Outer membrane vesicles of Porphyromonas gingivalis attenuate insulin sensitivity by delivering gingipains to the liver
Publication date: Available online 20 February 2020Source: Biochimica et Biophysica Acta (BBA) - Molecular Basis of DiseaseAuthor(s): Mariko Seyama, Kaya Yoshida, Kayo Yoshida, Natsumi Fujiwara, Kisho Ono, Takanori Eguchi, Hotaka Kawai, Jiajie Guo, Yao Weng, Yuan Haoze, Kenta Uchibe, Mika Ikegame, Akira Sasaki, Hitoshi Nagatsuka, Kuniaki Okamoto, Hirohiko Okamura, Kazumi OzakiAbstractOuter membrane vesicles (OMVs) are nanosized particles derived from the outer membrane of gram-negative bacteria. Oral bacterium Porphyromonas gingivalis (Pg) is known to be a major pathogen of periodontitis that contributes to the progression...
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - February 22, 2020 Category: Molecular Biology Source Type: research

Recovery of muscle mass and muscle oxidative phenotype following disuse does not require GSK-3 inactivation
ConclusionThis study indicates that GSK-3 inactivation is dispensable for reloading-induced recovery of muscle mass and OXPHEN. (Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease)
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - February 20, 2020 Category: Molecular Biology Source Type: research

Cardiac ketone body metabolism
Publication date: Available online 19 February 2020Source: Biochimica et Biophysica Acta (BBA) - Molecular Basis of DiseaseAuthor(s): Azrul Abdul Kadir, Kieran Clarke, Rhys D. EvansAbstractThe ketone bodies, d-β-hydroxybutyrate and acetoacetate, are soluble 4-carbon compounds derived principally from fatty acids, that can be metabolised by many oxidative tissues, including heart, in carbohydrate-depleted conditions as glucose-sparing energy substrates. They also have important signalling functions, acting through G-protein coupled receptors and histone deacetylases to regulate metabolism and gene expression including ...
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - February 20, 2020 Category: Molecular Biology Source Type: research

MeCP2 is involved in random mono-allelic expression for a subset of human autosomal genes
Publication date: Available online 15 February 2020Source: Biochimica et Biophysica Acta (BBA) - Molecular Basis of DiseaseAuthor(s): Marine Brousseau, Juliette Nectoux, Benjamin Saintpierre, Nicolas Lebrun, Nicolas Cagnard, Brigitte Izac, Emmanuelle Olivier, Franck Letourneur, Thierry BienvenuAbstractWidespread random monoallelic gene expression (RMAE) effects influence about 10% of human genes. However, the mechanisms by which RME of autosomal genes is established and those by which it is maintained both remain open questions. Because the choice of allelic expression is randomly performed cell-by-cell, the RMAE mechanism...
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - February 17, 2020 Category: Molecular Biology Source Type: research

Effects of clofibrate and KH176 on life span and motor function in mitochondrial complex I-deficient mice
Publication date: Available online 15 February 2020Source: Biochimica et Biophysica Acta (BBA) - Molecular Basis of DiseaseAuthor(s): Sanne J.C.M. Frambach, Melissa A.E. van de Wal, Petra H.H. van den Broek, Jan A.M. Smeitink, Frans G.M. Russel, Ria de Haas, Tom J.J. SchirrisAbstractMitochondrial complex I (CI), the first multiprotein enzyme complex of the OXPHOS system, executes a major role in cellular ATP generation. Consequently, dysfunction of this complex has been linked to inherited metabolic disorders, including Leigh disease (LD), an often fatal disease in early life. Development of clinical effective treatments f...
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - February 17, 2020 Category: Molecular Biology Source Type: research

CaMKII and GLUT1 in heart failure and the role of gliflozins
Publication date: Available online 14 February 2020Source: Biochimica et Biophysica Acta (BBA) - Molecular Basis of DiseaseAuthor(s): M. Trum, S. Wagner, L.S. Maier, J. MustrophAbstractEmpagliflozin, a selective sodium-glucose co-transporter 2 (SGLT2) inhibitor, has been shown to reduce mortality and hospitalization for heart failure in diabetic patients in the EMPA-REG-OUTCOME trial (Zinman et al., 2015). Surprisingly, dapagliflozin, another SGLT2 inhibitor, exerted comparable effects on clinical endpoints even in the absence of diabetes mellitus (DAPA-HF trial) McMurray et al. (2019). There is a myriad of suggested under...
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - February 16, 2020 Category: Molecular Biology Source Type: research

