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Article ; Online: Primary mitochondrial diseases: The intertwined pathophysiology of bioenergetic dysregulation, oxidative stress and neuroinflammation.

Aguilar, Kevin / Jakubek, Patrycja / Zorzano, Antonio / Wieckowski, Mariusz R

European journal of clinical investigation

2024 , Page(s) e14217

Abstract: Objectives and scope: Primary mitochondrial diseases (PMDs) are rare genetic disorders resulting from mutations in genes crucial for effective oxidative phosphorylation (OXPHOS) that can affect mitochondrial function. In this review, we examine the ... ...

Abstract	Objectives and scope: Primary mitochondrial diseases (PMDs) are rare genetic disorders resulting from mutations in genes crucial for effective oxidative phosphorylation (OXPHOS) that can affect mitochondrial function. In this review, we examine the bioenergetic alterations and oxidative stress observed in cellular models of primary mitochondrial diseases (PMDs), shedding light on the intricate complexity between mitochondrial dysfunction and cellular pathology. We explore the diverse cellular models utilized to study PMDs, including patient-derived fibroblasts, induced pluripotent stem cells (iPSCs) and cybrids. Moreover, we also emphasize the connection between oxidative stress and neuroinflammation. Insights: The central nervous system (CNS) is particularly vulnerable to mitochondrial dysfunction due to its dependence on aerobic metabolism and the correct functioning of OXPHOS. Similar to other neurodegenerative diseases affecting the CNS, individuals with PMDs exhibit several neuroinflammatory hallmarks alongside neurodegeneration, a pattern also extensively observed in mouse models of mitochondrial diseases. Based on histopathological analysis of postmortem human brain tissue and findings in mouse models of PMDs, we posit that neuroinflammation is not merely a consequence of neurodegeneration but a potential pathogenic mechanism for disease progression that deserves further investigation. This recognition may pave the way for novel therapeutic strategies for this group of devastating diseases that currently lack effective treatments. Summary: In summary, this review provides a comprehensive overview of bioenergetic alterations and redox imbalance in cellular models of PMDs while underscoring the significance of neuroinflammation as a potential driver in disease progression.
Language	English
Publishing date	2024-04-21
Publishing country	England
Document type	Journal Article ; Review
ZDB-ID	186196-7
ISSN	1365-2362 ; 0014-2972 ; 0960-135X
ISSN (online)	1365-2362
ISSN	0014-2972 ; 0960-135X
DOI	10.1111/eci.14217
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Zs.A 677: Show issues

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Article ; Online: An Update on Isolation of Functional Mitochondria from Cells for Bioenergetics Studies.

Lebiedzinska-Arciszewska, Magdalena / Wojtczak, Lech / Wieckowski, Mariusz R

Methods in molecular biology (Clifton, N.J.)

2021 Volume 2310, Page(s) 79–89

Abstract: Mitochondria are the organelles where the most fundamental processes of energy transformation within the cell are located. They are also involved in several processes like apoptosis and autophagy, reactive oxygen species formation, and calcium signaling, ...

Abstract	Mitochondria are the organelles where the most fundamental processes of energy transformation within the cell are located. They are also involved in several processes like apoptosis and autophagy, reactive oxygen species formation, and calcium signaling, which are crucial for proper cell functioning. In addition, mitochondrial genome hosts genes encoding important proteins incorporated in respiratory chain complexes and indispensable for the oxidative phosphorylation. Studying isolated mitochondria is, therefore, crucial for better understanding of cell physiology. The presented protocol describes a relatively simple and handy method for crude mitochondrial fraction isolation from different mammalian cell lines. It includes mechanical cells disruption (homogenization) and differential centrifugation. In addition, this chapter presents two basic ways to assess mitochondrial functionality: by measuring mitochondrial inner membrane potential and coupled respiration.
MeSH term(s)	Animals ; Cell Fractionation ; Cell Respiration ; Centrifugation ; Energy Metabolism ; Fluorometry ; HeLa Cells ; Humans ; Jurkat Cells ; Membrane Potential, Mitochondrial ; Mice ; Mitochondria/metabolism ; Oxygen Consumption ; Rats
Language	English
Publishing date	2021-06-06
Publishing country	United States
Document type	Journal Article ; Research Support, Non-U.S. Gov't
ISSN	1940-6029
ISSN (online)	1940-6029
DOI	10.1007/978-1-0716-1433-4_7
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Article ; Online: Update on the Histoenzymatic Methods for Visualization of the Activity of Individual Mitochondrial Respiratory Chain Complexes in the Human Frozen Sections.

Wieckowski, Mariusz R / Pronicki, Maciej / Karkucinska-Wieckowska, Agnieszka

Methods in molecular biology (Clifton, N.J.)

