mitochondrial membrane architecture. Through systematic proteomic analysis of human MICOS, we identified QIL1 (C19orf70) as a novel conserved MICOS ...
Virginia Guarani, Elizabeth M McNeill, Joao A Paulo, Edward L Huttlin, Florian Fröhlich, Steven P Gygi, David Van Vactor, J Wade Harper   +7 more
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Altered MICOS Morphology and Mitochondrial Ion Homeostasis Contribute to Poly(GR) Toxicity Associated with C9-ALS/FTD

Cell Reports, 2020
Summary: Amyotrophic lateral sclerosis (ALS) manifests pathological changes in motor neurons and various other cell types. Compared to motor neurons, the contribution of the other cell types to the ALS phenotypes is understudied. G4C2 repeat expansion in
Shuangxi Li, Zhihao Wu, Yu Li, Ishaq Tantray, Diego De Stefani, Andrea Mattarei, Gopinath Krishnan, Fen-Biao Gao, Hannes Vogel, Bingwei Lu   +9 more
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Mic13 Is Essential for Formation of Crista Junctions in Mammalian Cells. [PDF]

PLoS ONE, 2016
Mitochondrial cristae are connected to the inner boundary membrane via crista junctions which are implicated in the regulation of oxidative phosphorylation, apoptosis, and import of lipids and proteins.
Ruchika Anand, Valentina Strecker, Jennifer Urbach, Ilka Wittig, Andreas S Reichert   +4 more
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La obesidad materna: mecanismos genómicos y no genómicos de transmisión inter- o transgeneracional

Revista de la Alad. Asociación Latinoamericana de Diabetes, 2023
La obesidad es el problema de salud más común en las mujeres en edad reproductiva. La obesidad durante el embarazo genera desenlaces adversos para las mujeres en esta etapa, el parto y el posparto, y afecta negativamente al feto y a las etapas neonatal e
Ana L. Elías-López, Óscar Vázquez-Mena   +1 more
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Protocol for generating reproducible miniaturized controlled midbrain organoids

STAR Protocols, 2023
Summary: Here, we present a protocol for generating miniaturized controlled midbrain organoids (MiCOs) of reproducible size and cellular composition, without a necrotic center. We describe steps for maintaining and passaging human pluripotent stem cells,
Muwan Chen, Jonathan Christos Niclis, Mark Denham   +2 more
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