Regulation of metabolism by mitochondrial enzyme acetylation in cardiac ischemia-reperfusion injury
Publication date: Available online 15 February 2020Source: Biochimica et Biophysica Acta (BBA) - Molecular Basis of DiseaseAuthor(s): Daniel J. Herr, Toolika Singh, Tajinder Dhammu, Donald R. MenickAbstractIschemia reperfusion injury (I/R injury) contributes significantly to morbidity and mortality following myocardial infarction (MI). Although rapid reperfusion of the ischemic myocardium was established decades ago as a highly beneficial therapy for MI, significant cell death still occurs after the onset of reperfusion. Mitochondrial dysfunction is closely associated with I/R injury, resulting in the uncontrolled producti...
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - February 16, 2020 Category: Molecular Biology Source Type: research

Parkin-mediated mitophagy and autophagy flux disruption in cellular models of MERRF syndrome
Publication date: Available online 13 February 2020Source: Biochimica et Biophysica Acta (BBA) - Molecular Basis of DiseaseAuthor(s): Marina Villanueva-Paz, Suleva Povea-Cabello, Irene Villalón-García, Mónica Álvarez-Córdoba, Juan M. Suárez-Rivero, Marta Talaverón-Rey, Sandra Jackson, Rafael Falcón-Moya, Antonio Rodríguez-Moreno, José A. Sánchez-AlcázarAbstractMitochondrial diseases are considered rare genetic disorders characterized by defects in oxidative phosphorylation (OXPHOS). They can be provoked by mutations in nuclear DNA (nDNA) or...
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - February 14, 2020 Category: Molecular Biology Source Type: research

Knockout of DNase1l1l abrogates lens denucleation process and causes cataract in zebrafish
In this study, it was found that, instead of DNase2β, DNase1l1l is uniquely expressed in zebrafish lens and was absent in Hsf4−/− zebrafish lens. Using CRISPR-Cas9 technology, a DNase1l1l knockout zebrafish line was constructed, which developed cataract. Deletion of DNase1l1l totally abrogated lens primary and secondary fiber cell denucleation process, whereas had little effect on the clearance of other organelles. The transcriptional regulation of DNase1l1l was dramatically impaired in Hsf4−/− zebrafish lens. Rescue of DNase1l1l mRNA into Hsf4−/− zebrafish embryos alleviated its de...
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - February 13, 2020 Category: Molecular Biology Source Type: research

Prediction of VLCAD deficiency phenotype by a metabolic fingerprint in newborn screening bloodspots
ConclusionDistinctive metabolomic profiles in DBS of newborns with VLCADD may allow phenotypic prognostication. The full potential of this approach as well as the underlying biochemical mechanisms need further investigation.Graphical abstract (Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease)
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - February 13, 2020 Category: Molecular Biology Source Type: research

Deacetylation of MRTF-A by SIRT1 defies senescence induced down-regulation of collagen type I in fibroblast cells
In conclusion, our data unveil a novel mechanism that underscores aging-associated loss of collagen type I in fibroblasts via SIRT1-mediated post-translational modification of MRTF-A. (Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease)
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - February 13, 2020 Category: Molecular Biology Source Type: research

Unraveling the role of thiosulfate sulfurtransferase in metabolic diseases
Publication date: Available online 12 February 2020Source: Biochimica et Biophysica Acta (BBA) - Molecular Basis of DiseaseAuthor(s): Paul D. Kruithof, Sergey Lunev, Sheila P. Aguilar Lozano, Fernando de Assis Batista, Zayana M. Al-dahmani, Jaap A. Joles, Amalia M. Dolga, Matthew R. Groves, Harry van GoorAbstractThiosulfate sulfurtransferase (TST, EC 2.8.1.1), also known as Rhodanese, is a mitochondrial enzyme which catalyzes the transfer of sulfur in several molecular pathways. After its initial identification as a cyanide detoxification enzyme, it was found that its functions also include sulfur metabolism, modification ...
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - February 13, 2020 Category: Molecular Biology Source Type: research

Gender issues in cardiovascular diseases. Focus on energy metabolism
Publication date: Available online 11 February 2020Source: Biochimica et Biophysica Acta (BBA) - Molecular Basis of DiseaseAuthor(s): R. Ventura-Clapier, J. Piquereau, A. Garnier, M. Mericskay, C. Lemaire, B. CrozatierAbstractIt is increasingly recognized that sex and gender differences (S&G) influence cardiovascular diseases (CVD), greatly impacting disease management. In terms of definition, sex refers to biological aspects, gender effects being mainly related to socio-cultural factors. Both sex and gender are interpenetrated in humans and difficult to separate. This is more clearly feasible in animal models were sex...
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - February 12, 2020 Category: Molecular Biology Source Type: research