2021 Volume 2310, Page(s) 69–77

Abstract: Investigation of mitochondrial metabolism perturbations and successful diagnosis of patients with mitochondrial abnormalities often requires assessment of human samples like muscle or liver biopsy as well as autopsy material. Immunohistochemical and ... ...

Abstract	Investigation of mitochondrial metabolism perturbations and successful diagnosis of patients with mitochondrial abnormalities often requires assessment of human samples like muscle or liver biopsy as well as autopsy material. Immunohistochemical and histochemical examination is an important technique to investigate mitochondrial dysfunction that combined with spectrophotometric and Blue Native electrophoresis techniques can be an important tool to provide diagnosis of mitochondrial disorders. In this chapter, we focus on technical description of the methods that are suitable to detect the activity of complex I, II, and IV of mitochondrial respiratory chain in frozen sections of brain, heart, muscle, and liver biopsies/autopsy. The protocols provided can be useful not only for general assessment of mitochondrial activity in studied material, but they are also successfully used in the diagnostic procedures in case of suspicion of mitochondrial disorders. In the age of high-performance NGS sequencing, these methods can be used to confirm whether mutations are pathogenic by proving their impact on the activity of individual respiratory chain complexes.
MeSH term(s)	Brain/enzymology ; Electron Transport Chain Complex Proteins/analysis ; Frozen Sections ; Humans ; Microscopy ; Mitochondria, Heart/enzymology ; Mitochondria, Liver/enzymology ; Mitochondria, Muscle/enzymology ; Staining and Labeling
Chemical Substances	Electron Transport Chain Complex Proteins
Language	English
Publishing date	2021-06-06
Publishing country	United States
Document type	Journal Article ; Research Support, Non-U.S. Gov't
ISSN	1940-6029
ISSN (online)	1940-6029
DOI	10.1007/978-1-0716-1433-4_6
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Article: Editorial: Organelles Relationships and Interactions: A Cancer Perspective.

Patergnani, Simone / Marchi, Saverio / Delprat, Benjamin / Wieckowski, Mariusz R

Frontiers in cell and developmental biology

2021 Volume 9, Page(s) 678307

Language	English
Publishing date	2021-04-08
Publishing country	Switzerland
Document type	Editorial
ZDB-ID	2737824-X
ISSN	2296-634X
ISSN	2296-634X
DOI	10.3389/fcell.2021.678307
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Article ; Online: Calcium Homeostasis in the Control of Mitophagy.

Perrone, Mariasole / Patergnani, Simone / Di Mambro, Tommaso / Palumbo, Laura / Wieckowski, Mariusz R / Giorgi, Carlotta / Pinton, Paolo

Antioxidants & redox signaling

2023 Volume 38, Issue 7-9, Page(s) 581–598

Abstract: Significance: ...

Abstract	Significance:
MeSH term(s)	Mitophagy ; Calcium/metabolism ; Mitochondria/metabolism ; Homeostasis ; Biological Transport ; Autophagy
Chemical Substances	Calcium (SY7Q814VUP)
Language	English
Publishing date	2023-03-01
Publishing country	United States
Document type	Journal Article ; Review ; Research Support, Non-U.S. Gov't
ZDB-ID	1483836-9
ISSN	1557-7716 ; 1523-0864
ISSN (online)	1557-7716
ISSN	1523-0864
DOI	10.1089/ars.2022.0122
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Zs.A 5488: Show issues

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Article: From mitochondrial large amplitude swelling to the permeability transition - a short historic overview.

Wojtczak, Lech / Więckowski, Mariusz R

Postepy biochemii

2016 Volume 62, Issue 3, Page(s) 298–302

Abstract: An outline of studies on the mitochondrial large conductance permeability pore is presented starting from the early observations in the 1950s on the large amplitude mitochondrial swelling, through the concept of the permeability transition and various ... ...

Title translation	Od pęcznienia mitochondriów po koncepcję transformacji przepuszczalnościowej - krótki zarys historyczny.
Abstract	An outline of studies on the mitochondrial large conductance permeability pore is presented starting from the early observations in the 1950s on the large amplitude mitochondrial swelling, through the concept of the permeability transition and various theories on the structure of the related permeability transition pore, up to its present identification as a part of mitochondrial (F
Language	English
Publishing date	2016
Publishing country	Poland
Document type	Journal Article ; Review
ZDB-ID	414019-9
ISSN	0032-5422
ISSN	0032-5422
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Zs.B 633: Show issues

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Article: Some Insights into the Regulation of Cardiac Physiology and Pathology by the Hippo Pathway.

