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  • Feature Paper
  • Review
  • Open Access
134 Citations
14,193 Views
15 Pages

Mitochondrial Dynamics in Mitochondrial Diseases

  • Juan M. Suárez-Rivero,
  • Marina Villanueva-Paz,
  • Patricia De la Cruz-Ojeda,
  • Mario De la Mata,
  • David Cotán,
  • Manuel Oropesa-Ávila,
  • Isabel De Lavera,
  • Mónica Álvarez-Córdoba,
  • Raquel Luzón-Hidalgo and
  • José A. Sánchez-Alcázar

23 December 2016

Mitochondria are very versatile organelles in continuous fusion and fission processes in response to various cellular signals. Mitochondrial dynamics, including mitochondrial fission/fusion, movements and turnover, are essential for the mitochondrial...

  • Review
  • Open Access
75 Citations
9,864 Views
13 Pages

Mitochondrial Transplantation as a Novel Therapeutic Strategy for Mitochondrial Diseases

  • Anna Park,
  • Mihee Oh,
  • Su Jeong Lee,
  • Kyoung-Jin Oh,
  • Eun-Woo Lee,
  • Sang Chul Lee,
  • Kwang-Hee Bae,
  • Baek Soo Han and
  • Won Kon Kim

Mitochondria are the major source of intercellular bioenergy in the form of ATP. They are necessary for cell survival and play many essential roles such as maintaining calcium homeostasis, body temperature, regulation of metabolism and apoptosis. Mit...

  • Review
  • Open Access
18 Citations
5,782 Views
27 Pages

11 September 2022

Mitochondria are small double-membraned organelles responsible for the generation of energy used in the body in the form of ATP. Mitochondria are unique in that they contain their own circular mitochondrial genome termed mtDNA. mtDNA codes for 37 gen...

  • Review
  • Open Access
65 Citations
9,304 Views
42 Pages

26 August 2020

Mitochondria are complex organelles that harbour their own genome. Mitochondrial DNA (mtDNA) exists in the form of a circular double-stranded DNA molecule that must be replicated, segregated and distributed around the mitochondrial network. Human cel...

  • Article
  • Open Access
31 Citations
6,879 Views
26 Pages

Quantifying Mitochondrial Dynamics in Patient Fibroblasts with Multiple Developmental Defects and Mitochondrial Disorders

  • Ajibola B. Bakare,
  • Julienne Daniel,
  • Joshua Stabach,
  • Anapaula Rojas,
  • Austin Bell,
  • Brooke Henry and
  • Shilpa Iyer

Mitochondria are dynamic organelles that undergo rounds of fission and fusion and exhibit a wide range of morphologies that contribute to the regulation of different signaling pathways and various cellular functions. It is important to understand the...

  • Review
  • Open Access
47 Citations
9,888 Views
22 Pages

Mitochondrial Dysfunction in Parkinson’s Disease: Focus on Mitochondrial DNA

  • Olga Buneeva,
  • Valerii Fedchenko,
  • Arthur Kopylov and
  • Alexei Medvedev

Mitochondria, the energy stations of the cell, are the only extranuclear organelles, containing their own (mitochondrial) DNA (mtDNA) and the protein synthesizing machinery. The location of mtDNA in close proximity to the oxidative phosphorylation sy...

  • Review
  • Open Access
60 Citations
11,448 Views
23 Pages

Mitochondrial Transplantation in Mitochondrial Medicine: Current Challenges and Future Perspectives

  • Marco D’Amato,
  • Francesca Morra,
  • Ivano Di Meo and
  • Valeria Tiranti

19 January 2023

Mitochondrial diseases (MDs) are inherited genetic conditions characterized by pathogenic mutations in nuclear DNA (nDNA) or mitochondrial DNA (mtDNA). Current therapies are still far from being fully effective and from covering the broad spectrum of...

  • Review
  • Open Access
44 Citations
12,051 Views
17 Pages

Obesity, insulin resistance and type 2 diabetes are accompanied by a variety of systemic and tissue-specific metabolic defects, including inflammation, oxidative and endoplasmic reticulum stress, lipotoxicity, and mitochondrial dysfunction. Over the...