Metabolic reprogramming and disease progression in cancer patients
Publication date: Available online 11 February 2020Source: Biochimica et Biophysica Acta (BBA) - Molecular Basis of DiseaseAuthor(s): Laura Torresano, Cristina Nuevo-Tapioles, Fulvio Santacatterina, José M. CuezvaAbstractGenomics has contributed to the treatment of a fraction of cancer patients. However, there is a need to profile the proteins that define the phenotype of cancer and its pathogenesis. The reprogramming of metabolism is a major trait of the cancer phenotype with great potential for prognosis and targeted therapy. This review overviews the major changes reported in the steady-state levels of proteins o...
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - February 12, 2020 Category: Molecular Biology Source Type: research

Corrigendum to “Icariin protects human nucleus pulposus cells from hydrogen peroxide-induced mitochondria-mediated apoptosis by activating nuclear factor erythroid 2-related factor 2” [(BBA) — Mol. Basis Dis. Volume 1866, Issue 1, 1 January 2020, 165575]
Publication date: Available online 10 February 2020Source: Biochimica et Biophysica Acta (BBA) - Molecular Basis of DiseaseAuthor(s): Wenbin Hua, Shuai Li, Rongjin Luo, Xinghuo Wu, Yukun Zhang, Zhiwei Liao, Yu Song, Kun Wang, Kangcheng Zhao, Shuhua Yang, Cao Yang (Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease)
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - February 11, 2020 Category: Molecular Biology Source Type: research

Metabolic interactions between peroxisomes and mitochondria with a special focus on acylcarnitine metabolism
Publication date: Available online 10 February 2020Source: Biochimica et Biophysica Acta (BBA) - Molecular Basis of DiseaseAuthor(s): Sander M. Houten, Ronald J.A. Wanders, Pablo Ranea-RoblesAbstractCarnitine plays an essential role in mitochondrial fatty acid β-oxidation as a part of a cycle that transfers long-chain fatty acids across the mitochondrial membrane and involves two carnitine palmitoyltransferases (CPT1 and CPT2). Two distinct carnitine acyltransferases, carnitine octanoyltransferase (COT) and carnitine acetyltransferase (CAT), are peroxisomal enzymes, which indicates that carnitine is not only important...
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - February 10, 2020 Category: Molecular Biology Source Type: research

House dust mite allergens induce interleukin 33 (IL-33) synthesis and release from keratinocytes via ATP-mediated extracellular signaling
Publication date: Available online 7 February 2020Source: Biochimica et Biophysica Acta (BBA) - Molecular Basis of DiseaseAuthor(s): Xiuju Dai, Mikiko Tohyama, Masamoto Murakami, Ken Shiraishi, Shuang Liu, Hideki Mori, Ryo Utsunomiya, Kazutaka Maeyama, Koji SayamaAbstractIn atopic diseases, the epithelium releases cytokines and chemokines that initiate skin inflammation. Atopic dermatitis (AD) is characterized by a disrupted epidermal barrier and is triggered or exacerbated by environmental stimuli such as house dust mite (HDM) allergens. The proinflammatory cytokine interleukin 33 (IL-33) plays an important role in the pa...
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - February 8, 2020 Category: Molecular Biology Source Type: research

Cell cycle regulators in cancer cell metabolism
Publication date: Available online 5 February 2020Source: Biochimica et Biophysica Acta (BBA) - Molecular Basis of DiseaseAuthor(s): Lucia C. Leal-Esteban, Lluis FajasAbstractCancer proliferation and progression involves altered metabolic pathways as a result of continuous demand for energy and nutrients. In the last years, cell cycle regulators have been involved in the control of metabolic processes, such as glucose and insulin pathways and lipid synthesis, in addition to their canonical function controlling cell cycle progression. Here we describe recent data demonstrating the role of cell cycle regulators in the metabo...
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - February 7, 2020 Category: Molecular Biology Source Type: research

Editorial Board
Publication date: 1 April 2020Source: Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease, Volume 1866, Issue 4Author(s): (Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease)
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - February 7, 2020 Category: Molecular Biology Source Type: research

Tumour functional imaging by PET
Publication date: Available online 5 February 2020Source: Biochimica et Biophysica Acta (BBA) - Molecular Basis of DiseaseAuthor(s): Ana Margarida Abrantes, Ana Salomé Pires, Lúcia Monteiro, Ricardo Teixo, Ana Rita Neves, Nuno Tiago Tavares, Inês Alexandra Marques, Maria Filomena BotelhoAbstractCarcinogenesis is a complex multistep process, characterized by changes at different levels, both genetic and epigenetic, which alter cell metabolism. Positron emission tomography (PET) is a very sensitive image modality that allows to evaluate oncometabolism. PET functionalities are immense, since by labelling a...
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - February 5, 2020 Category: Molecular Biology Source Type: research

