Explanipedia

Advanced AI-enabled technologies for sustainable biohydrogen production and environmental stewardship Open

Muthukumar Paramasivan, Thanikanti Sudhakar Babu, G. Flora, Ruchika Anand, K Vanchinathan , et al. · 2025

SLP2 and MIC13 synergistically coordinate MICOS assembly and crista junction formation Open

Ritam Naha, Rebecca Strohm, Yulia Schaumkessel, Jennifer Urbach, Ilka Wittig , et al. · 2024

The MICOS complex, essential for cristae organization, comprises MIC10 and MIC60 subcomplexes, with MIC13 as a crucial subunit. MIC13 mutations cause severe mitochondrial hepato-encephalopathy, cristae defects, and MIC10-subcomplex …

Mitochondrial apolipoprotein MIC26 is a metabolic rheostat regulating central cellular fuel pathways Open

Melissa Damiecki, Ritam Naha, Yulia Schaumkessel, Philipp Westhoff, Nika Atanelov , et al. · 2024

Mitochondria play central roles in metabolism and metabolic disorders such as type 2 diabetes. MIC26, a mitochondrial contact site and cristae organising system complex subunit, was linked to diabetes and modulation of lipid metabolism. Ye…

Mutant huntingtin impairs neurodevelopment in human brain organoids through CHCHD2-mediated neurometabolic failure Open

Paweł Lisowski, Selene Lickfett, Agnieszka Rybak‐Wolf, Carmen Menacho, Stephanie Le , et al. · 2024

Cortical brain organoid slices (cBOS) for the study of human neural cells in minimal networks Open

Laura Petersilie, Sonja Heiduschka, Joel S.E. Nelson, Louis A. Neu, Stephanie Le , et al. · 2024

Brain organoids derived from human pluripotent stem cells are a promising tool for studying human neurodevelopment and related disorders. Here, we generated long-term cultures of cortical brain organoid slices (cBOS) grown at the air-liqui…

Mitochondrial Apolipoprotein MIC26 is a metabolic rheostat regulating central cellular fuel pathways Open

Melissa Lubeck, Ritam Naha, Yulia Schaumkessel, Philipp Westhoff, Anja Stefanski , et al. · 2023

Summary Mitochondria play central roles in metabolism and metabolic disorders such as type 2 diabetes. MIC26, a MICOS complex subunit, was linked to diabetes and modulation of lipid metabolism. Yet, the functional role of MIC26 in regulati…

Cristae dynamics is modulated in bioenergetically compromised mitochondria Open

Mathias Golombek, Thanos Tsigaras, Yulia Schaumkessel, Sebastian Hänsch, Stefanie Weidtkamp‐Peters , et al. · 2023

Cristae membranes have been recently shown to undergo intramitochondrial merging and splitting events. Yet, the metabolic and bioenergetic factors regulating them are unclear. Here, we investigated whether and how cristae morphology and dy…

NECAB2 is an endosomal protein important for striatal function Open

Diones Bueno, Partha Narayan Dey, Teresa Schacht, Christina Wolf, Verena Wüllner , et al. · 2023

Synaptic signaling depends on ATP generated by mitochondria. Dysfunctional mitochondria shift the redox balance towards a more oxidative environment. Due to extensive connectivity, the striatum is especially vulnerable to mitochondrial dys…

MIC13 and SLP2 seed the assembly of MIC60-subcomplex to facilitate crista junction formation Open

Ritam Naha, Rebecca Strohm, Jennifer Urbach, Ilka Wittig, Andreas S. Reichert , et al. · 2023

The MICOS complex subunit MIC13 is essential for mitochondrial cristae organization. Mutations in MIC13 cause severe mitochondrial hepato-encephalopathy displaying defective cristae morphology and loss of the MIC10-subcomplex. Here we iden…

A X‐linked nonsense <i>APOO/MIC26</i> variant causes a lethal mitochondrial disease with progeria‐like phenotypes Open

Leon Peifer‐Weiß, Mazen Kurban, Céline David, Melissa Lubeck, Arun Kumar Kondadi , et al. · 2023

APOO/MIC26 is a subunit of the MICOS complex required for mitochondrial cristae morphology and function. Here, we report a novel variant of the APOO/MIC26 gene that causes a severe mitochondrial disease with overall progeria‐like phenotype…