Ramaccini, Daniela / Pedriali, Gaia / Perrone, Mariasole / Bouhamida, Esmaa / Modesti, Lorenzo / Wieckowski, Mariusz R / Giorgi, Carlotta / Pinton, Paolo / Morciano, Giampaolo

Biomedicines

2022 Volume 10, Issue 3

Abstract: The heart is one of the most fascinating organs in living beings. It beats up to 100,000 times a day throughout the lifespan, without resting. The heart undergoes profound anatomical, biochemical, and functional changes during life, from hypoxemic fetal ... ...

Abstract	The heart is one of the most fascinating organs in living beings. It beats up to 100,000 times a day throughout the lifespan, without resting. The heart undergoes profound anatomical, biochemical, and functional changes during life, from hypoxemic fetal stages to a completely differentiated four-chambered cardiac muscle. In the middle, many biological events occur after and intersect with each other to regulate development, organ size, and, in some cases, regeneration. Several studies have defined the essential roles of the Hippo pathway in heart physiology through the regulation of apoptosis, autophagy, cell proliferation, and differentiation. This molecular route is composed of multiple components, some of which were recently discovered, and is highly interconnected with multiple known prosurvival pathways. The Hippo cascade is evolutionarily conserved among species, and in addition to its regulatory roles, it is involved in disease by drastically changing the heart phenotype and its function when its components are mutated, absent, or constitutively activated. In this review, we report some insights into the regulation of cardiac physiology and pathology by the Hippo pathway.
Language	English
Publishing date	2022-03-21
Publishing country	Switzerland
Document type	Journal Article ; Review
ZDB-ID	2720867-9
ISSN	2227-9059
ISSN	2227-9059
DOI	10.3390/biomedicines10030726
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Article ; Online: Isolation of crude mitochondrial fraction from cells.

Wieckowski, Mariusz R / Wojtczak, Lech

Methods in molecular biology (Clifton, N.J.)

2015 Volume 1241, Page(s) 1–8

Abstract: Mitochondria are intracellular organelles where most fundamental processes of energy transformation within the cell are located. They also play important roles in programmed cell death (apoptosis), free radical formation, and signal transduction. In ... ...

Abstract	Mitochondria are intracellular organelles where most fundamental processes of energy transformation within the cell are located. They also play important roles in programmed cell death (apoptosis), free radical formation, and signal transduction. In addition, mitochondria host genes encoding several important proteins. Studying isolated mitochondria is therefore crucial for better understanding cell functioning. The present article describes a relatively simple and handy procedure for isolation of crude mitochondrial fraction from cultivated mammalian and human cells. It consists of mechanical homogenization and fractionating centrifugation. Assays of checking mitochondrial competence by measuring membrane potential formation and coupled respiration are also presented.
MeSH term(s)	Animals ; Cell Fractionation/methods ; Cell Line ; Humans ; Membrane Potential, Mitochondrial ; Mice ; Mitochondria/metabolism ; Mitochondrial Proteins/analysis ; Mitochondrial Proteins/metabolism ; Oxygen Consumption ; Phenazines/metabolism
Chemical Substances	Mitochondrial Proteins ; Phenazines ; safranine T (XTX0YXU2HV)
Language	English
Publishing date	2015
Publishing country	United States
Document type	Journal Article ; Research Support, Non-U.S. Gov't
ISSN	1940-6029
ISSN (online)	1940-6029
DOI	10.1007/978-1-4939-1875-1_1
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Article ; Online: Oxidative stress in metabolic dysfunction-associated steatotic liver disease (MASLD): How does the animal model resemble human disease?

Jakubek, Patrycja / Kalinowski, Piotr / Karkucinska-Wieckowska, Agnieszka / Kaikini, Aakruti / Simões, Inês C M / Potes, Yaiza / Kruk, Beata / Grajkowska, Wieslawa / Pinton, Paolo / Milkiewicz, Piotr / Grąt, Michał / Pronicki, Maciej / Lebiedzinska-Arciszewska, Magdalena / Krawczyk, Marcin / Wieckowski, Mariusz R

FASEB journal : official publication of the Federation of American Societies for Experimental Biology

2024 Volume 38, Issue 3, Page(s) e23466

Abstract: Despite decades of research, the pathogenesis of metabolic dysfunction-associated steatotic liver disease (MASLD) is still not completely understood. Based on the evidence from preclinical models, one of the factors proposed as a main driver of disease ... ...