Visualization and analysis of the interaction network of proteins associated with blood-cell targeting autoimmune diseases
This study aims to investigate the interaction network of proteins associated with BLADs (BLAD interactome) and to identify novel associations with other human proteins. The method followed in this study combines information regarding protein-protein interaction network properties and autoimmune disease terms. Proteins with high network scores and statistically significant autoimmune disease term enrichment were obtained and 14 of them were designated as candidate proteins associated with BLADs. Additionally, clustering analysis of the BLAD interactome was used and allowed the detection of 17 proteins that act as “co...
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - February 4, 2020 Category: Molecular Biology Source Type: research

Metabolomic studies of breast cancer in murine models: A review
Publication date: Available online 31 January 2020Source: Biochimica et Biophysica Acta (BBA) - Molecular Basis of DiseaseAuthor(s): Rita Araújo, Daniela Bispo, Luisa A. Helguero, Ana M. GilAbstractBackgroundMetabolomic strategies have been extensively used to search for biomarkers of disease, including cancer, in biological complex mixtures such as cells, tissues and biofluids. In breast cancer research, murine models are of great value and metabolomics has been increasingly applied to characterize tumor or organ tissues, or biofluids, for instance to follow-up metabolism during cancer progression or response to sp...
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - February 1, 2020 Category: Molecular Biology Source Type: research

Regulation of cardiac O-GlcNAcylation: More than just nutrient availability
Publication date: Available online 31 January 2020Source: Biochimica et Biophysica Acta (BBA) - Molecular Basis of DiseaseAuthor(s): Helen E. Collins, John C. ChathamAbstractThe post-translational modification of serine and threonine residues of nuclear, cytosolic, and mitochondrial proteins by O-linked β-N-acetyl glucosamine (O-GlcNAc) has long been seen as an important regulatory mechanism in the cardiovascular system. O-GlcNAcylation of cardiac proteins has been shown to contribute to the regulation of transcription, metabolism, mitochondrial function, protein quality control and turnover, autophagy, and calcium ha...
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - January 31, 2020 Category: Molecular Biology Source Type: research

What are we talking about when we talk about education and Chagas? A systematic review of the issue
In this study, we surveyed, described, and critically analyzed studies approaching the link education-Chagas disease in scientific publications from the last 15 years. We aimed at contributing methodological-theoretical elements to (re)think the development of educational research and experiences that truly help facing this issue. From the electronic search of scientific literature in 6 databases, we found 426 articles, out of which we selected 25. We incorporated 10 articles from other sources to this initial corpus and performed both qualitative and quantitative analyses over the total number [35] to characterize the s...
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - January 30, 2020 Category: Molecular Biology Source Type: research

Signaling pathways that regulate Trypanosoma cruzi infection and immune response
Publication date: Available online 28 January 2020Source: Biochimica et Biophysica Acta (BBA) - Molecular Basis of DiseaseAuthor(s): Fabio Marcelo Cerbán, Cinthia Carolina Stempin, Ximena Volpini, Antonio Carrera Silva, Susana Gea, Claudia Cristina MotranAbstractCurrent understanding of key cellular pathways, which are activated by the interaction between T. cruzi and host immunity, is crucial for controlling T. cruzi infection and also for limiting the development of the immunopathological symptoms of Chagas´ disease. Here, we focus on recent advances in the knowledge of modulation of innate receptors such as...
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - January 29, 2020 Category: Molecular Biology Source Type: research

Hepatic FNDC5 is a potential local protective factor against Non-Alcoholic Fatty Liver
In conclusion, our human and experimental data strongly suggest that the hepatic expression of FNDC5 increased with hepatic steatosis and its upregulation in hepatocytes could dampen the development of NAFLD by negatively regulating steatogenesis and hepatocyte death. (Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease)
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - January 29, 2020 Category: Molecular Biology Source Type: research

Chagas disease: Historic perspective
Publication date: Available online 27 January 2020Source: Biochimica et Biophysica Acta (BBA) - Molecular Basis of DiseaseAuthor(s): Carlos A. Vigliano, Chen Chao, José L. LeoneAbstractThis review is a perspective on the history of Chagas disease, and it adopts a novel approach from literary studies, historical documents and the science and epidemiology of the nature of the disease. From this analysis, comes the review's working definition of the Contact Zone (CZ): “the space in which geographically and historically separated people come into contact with each other and establish long-lasting relationships, wh...
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - January 29, 2020 Category: Molecular Biology Source Type: research