  • Article
  • Open Access
18 Citations
6,651 Views
13 Pages

Mitochondrial Dynamics Regulation in Skin Fibroblasts from Mitochondrial Disease Patients

  • Takeshi Tokuyama,
  • Asei Hirai,
  • Isshin Shiiba,
  • Naoki Ito,
  • Keigo Matsuno,
  • Keisuke Takeda,
  • Kanata Saito,
  • Koki Mii,
  • Nobuko Matsushita and
  • Shigeru Yanagi
  • + 2 authors

13 March 2020

Mitochondria are highly dynamic organelles that constantly fuse, divide, and move, and their function is regulated and maintained by their morphologic changes. Mitochondrial disease (MD) comprises a group of disorders involving mitochondrial dysfunct...

  • Article
  • Open Access
657 Views
13 Pages

18 December 2025

Background/Objectives: In recent years, it has been suggested that sedatives may cause brain damage. One possible mechanism is interference with oxidative phosphorylation of brain mitochondria, but much remains unknown. In this study, we focused on d...

  • Review
  • Open Access
13 Citations
9,866 Views
28 Pages

Creation of Mitochondrial Disease Models Using Mitochondrial DNA Editing

  • Victoria A. Khotina,
  • Andrey Y. Vinokurov,
  • Mariam Bagheri Ekta,
  • Vasily N. Sukhorukov and
  • Alexander N. Orekhov

Mitochondrial diseases are a large class of human hereditary diseases, accompanied by the dysfunction of mitochondria and the disruption of cellular energy synthesis, that affect various tissues and organ systems. Mitochondrial DNA mutation-caused di...

  • Article
  • Open Access
10 Citations
4,291 Views
22 Pages

4 November 2020

Friedreich’s ataxia (FRDA) is a neurodegenerative disease caused by recessive mutations in the frataxin gene that lead to a deficiency of the mitochondrial frataxin (FXN) protein. Alternative forms of frataxin have been described, with differen...

  • Review
  • Open Access
206 Citations
22,661 Views
19 Pages

24 April 2009

Beyond their fundamental role in energy metabolism, mitochondria perform a great variety of other important cellular functions. However, the interplayamong these various roles of mitochondria is still poorly understood, and the underlying mechanisms...

  • Article
  • Open Access
34 Citations
15,315 Views
12 Pages

The Effect of Mitochondrial Supplements on Mitochondrial Activity in Children with Autism Spectrum Disorder

  • Leanna M. Delhey,
  • Ekim Nur Kilinc,
  • Li Yin,
  • John C. Slattery,
  • Marie L. Tippett,
  • Shannon Rose,
  • Sirish C. Bennuri,
  • Stephen G. Kahler,
  • Shirish Damle and
  • Richard E. Frye
  • + 2 authors

13 February 2017

Treatment for mitochondrial dysfunction is typically guided by expert opinion with a paucity of empirical evidence of the effect of treatment on mitochondrial activity. We examined citrate synthase and Complex I and IV activities using a validated bu...

  • Review
  • Open Access
22 Citations
5,655 Views
34 Pages

Molecular Epidemiology of Mitochondrial Cardiomyopathy: A Search Among Mitochondrial and Nuclear Genes

  • Cristina Mazzaccara,
  • Bruno Mirra,
  • Ferdinando Barretta,
  • Martina Caiazza,
  • Barbara Lombardo,
  • Olga Scudiero,
  • Nadia Tinto,
  • Giuseppe Limongelli and
  • Giulia Frisso

Mitochondrial Cardiomyopathy (MCM) is a common manifestation of multi-organ Mitochondrial Diseases (MDs), occasionally present in non-syndromic cases. Diagnosis of MCM is complex because of wide clinical and genetic heterogeneity and requires medical...

  • Review
  • Open Access
15 Citations
7,563 Views
14 Pages

18 January 2024

Mitochondria are double-membrane organelles that are involved in energy production, apoptosis, and signaling in eukaryotic cells. Several studies conducted over the past decades have correlated mitochondrial dysfunction with various diseases, includi...

  • Review
  • Open Access
100 Citations
10,338 Views
18 Pages

18 September 2021

Pluripotent stem cells (PSCs) can undergo unlimited self-renewal and can differentiate into all the cell types present in our body, including cardiomyocytes. Therefore, PSCs can be an excellent source of cardiomyocytes for future regenerative medicin...