Abstract	Despite decades of research, the pathogenesis of metabolic dysfunction-associated steatotic liver disease (MASLD) is still not completely understood. Based on the evidence from preclinical models, one of the factors proposed as a main driver of disease development is oxidative stress. This study aimed to search for the resemblance between the profiles of oxidative stress and antioxidant defense in the animal model of MASLD and the group of MASLD patients. C57BL/6J mice were fed with the Western diet for up to 24 weeks and served as the animal model of MASLD. The antioxidant profile of mice hepatic tissue was determined by liquid chromatography-MS3 spectrometry (LC-MS/MS). The human cohort consisted of 20 patients, who underwent bariatric surgery, and 6 controls. Based on histological analysis, 4 bariatric patients did not have liver steatosis and as such were also classified as controls. Total antioxidant activity was measured in sera and liver biopsy samples. The hepatic levels of antioxidant enzymes and oxidative damage were determined by Western Blot. The levels of antioxidant enzymes were significantly altered in the hepatic tissue of mice with MASLD. In contrast, there were no significant changes in the antioxidant profile of hepatic tissue of MASLD patients, except for the decreased level of carbonylated proteins. Decreased protein carbonylation together with significant correlations between the thioredoxin system and parameters describing metabolic health suggest alterations in the thiol-redox signaling. Altogether, these data show that even though the phenotype of mice closely resembles human MASLD, the animal-to-human translation of cellular and molecular processes such as oxidative stress may be more challenging.
MeSH term(s)	Humans ; Animals ; Mice ; Mice, Inbred C57BL ; Antioxidants ; Chromatography, Liquid ; Tandem Mass Spectrometry ; Oxidative Stress ; Metabolic Diseases ; Fatty Liver ; Models, Animal
Chemical Substances	Antioxidants
Language	English
Publishing date	2024-02-06
Publishing country	United States
Document type	Journal Article
ZDB-ID	639186-2
ISSN	1530-6860 ; 0892-6638
ISSN (online)	1530-6860
ISSN	0892-6638
DOI	10.1096/fj.202302447R
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Zs.A 2266: Show issues			Location: Je nach Verfügbarkeit (siehe Angabe bei Bestand) bis Jg. 1994: Bestellungen von Artikeln über das Online-Bestellformular Jg. 1995 - 2021: Lesesall (1.OG) ab Jg. 2022: Lesesaal (EG)
Zs.MO 296: Show issues

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Article ; Online: Dynamic adaptation of the extremophilic red microalga Cyanidioschyzon merolae to high nickel stress.

Marchetto, Francesca / Santaeufemia, Sergio / Lebiedzińska-Arciszewska, Magdalena / Śliwińska, Małgorzata A / Pich, Magdalena / Kurek, Eliza / Naziębło, Aleksandra / Strawski, Marcin / Solymosi, Daniel / Szklarczyk, Marek / Bulska, Ewa / Szymański, Jędrzej / Wierzbicka, Małgorzata / Allahverdiyeva, Yagut / Więckowski, Mariusz R / Kargul, Joanna

Plant physiology and biochemistry : PPB

2024 Volume 207, Page(s) 108365

Abstract: The order of Cyanidiales comprises seven acido-thermophilic red microalgal species thriving in hot springs of volcanic origin characterized by extremely low pH, moderately high temperatures and the presence of high concentrations of sulphites and heavy ... ...

Abstract	The order of Cyanidiales comprises seven acido-thermophilic red microalgal species thriving in hot springs of volcanic origin characterized by extremely low pH, moderately high temperatures and the presence of high concentrations of sulphites and heavy metals that are prohibitive for most other organisms. Little is known about the physiological processes underlying the long-term adaptation of these extremophiles to such hostile environments. Here, we investigated the long-term adaptive responses of a red microalga Cyanidioschyzon merolae, a representative of Cyanidiales, to extremely high nickel concentrations. By the comprehensive physiological, microscopic and elemental analyses we dissected the key physiological processes underlying the long-term adaptation of this model extremophile to high Ni exposure. These include: (i) prevention of significant Ni accumulation inside the cells; (ii) activation of the photoprotective response of non-photochemical quenching; (iii) significant changes of the chloroplast ultrastructure associated with the formation of prolamellar bodies and plastoglobuli together with loosening of the thylakoid membranes; (iv) activation of ROS amelioration machinery; and (v) maintaining the efficient respiratory chain functionality. The dynamically regulated processes identified in this study are discussed in the context of the mechanisms driving the remarkable adaptability of C. merolae to extremely high Ni levels exceeding by several orders of magnitude those found in the natural environment of the microalga. The processes identified in this study provide a solid basis for the future investigation of the specific molecular components and pathways involved in the adaptation of Cyanidiales to the extremely high Ni concentrations.
MeSH term(s)	Extremophiles ; Nickel ; Microalgae ; Chloroplasts
Chemical Substances	Nickel (7OV03QG267)
Language	English
Publishing date	2024-01-11
Publishing country	France
Document type	Journal Article
ZDB-ID	742978-2
ISSN	1873-2690 ; 0981-9428
ISSN (online)	1873-2690
ISSN	0981-9428
DOI	10.1016/j.plaphy.2024.108365
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