Molecular changes in hepatic metabolism in ZDSD rats–A new polygenic rodent model of obesity, metabolic syndrome, and diabetes
Publication date: Available online 24 January 2020Source: Biochimica et Biophysica Acta (BBA) - Molecular Basis of DiseaseAuthor(s): Lu Han, Stefanie Bittner, Dachuan Dong, Yuan Cortez, Alex Bittner, Jackie Chan, Meenakshi Umar, Wen-Jun Shen, Richard G. Peterson, Fredric B. Kraemer, Salman AzharAbstractIn recent years, the prevalence of obesity, metabolic syndrome and type 2 diabetes is increasing dramatically. They share pathophysiological mechanisms and often lead to cardiovascular diseases. The ZDSD rat was suggested as a new animal model to study diabetes and the metabolic syndrome. In the current study, we have furthe...
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - January 26, 2020 Category: Molecular Biology Source Type: research

Interleukin-17 mediated immunity during infections with Trypanosoma cruzi and other protozoans
Publication date: Available online 24 January 2020Source: Biochimica et Biophysica Acta (BBA) - Molecular Basis of DiseaseAuthor(s): María Carolina Amezcua Vesely, Constanza Rodriguez, Adriana Gruppi, Eva Virginia Acosta RodríguezAbstractHost resistance during infection with Trypanosoma cruzi, and other protozoans, is dependent on a balanced immune response. Robust immunity against these pathogens requires of the concerted action of many innate and adaptive cell populations including macrophages, neutrophils, dendritic cells, CD4+, and CD8+ T cells and B cells among others. Indeed, during most protozoan infec...
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - January 26, 2020 Category: Molecular Biology Source Type: research

Parasite-host glycan interactions during Trypanosoma cruzi infection: trans-Sialidase rides the show
Publication date: Available online 20 January 2020Source: Biochimica et Biophysica Acta (BBA) - Molecular Basis of DiseaseAuthor(s): Oscar Campetella, Carlos A. Buscaglia, Juan Mucci, María Susana LeguizamónAbstractMany important pathogen-host interactions rely on highly specific carbohydrate binding events. In the case of the protozoan Trypanosoma cruzi, the causative agent of Chagas disease, glycointeractions involving sialic acid (SA) residues are pivotal for parasite infectivity, escape from immune surveillance and pathogenesis. Though unable to synthesize SA de novo, T. cruzi displays a unique trans-Sial...
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - January 21, 2020 Category: Molecular Biology Source Type: research

Mass spectrometry based proteomics and metabolomics in personalized oncology
Publication date: Available online 18 January 2020Source: Biochimica et Biophysica Acta (BBA) - Molecular Basis of DiseaseAuthor(s): Tomasz Kowalczyk, Michal Ciborowski, Joanna Kisluk, Adam Kretowski, Coral BarbasAbstractPrecision medicine (PM) means the customization of healthcare with decisions and practices adjusted to the individual patient. It includes personalized diagnostics, patients' sub-classification, individual treatment selection and the monitoring of its effectiveness. Currently, in oncology, PM is based on the molecular and cellular features of a tumor, its microenvironment and the patient's genetics and lif...
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - January 18, 2020 Category: Molecular Biology Source Type: research

Arachidonic acid inhibits inflammatory responses by binding to myeloid differentiation factor-2 (MD2) and preventing MD2/toll-like receptor 4 signaling activation
Publication date: Available online 14 January 2020Source: Biochimica et Biophysica Acta (BBA) - Molecular Basis of DiseaseAuthor(s): Yali Zhang, Hongjin Chen, Wenxin Zhang, Yan Cai, Peiren Shan, Di Wu, Bing Zhang, Hui Liu, Khan Zia, Guang LiangAbstractArachidonic acid (AA) plays a fundamental role in the function of all cells. Metabolites of AA contribute to inflammation as well as for resolving inflammation. Although AA-derived metabolites exhibit well-substantiated bioactivity, it is not known whether AA regulates inflammatory responses independent of its metabolites. With the recent discovery that saturated fatty acids ...
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - January 15, 2020 Category: Molecular Biology Source Type: research

IL-20 promotes hypoxia/reoxygenation-induced mitochondrial dysfunction and apoptosis in cardiomyocytes by upregulating oxidative stress by activating the PKC/NADPH oxidase pathway
Publication date: Available online 15 January 2020Source: Biochimica et Biophysica Acta (BBA) - Molecular Basis of DiseaseAuthor(s): Kun-Ling Tsai, Pei-Ling Hsieh, Wan-Ching Chou, Ching-Hsia Hung, Hsin-Lun Yang, Yun-Ching Chang, Pei-Ming Chu, Ming-Shi Chang, Shih-Hung ChanAbstractAcute myocardial infarction (AMI) is the maximum critical cardiovascular event and causes high morbidity and mortality worldwide. The ischemia and reperfusion that occur in AMI cause apoptosis and cellular dysfunction in cardiomyocytes. IL-20, an IL-10 family member, is involved in various inflammatory diseases. Therefore, we sought to elucidate t...
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - January 15, 2020 Category: Molecular Biology Source Type: research