Molecular and cellular evidence for the impact of a hypertrophic cardiomyopathy-associated RAF1 variant on the structure and function of contractile machinery in bioartificial cardiac tissues Open

Saeideh Nakhaei‐Rad, Fereshteh Haghighi, Farhad Bazgir, Julia Dahlmann, Alexandra Viktoria Busley , et al. · 2023

Noonan syndrome (NS), the most common among RASopathies, is caused by germline variants in genes encoding components of the RAS-MAPK pathway. Distinct variants, including the recurrent Ser257Leu substitution in RAF1, are associated with se…

MIC26 and MIC27 are bona fide subunits of the MICOS complex in mitochondria and do not exist as glycosylated apolipoproteins Open

Melissa Lubeck, Nick H. Derkum, Ritam Naha, Rebecca Strohm, Marc D. Drießen , et al. · 2023

Impairments of mitochondrial functions are linked to human ageing and pathologies such as cancer, cardiomyopathy, neurodegeneration and diabetes. Specifically, aberrations in ultrastructure of mitochondrial inner membrane (IM) and factors …

Live-cell super-resolution nanoscopy reveals modulation of cristae dynamics in bioenergetically compromised mitochondria Open

Mathias Golombek, Thanos Tsigaras, Yulia Schaumkessel, Sebastian Hänsch, Stefanie Weidtkamp‐Peters , et al. · 2023

Cristae membranes have been recently shown to undergo intramitochondrial merging and splitting events. Yet, the metabolic and bioenergetic factors regulating them are unclear. Here we investigated whether and how cristae membrane remodelli…

Mesenchymal stem cells improve redox homeostasis and mitochondrial respiration in fibroblast cell lines with pathogenic MT-ND3 and MT-ND6 variants Open

Tharsini Navaratnarajah, Marlen Bellmann, Annette Seibt, Ruchika Anand, Özer Degistirici , et al. · 2022

The most frequent biochemical defect of inherited mitochondrial disease is isolated complex I deficiency. There is no cure for this disorder, and treatment is mainly supportive. In this study, we investigated the effects of human mesenchym…

Alteration of myocardial structure and function in RAF1-associated Noonan syndrome: Insights from cardiac disease modeling based on patient-derived iPSCs Open

Saeideh Nakhaei‐Rad, Farhad Bazgir, Julia Dahlmann, Alexandra Viktoria Busley, Marcel Buchholzer , et al. · 2022

Noonan syndrome (NS), the most common among the RASopathies, is caused by germline variants in genes encoding components of the RAS-MAPK pathway. Distinct variants, including the recurrent Ser257Leu substitution in RAF1, are associated wit…

Emerging Roles of the MICOS Complex in Cristae Dynamics and Biogenesis Open

Ruchika Anand, Andreas S. Reichert, Arun Kumar Kondadi · 2021

Mitochondria are double membrane-enclosed organelles performing important cellular and metabolic functions such as ATP generation, heme biogenesis, apoptosis, ROS production and calcium buffering. The mitochondrial inner membrane (IM) is f…

High-throughput screening for natural compound-based autophagy modulators reveals novel chemotherapeutic mode of action for arzanol Open

Jana Deitersen, Lena Berning, Fabian Stuhldreier, Sara Ceccacci, David Schlütermann , et al. · 2021

Autophagy is an intracellular recycling pathway with implications for intracellular homeostasis and cell survival. Its pharmacological modulation can aid chemotherapy by sensitizing cancer cells toward approved drugs and overcoming chemore…

NECAB2 participates in an endosomal pathway of mitochondrial stress response at striatal synapses Open

Diones Bueno, Partha Narayan Dey, Teresa Schacht, Christina Wolf, Verena Wüllner , et al. · 2021

Synaptic signaling depends on ATP generated by mitochondria. Due to extensive connectivity, the striatum is especially vulnerable to mitochondrial dysfunction and thus requires efficient mitochondrial quality control and repair. We found t…

Protease OMA1 modulates mitochondrial bioenergetics and ultrastructure through dynamic association with MICOS complex Open