  • Review
  • Open Access
43 Citations
11,083 Views
34 Pages

Mitochondrial Quality Control via Mitochondrial Unfolded Protein Response (mtUPR) in Ageing and Neurodegenerative Diseases

  • Paula Cilleros-Holgado,
  • David Gómez-Fernández,
  • Rocío Piñero-Pérez,
  • Jose Manuel Romero-Domínguez,
  • Diana Reche-López,
  • Alejandra López-Cabrera,
  • Mónica Álvarez-Córdoba,
  • Manuel Munuera-Cabeza,
  • Marta Talaverón-Rey and
  • Jose Antonio Sánchez-Alcázar
  • + 2 authors

13 December 2023

Mitochondria play a key role in cellular functions, including energy production and oxidative stress regulation. For this reason, maintaining mitochondrial homeostasis and proteostasis (homeostasis of the proteome) is essential for cellular health. T...

  • Review
  • Open Access
29 Citations
7,309 Views
15 Pages

Mitochondrial Unfolded Protein Response and Integrated Stress Response as Promising Therapeutic Targets for Mitochondrial Diseases

  • Hedong Lu,
  • Xiaolei Wang,
  • Min Li,
  • Dongmei Ji,
  • Dan Liang,
  • Chunmei Liang,
  • Yajing Liu,
  • Zhiguo Zhang,
  • Yunxia Cao and
  • Weiwei Zou

21 December 2022

The development and application of high-throughput omics technologies have enabled a more in-depth understanding of mitochondrial biosynthesis metabolism and the pathogenesis of mitochondrial diseases. In accordance with this, a host of new treatment...

  • Review
  • Open Access
32 Citations
5,467 Views
19 Pages

Disturbance of Mitochondrial Dynamics and Mitochondrial Therapies in Atherosclerosis

  • Alexander M. Markin,
  • Viktoria A. Khotina,
  • Xenia G. Zabudskaya,
  • Anastasia I. Bogatyreva,
  • Antonina V. Starodubova,
  • Ekaterina Ivanova,
  • Nikita G. Nikiforov and
  • Alexander N. Orekhov

20 February 2021

Mitochondrial dysfunction is associated with a wide range of chronic human disorders, including atherosclerosis and diabetes mellitus. Mitochondria are dynamic organelles that undergo constant turnover in living cells. Through the processes of mitoch...

  • Review
  • Open Access
137 Citations
17,123 Views
29 Pages

Mitochondria, the membrane-bound cell organelles that supply most of the energy needed for cell function, are highly regulated, dynamic organelles bearing the ability to alter both form and functionality rapidly to maintain normal physiological event...

  • Review
  • Open Access
7 Citations
2,061 Views
22 Pages

Mitochondrial Quality Control and Cell Death

  • Zurui Zhang,
  • Mengyuan Zhang,
  • Hongchi Jin,
  • Shuang Lv,
  • Yilei Li and
  • Yanru Li

16 November 2025

Mitochondrial quality control includes mitochondrial biogenesis, fusion, fission (to maintain mitochondrial function), and mitochondrial autophagy (for removing damaged mitochondria). This is a highly delicate and complex process involving many molec...

  • Review
  • Open Access
60 Citations
9,483 Views
33 Pages

Mitochondrial Retinopathies

  • Massimo Zeviani and
  • Valerio Carelli

25 December 2021

The retina is an exquisite target for defects of oxidative phosphorylation (OXPHOS) associated with mitochondrial impairment. Retinal involvement occurs in two ways, retinal dystrophy (retinitis pigmentosa) and subacute or chronic optic atrophy, whic...

  • Article
  • Open Access
10 Citations
4,799 Views
23 Pages

Silencing of Poly(ADP-Ribose) Polymerase-2 Induces Mitochondrial Reactive Species Production and Mitochondrial Fragmentation

  • Laura Jankó,
  • Tünde Kovács,
  • Miklós Laczik,
  • Zsanett Sári,
  • Gyula Ujlaki,
  • Gréta Kis,
  • Ibolya Horváth,
  • Miklós Antal,
  • László Vígh and
  • Péter Bai
  • + 2 authors

4 June 2021

PARP2 is a DNA repair protein. The deletion of PARP2 induces mitochondrial biogenesis and mitochondrial activity by increasing NAD+ levels and inducing SIRT1 activity. We show that the silencing of PARP2 causes mitochondrial fragmentation in myoblast...