Plasma-derived exosomes contribute to pancreatitis-associated lung injury by triggering NLRP3-dependent pyroptosis in alveolar macrophages
Publication date: Available online 15 January 2020Source: Biochimica et Biophysica Acta (BBA) - Molecular Basis of DiseaseAuthor(s): Xiao-Bo Wu, Hong-Yu Sun, Zhu-Lin Luo, Long Cheng, Xing-Mei Duan, Jian-Dong RenAbstractProgression of acute pancreatitis (AP) into a severe form usually results in a life-threatening condition with multiple organ dysfunction, and in particular acute lung injury (ALI), often contributes to the majority of AP-associated deaths. Increasing evidence has shown that uncontrolled activation of the immune system with rapid production of inflammatory cytokines play a dominant role in this process. As a...
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - January 15, 2020 Category: Molecular Biology Source Type: research

Neuroprotection from optic nerve injury and modulation of oxidative metabolism by transplantation of active mitochondria to the retina
Publication date: Available online 15 January 2020Source: Biochimica et Biophysica Acta (BBA) - Molecular Basis of DiseaseAuthor(s): Gabriel Nascimento-dos-Santos, Eduardo de-Souza-Ferreira, Rafael Lani, Caroline Coelho de Faria, Victor Guedes Araujo, Leandro Coelho Teixeira-Pinheiro, Taliane de Souza Vasconcelos, Thaís Gonçalo Leandro, Marcelo Felippe Santiago, Rafael Linden, Antonio Galina, Hilda Petrs-SilvaAbstractMitochondrial dysfunctions are linked to a series of neurodegenerative human conditions, including Parkinson's disease, schizophrenia, optic neuropathies, and glaucoma. Recently, a series of stud...
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - January 15, 2020 Category: Molecular Biology Source Type: research

Relationship between changes in the exon-recognition machinery and SLC22A1 alternative splicing in hepatocellular carcinoma
Publication date: Available online 15 January 2020Source: Biochimica et Biophysica Acta (BBA) - Molecular Basis of DiseaseAuthor(s): Meraris Soto, Maria Reviejo, Ruba Al-Abdulla, Marta R. Romero, Rocio I.R. Macias, Loreto Boix, Jordi Bruix, Maria A. Serrano, Jose J.G. MarinAbstractChanges in the phenotype that characterizes cancer cells are partly due to altered processing of pre-mRNA by the spliceosome. We have previously reported that aberrant splicing plays an essential role in the impaired response of hepatocellular carcinoma (HCC) to sorafenib by reducing the expression of functional organic cation transporter type 1 ...
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - January 15, 2020 Category: Molecular Biology Source Type: research

Disturbance of bioenergetics and calcium homeostasis provoked by metabolites accumulating in propionic acidemia in heart mitochondria of developing rats
Publication date: Available online 10 January 2020Source: Biochimica et Biophysica Acta (BBA) - Molecular Basis of DiseaseAuthor(s): Ana Cristina Roginski, Alessandro Wajner, Cristiane Cecatto, Simone Magagnin Wajner, Roger Frigério Castilho, Moacir Wajner, Alexandre Umpierrez AmaralAbstractPropionic acidemia is caused by lack of propionyl-CoA carboxylase activity. It is biochemically characterized by accumulation of propionic (PA) and 3-hydroxypropionic (3OHPA) acids and clinically by severe encephalopathy and cardiomyopathy. High urinary excretion of maleic acid (MA) and 2-methylcitric acid (2MCA) is also found in...
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - January 11, 2020 Category: Molecular Biology Source Type: research

Duchenne muscular dystrophy is associated with the inhibition of calcium uniport in mitochondria and an increased sensitivity of the organelles to the calcium-induced permeability transition
Publication date: Available online 8 January 2020Source: Biochimica et Biophysica Acta (BBA) - Molecular Basis of DiseaseAuthor(s): Mikhail V. Dubinin, Eugeny Yu. Talanov, Kirill S. Tenkov, Vlada S. Starinets, Irina B. Mikheeva, Mars G. Sharapov, Konstantin N. BelosludtsevAbstractDuchenne muscular dystrophy (DMD) is characterized by a pronounced and progressive degradation of the structure of skeletal muscles, which decreases their strength and lowers endurance of the organism. At muscular dystrophy, mitochondria are known to undergo significant functional changes, which is manifested in a decreased efficiency of oxidative...
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - January 9, 2020 Category: Molecular Biology Source Type: research