Martônio Ponte Viana, Roman M. Levytskyy, Ruchika Anand, Andreas S. Reichert, Oleh Khalimonchuk · 2021

Protease OMA1 Modulates Mitochondrial Bioenergetics and Ultrastructure through Dynamic Association with MICOS Complex Open

Martônio Ponte Viana, Roman M. Levytskyy, Ruchika Anand, Andreas S. Reichert, Oleh Khalimonchuk · 2020

Remodeling of mitochondrial ultrastructure is a complex dynamic process that is critical for a variety of mitochondrial functions and apoptosis. Although the key regulators of this process - mitochondrial contact site and cristae junction …

Cristae Membrane Dynamics – A Paradigm Change Open

Arun Kumar Kondadi, Ruchika Anand, Andreas S. Reichert · 2020

MIC26 and MIC27 cooperate to regulate cardiolipin levels and the landscape of OXPHOS complexes Open

Ruchika Anand, Arun Kumar Kondadi, Jana Meisterknecht, Mathias Golombek, Oliver Nortmann , et al. · 2020

Homologous apolipoproteins of MICOS complex, MIC26 and MIC27, show an antagonistic regulation of their protein levels, making it difficult to deduce their individual functions using a single gene deletion. We obtained single and double kno…

Cristae undergo continuous cycles of membrane remodelling in a <span>MICOS</span> ‐dependent manner Open

Arun Kumar Kondadi, Ruchika Anand, Sebastian Hänsch, Jennifer Urbach, Thomas Zobel , et al. · 2020

The mitochondrial inner membrane can reshape under different physiological conditions. How, at which frequency this occurs in living cells, and the molecular players involved are unknown. Here, we show using state-of-the-art live-cell stim…

Individual cristae within the same mitochondrion display different membrane potentials and are functionally independent Open

Dane M. Wolf, Mayuko Segawa, Arun Kumar Kondadi, Ruchika Anand, Sean T. Bailey , et al. · 2019

Functional Interplay between Cristae Biogenesis, Mitochondrial Dynamics and Mitochondrial DNA Integrity Open

Arun Kumar Kondadi, Ruchika Anand, Andreas S. Reichert · 2019

Mitochondria are vital cellular organelles involved in a plethora of cellular processes such as energy conversion, calcium homeostasis, heme biogenesis, regulation of apoptosis and ROS reactive oxygen species (ROS) production. Although the…

Cristae undergo continuous cycles of fusion and fission in a MICOS-dependent manner Open

Arun Kumar Kondadi, Ruchika Anand, Sebastian Hänsch, Jennifer Urbach, Thomas Zobel , et al. · 2019

The mitochondrial inner membrane can reshape under different physiological conditions. How and at which frequency this occurs in vivo and what are the molecular players involved is unknown. Here we show using state-of-the-art live-cell sti…

Correction: SIRT4 interacts with OPA1 and regulates mitochondrial quality control and mitophagy Open

Alexander Lang, Ruchika Anand, Simone Altinoluk-Hambüchen, Hakima Ezzahoini, Anja Stefanski , et al. · 2018

The mycotoxin phomoxanthone A disturbs the form and function of the inner mitochondrial membrane Open

Philip Böhler, Fabian Stuhldreier, Ruchika Anand, Arun Kumar Kondadi, David Schlütermann , et al. · 2018

Mitochondria are cellular organelles with crucial functions in the generation and distribution of ATP, the buffering of cytosolic Ca 2+ and the initiation of apoptosis. Compounds that interfere with these functions are termed mitochondrial…

SIRT4 interacts with OPA1 and regulates mitochondrial quality control and mitophagy Open

Alexander Lang, Ruchika Anand, Simone Altinoluk-Hambüchen, Hakima Ezzahoini, Anja Stefanski , et al. · 2017

The stress-responsive mitochondrial sirtuin SIRT4 controls cellular energy metabolism in a NAD⁺-dependent manner and is implicated in cellular senescence and aging. Here we reveal a novel function of SIRT4 in mitochondrial morph…

Mic13 Is Essential for Formation of Crista Junctions in Mammalian Cells Open

Ruchika Anand, Valentina Strecker, Jennifer Urbach, Ilka Wittig, Andreas S. Reichert · 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. The MICOS complex determines formati…

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