  • Review
  • Open Access
433 Citations
23,664 Views
24 Pages

16 January 2018

Mitochondria are organelles with a highly dynamic ultrastructure maintained by a delicate equilibrium between its fission and fusion rates. Understanding the factors influencing this balance is important as perturbations to mitochondrial dynamics can...

  • Brief Report
  • Open Access
3,102 Views
10 Pages

Involvement of Mitochondrial Dysfunction in FOXG1 Syndrome

  • Victoria A. Bjerregaard,
  • Amanda M. Levy,
  • Mille S. Batz,
  • Ravina Salehi,
  • Mathis Hildonen,
  • Trine B. Hammer,
  • Rikke S. Møller,
  • Claus Desler and
  • Zeynep Tümer

17 January 2023

FOXG1 (Forkhead box g1) syndrome is a neurodevelopmental disorder caused by a defective transcription factor, FOXG1, important for normal brain development and function. As FOXG1 syndrome and mitochondrial disorders have shared symptoms and FOXG1 reg...

  • Article
  • Open Access
15 Citations
4,018 Views
20 Pages

Impact of Mitochondrial A3243G Heteroplasmy on Mitochondrial Bioenergetics and Dynamics of Directly Reprogrammed MELAS Neurons

  • Dar-Shong Lin,
  • Yu-Wen Huang,
  • Che-Sheng Ho,
  • Tung-Sun Huang,
  • Tsung-Han Lee,
  • Tsu-Yen Wu,
  • Zon-Darr Huang and
  • Tuan-Jen Wang

21 December 2022

The MELAS syndrome primarily affecting the CNS is mainly caused by the m.A3243G mutation. The heteroplasmy in different tissues affects the phenotypic spectrum, yet the impact of various levels of m.A3243G heteroplasmy on CNS remains elusive due to t...

  • Article
  • Open Access
4 Citations
3,552 Views
20 Pages

PKR Mediates the Mitochondrial Unfolded Protein Response through Double-Stranded RNA Accumulation under Mitochondrial Stress

  • Fedho Kusuma,
  • Soyoung Park,
  • Kim Anh Nguyen,
  • Rosalie Elvira,
  • Duckgue Lee and
  • Jaeseok Han

Mitochondrial stress, resulting from dysfunction and proteostasis disturbances, triggers the mitochondrial unfolded protein response (UPRMT), which activates gene encoding chaperones and proteases to restore mitochondrial function. Although ATFS-1 me...

  • Review
  • Open Access
29 Citations
17,112 Views
21 Pages

Mitochondrial DNA Replacement Techniques to Prevent Human Mitochondrial Diseases

  • Luis Sendra,
  • Alfredo García-Mares,
  • María José Herrero and
  • Salvador F. Aliño

Background: Mitochondrial DNA (mtDNA) diseases are a group of maternally inherited genetic disorders caused by a lack of energy production. Currently, mtDNA diseases have a poor prognosis and no known cure. The chance to have unaffected offspring wit...

  • Article
  • Open Access
2 Citations
2,902 Views
18 Pages

21 August 2024

According to many research groups, high glucose induces the overproduction of superoxide anions, with reactive oxygen species (ROS) generally being considered the link between high glucose levels and the toxicity seen at cellular levels. Respiratory...

  • Review
  • Open Access
7 Citations
7,701 Views
22 Pages

The Mitochondrial Genome in Aging and Disease and the Future of Mitochondrial Therapeutics

  • Sanjana Saravanan,
  • Caitlin J. Lewis,
  • Bhavna Dixit,
  • Matthew S. O’Connor,
  • Alexandra Stolzing and
  • Amutha Boominathan

Mitochondria are intracellular organelles that utilize nutrients to generate energy in the form of ATP by oxidative phosphorylation. Mitochondrial DNA (mtDNA) in humans is a 16,569 base pair double-stranded circular DNA that encodes for 13 vital prot...