Future perspectives: What lies ahead for Neuronal Ceroid Lipofuscinosis research?
Publication date: Available online 8 January 2020Source: Biochimica et Biophysica Acta (BBA) - Molecular Basis of DiseaseAuthor(s): Jonathan D. Cooper, Sara E. MoleAbstractThe increasing pace of progress in all aspects of Neuronal Ceroid Lipofuscinosis (NCL) research is highlighted by the breadth and depth of review articles [[1], [2], [3], [4], [5], [6], [7], [8], [9], [10]] in this Special Issue that provide a helpful snapshot of the field between the previous international meeting, NCL 2018, and the next, NCL 2020. These advances encompass several areas that were previously thought intractable, ranging from basic scienc...
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - January 9, 2020 Category: Molecular Biology Source Type: research

Dietary zinc restriction promotes degeneration of the endocrine pancreas in mice
Publication date: Available online 9 January 2020Source: Biochimica et Biophysica Acta (BBA) - Molecular Basis of DiseaseAuthor(s): Tháyna Sisnande, Cleverton K. Lima, Dayana Cabral da Silva, Thayana Moulin Beninatto, Natália Leão Alves, Mariana J. Amaral, Leandro Miranda-Alves, Luís Maurício T.R. LimaAbstractZinc is a key component of several proteins, interacting with the pancreatic hormones insulin and amylin. The role of zinc in insulin oligomerization and crystallinity is well established, although the effects of dietary zinc restriction on both energetic metabolism and β-pancre...
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - January 9, 2020 Category: Molecular Biology Source Type: research

Stem Cells for Treatment of Musculoskeletal Conditions - Orthopaedic/Sports Medicine Applications
Publication date: Available online 30 November 2019Source: Biochimica et Biophysica Acta (BBA) - Molecular Basis of DiseaseAuthor(s): Mimi Zumwalt, Arubala P. ReddyAbstractA myriad of musculoskeletal conditions afflicts a vast number of the world's population from birth to death. Countless pathological diseases and traumatic injuries (acute and chronic) contribute to different human disabilities, causing a tremendous financial toll on the economy of healthcare. The medical field is continually searching for novel ways to combat orthopedically related conditions. The immediate goal is the restoration of anatomy then ultimat...
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - January 8, 2020 Category: Molecular Biology Source Type: research

Mitochondrial permeability transition pore is involved in oxidative burst and NETosis of human neutrophils
Publication date: Available online 8 January 2020Source: Biochimica et Biophysica Acta (BBA) - Molecular Basis of DiseaseAuthor(s): Nina Vorobjeva, Ivan Galkin, Olga Pletjushkina, Sergei Golyshev, Roman Zinovkin, Anastasia Prikhodko, Vladimir Pinegin, Irina Kondratenko, Boris Pinegin, Boris ChernyakAbstractNeutrophils release neutrophil extracellular traps (NETs) in response to numerous pathogenic microbes as the last suicidal resource (NETosis) in the fight against infection. Apart from the host defense function, NETs play an essential role in the pathogenesis of various autoimmune and inflammatory diseases. Therefore, un...
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - January 8, 2020 Category: Molecular Biology Source Type: research

Editorial Board
Publication date: 1 March 2020Source: Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease, Volume 1866, Issue 3Author(s): (Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease)
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - January 8, 2020 Category: Molecular Biology Source Type: research

Glycogen storage in a zebrafish Pompe disease model is reduced by 3-BrPA treatment
Publication date: Available online 7 January 2020Source: Biochimica et Biophysica Acta (BBA) - Molecular Basis of DiseaseAuthor(s): Cinzia Bragato, Silvia Carra, Flavia Blasevich, Franco Salerno, Alessia Brix, Andrea Bassi, Monica Beltrame, Franco Cotelli, Lorenzo Maggi, Renato Mantegazza, Marina MoraAbstractPompe disease (PD) is an autosomal recessive muscular disorder caused by deficiency of the glycogen hydrolytic enzyme acid α-glucosidase (GAA). The enzyme replacement therapy, currently the only available therapy for PD patients, is efficacious in improving cardiomyopathy in the infantile form, but not equally ef...
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - January 8, 2020 Category: Molecular Biology Source Type: research

Protective effect of curcumin nanoparticles against cardiotoxicity induced by doxorubicin in rat
Publication date: Available online 7 January 2020Source: Biochimica et Biophysica Acta (BBA) - Molecular Basis of DiseaseAuthor(s): Haitham S. Mohammed, Eman N. Hosny, Yasser A. Khadrawy, Merna Magdy, Yasmen S. Attia, Omnia A. Sayed, Mahmoud AbdElaalAbstractThe present study designed to investigate the protective effect of curcumin nanoparticles (CUR-NPs) on the cardiotoxicity induced by doxorubicin. Rats were divided into four groups; control, rats treated daily with CUR-NPs (50 mg/kg) for 14 days, rats treated with an acute dose of doxorubicin (20 mg/kg) and rats treated daily with CUR-NPs for 14 days injected wi...
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - January 8, 2020 Category: Molecular Biology Source Type: research

A pantothenate kinase-deficient mouse model reveals a gene expression program associated with brain coenzyme a reduction
Publication date: Available online 7 January 2020Source: Biochimica et Biophysica Acta (BBA) - Molecular Basis of DiseaseAuthor(s): Chitra Subramanian, Jiangwei Yao, Matthew W. Frank, Charles O. Rock, Suzanne JackowskiAbstractPantothenate kinase (PanK) is the first enzyme in the coenzyme A (CoA) biosynthetic pathway. The differential expression of the four-active mammalian PanK isoforms regulates CoA levels in different tissues and PANK2 mutations lead to Pantothenate Kinase Associated Neurodegeneration (PKAN). The molecular mechanisms that potentially underlie PKAN pathophysiology are investigated in a mouse model of CoA ...
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - January 8, 2020 Category: Molecular Biology Source Type: research

Regulation of autophagy and apoptosis by Dp44mT-mediated activation of AMPK in pancreatic cancer cells
Publication date: Available online 2 January 2020Source: Biochimica et Biophysica Acta (BBA) - Molecular Basis of DiseaseAuthor(s): S. Krishan, S. Sahni, L.Y.W. Leck, P.J. Jansson, D.R. RichardsonAbstractUpon activation, the 5′-adenosine monophosphate-activated protein kinase (AMPK) increases catabolism, while inhibiting anabolism. The anti-cancer agent, di-2-pyridylketone 4,4-dimethyl-3-thiosemicarbazone (Dp44mT), activates AMPK in multiple tumor cell-types (Biochim. Biophys Acta 2016;1863:2916–2933). This acts as an initial cell “rescue response” after iron-depletion mediated by Dp44mT. Considerin...
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - January 3, 2020 Category: Molecular Biology Source Type: research

Chagas disease vaccine design: the search for an efficient Trypanosoma cruzi immune-mediated control
Publication date: Available online 3 January 2020Source: Biochimica et Biophysica Acta (BBA) - Molecular Basis of DiseaseAuthor(s): Augusto E. Bivona, Andrés Sánchez Alberti, Natacha Cerny, Sebastián N. Trinitario, Emilio L. MalchiodiAbstractChagas disease is currently endemic to 21 Latin-American countries and has also become a global concern because of globalization and mass migration of chronically infected individuals. Prophylactic and therapeutic vaccination might contribute to control the infection and the pathology, as complement of other strategies such as vector control and chemotherapy. Ideal...
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - January 3, 2020 Category: Molecular Biology Source Type: research

Mitochondrial genetics cooperate with nuclear genetics to selectively alter immune cell development/trafficking
Publication date: Available online 30 December 2019Source: Biochimica et Biophysica Acta (BBA) - Molecular Basis of DiseaseAuthor(s): T.C. Beadnell, C. Fain, C.J. Vivian, J.C.G. King, R. Hastings, M.A. Markiewicz, D.R. WelchAbstractThe nuclear genome drives differences in immune cell populations and differentiation potentials, in part regulated by changes in metabolism. Despite this connection, the role of mitochondrial DNA (mtDNA) polymorphisms (SNP) in this process has not been examined. Using mitochondrial nuclear exchange (MNX) mice, we and others have shown that mtDNA strongly influences varying aspects of cell biolog...
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - December 30, 2019 Category: Molecular Biology Source Type: research

Role of TNFα and leptin signaling in colon cancer incidence and tumor growth under obese phenotype
Publication date: Available online 28 December 2019Source: Biochimica et Biophysica Acta (BBA) - Molecular Basis of DiseaseAuthor(s): Snahlata Singh, Shyamananda Singh Mayengbam, Surbhi Chouhan, Bhavana Deshmukh, Pranay Ramteke, Dipti Athavale, Manoj Kumar BhatAbstractEpidemiological studies over the last few decades have shown a strong influence of obesity on colon cancer risk and its progression. These studies have primarily focussed on the role of adipokines in driving cancer progression. We investigated the incidence of cancerous polyp formation and tumor progression in presence and absence of functional leptin along w...
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - December 29, 2019 Category: Molecular Biology Source Type: research