  • Article
  • Open Access
10 Citations
5,472 Views
16 Pages

The Mitochondrial Genes BAK1, FIS1 and SFN are Linked with Alterations in Mitochondrial Membrane Potential in Barrett’s Esophagus

  • James J. Phelan,
  • Finbar MacCarthy,
  • Dermot O’Toole,
  • Narayanasamy Ravi,
  • John V. Reynolds and
  • Jacintha O’Sullivan

6 November 2018

Barrett’s esophagus and esophageal cancer lack prognostic markers that allow the tailoring of personalized medicine and biomarkers with potential to provide insight into treatment response. This study aims to characterize mitochondrial function...

  • Review
  • Open Access
229 Citations
18,285 Views
19 Pages

Abnormalities of Mitochondrial Dynamics in Neurodegenerative Diseases

  • Ju Gao,
  • Luwen Wang,
  • Jingyi Liu,
  • Fei Xie,
  • Bo Su and
  • Xinglong Wang

Neurodegenerative diseases are incurable and devastating neurological disorders characterized by the progressive loss of the structure and function of neurons in the central nervous system or peripheral nervous system. Mitochondria, organelles found...

  • Review
  • Open Access
352 Citations
18,220 Views
21 Pages

Melatonin: A Mitochondrial Targeting Molecule Involving Mitochondrial Protection and Dynamics

  • Dun-Xian Tan,
  • Lucien C. Manchester,
  • Lilan Qin and
  • Russel J. Reiter

16 December 2016

Melatonin has been speculated to be mainly synthesized by mitochondria. This speculation is supported by the recent discovery that aralkylamine N-acetyltransferase/serotonin N-acetyltransferase (AANAT/SNAT) is localized in mitochondria of oocytes and...

  • Review
  • Open Access
32 Citations
10,010 Views
18 Pages

23 February 2025

Mitochondrial dysfunction is increasingly recognized as a central contributor to the pathogenesis of cardiovascular diseases (CVDs), including heart failure, ischemic heart disease, hypertension, and cardiomyopathy. Mitochondria, known as the powerho...

  • Review
  • Open Access
66 Citations
11,759 Views
15 Pages

Mitochondrial Epilepsy, a Challenge for Neurologists

  • Piervito Lopriore,
  • Fábio Gomes,
  • Vincenzo Montano,
  • Gabriele Siciliano and
  • Michelangelo Mancuso

30 October 2022

Primary mitochondrial diseases are relatively common inborn errors of energy metabolism, with a combined prevalence of 1 in 4300. These disorders typically affect tissues with high energy requirements, including the brain. Epilepsy affects >1% of...

  • Review
  • Open Access
15 Citations
15,985 Views
25 Pages

Clinical Approaches for Mitochondrial Diseases

  • Seongho Hong,
  • Sanghun Kim,
  • Kyoungmi Kim and
  • Hyunji Lee

20 October 2023

Mitochondria are subcontractors dedicated to energy production within cells. In human mitochondria, almost all mitochondrial proteins originate from the nucleus, except for 13 subunit proteins that make up the crucial system required to perform &lsqu...

  • Article
  • Open Access
2 Citations
3,208 Views
15 Pages

Mitochondrial Methionyl-tRNA Formyltransferase Deficiency Alleviates Metaflammation by Modulating Mitochondrial Activity in Mice

  • Xiaoxiao Sun,
  • Suyuan Liu,
  • Jiangxue Cai,
  • Miaoxin Yang,
  • Chenxuan Li,
  • Meiling Tan and
  • Bin He

Various studies have revealed the association of metabolic diseases with inflammation. Mitochondria are key organelles involved in metabolic regulation and important drivers of inflammation. However, it is uncertain whether the inhibition of mitochon...

  • Feature Paper
  • Review
  • Open Access
27 Citations
8,620 Views
12 Pages

Insulin Resistance in Mitochondrial Diabetes

  • Chika Takano,
  • Erika Ogawa and
  • Satoshi Hayakawa

7 January 2023

Mitochondrial diabetes (MD) is generally classified as a genetic defect of β-cells. The main pathophysiology is insulin secretion failure in pancreatic β-cells due to impaired mitochondrial ATP production. However, several reports have ment...

  • Review
  • Open Access
23 Citations
7,172 Views
12 Pages

Morphological Pathways of Mitochondrial Division

  • Bernard Tandler,
  • Charles L. Hoppel and
  • Jason A. Mears

15 February 2018

Mitochondrial fission is essential for distributing cellular energy throughout cells and for isolating damaged regions of the organelle that are targeted for degradation. Excessive fission is associated with the progression of cell death as well. The...

  • Article
  • Open Access
20 Citations
4,687 Views
19 Pages

Metabolic Reprogramming in Response to Alterations of Mitochondrial DNA and Mitochondrial Dysfunction in Gastric Adenocarcinoma

  • Tzu-Ching Chang,
  • Hui-Ting Lee,
  • Siao-Cian Pan,
  • Shih-Han Cho,
  • Chieh Cheng,
  • Liang-Hung Ou,
  • Chia-I Lin,
  • Chen-Sung Lin and
  • Yau-Huei Wei

6 February 2022

We used gastric cancer cell line AGS and clinical samples to investigate the roles of mitochondrial DNA (mtDNA) alterations and mitochondrial respiratory dysfunction in gastric adenocarcinoma (GAC). A total of 131 clinical samples, including 17 norma...

  • Review
  • Open Access
142 Citations
10,621 Views
13 Pages

Calcium Overload and Mitochondrial Metabolism

  • Lauren L. Walkon,
  • Jasiel O. Strubbe-Rivera and
  • Jason N. Bazil

17 December 2022

Mitochondria calcium is a double-edged sword. While low levels of calcium are essential to maintain optimal rates of ATP production, extreme levels of calcium overcoming the mitochondrial calcium retention capacity leads to loss of mitochondrial func...

  • Review
  • Open Access
79 Citations
11,824 Views
24 Pages

3 September 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 they are frequ...

  • Review
  • Open Access
18 Citations
8,370 Views
18 Pages

Role of Mitochondrial Dynamics in Cocaine’s Neurotoxicity

  • Shuheng Wen,
  • Toshihiko Aki,
  • Takeshi Funakoshi,
  • Kana Unuma and
  • Koichi Uemura

The dynamic balance of mitochondrial fission and fusion maintains mitochondrial homeostasis and optimal function. It is indispensable for cells such as neurons, which rely on the finely tuned mitochondria to carry out their normal physiological activ...

  • Review
  • Open Access
10 Citations
3,595 Views
16 Pages

A Mitochondrial Perspective on Noncommunicable Diseases

  • Yifan Zheng,
  • Jing Zhang,
  • Xiaohong Zhu,
  • Yuanjuan Wei,
  • Wuli Zhao,
  • Shuyi Si and
  • Yan Li

Mitochondria are the center of energy metabolism in eukaryotic cells and play a central role in the metabolism of living organisms. Mitochondrial diseases characterized by defects in oxidative phosphorylation are the most common congenital diseases....

  • Feature Paper
  • Perspective
  • Open Access
41 Citations
8,867 Views
9 Pages

A Mitochondrial Encoded Messenger at the Nucleus

  • Cheryl Qian Ying Yong and
  • Bor Luen Tang

13 August 2018

Mitochondria–nucleus (mitonuclear) retrograde signaling via nuclear import of otherwise mitochondrial targeted factors occurs during mitochondrial unfolded protein response (UPRmt), a mechanism that counters mitochondrial and cellular stresses....

  • Review
  • Open Access
40 Citations
7,142 Views
13 Pages

Biogenesis of Mitochondrial Metabolite Carriers

  • Patrick Horten,
  • Lilia Colina-Tenorio and
  • Heike Rampelt

7 July 2020

Metabolite carriers of the mitochondrial inner membrane are crucial for cellular physiology since mitochondria contribute essential metabolic reactions and synthesize the majority of the cellular ATP. Like almost all mitochondrial proteins, carriers...

  • Review
  • Open Access
9 Citations
3,918 Views
23 Pages

Mitochondrial Extracellular Vesicles: A Novel Approach to Mitochondrial Quality Control

  • Jie Kong,
  • Rui Sun,
  • Chengying Du,
  • Yiyang Tang,
  • Chengzhi Xie,
  • Qian Li,
  • Li Lin and
  • Hongyan Wang

8 August 2025

Mitochondria are central to cellular energy metabolism and play a key role in regulating important physiological processes, including apoptosis and oxidative stress. Mitochondrial quality control has recently garnered significant attention, with the...

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