Editorial Board
Publication date: 1 July 2019Source: Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease, Volume 1865, Issue 7Author(s): (Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease)
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - May 18, 2019 Category: Molecular Biology Source Type: research

Post-translational modifications in brain health and disease
Publication date: Available online 15 May 2019Source: Biochimica et Biophysica Acta (BBA) - Molecular Basis of DiseaseAuthor(s): Sónia C. Correia, Cristina Carvalho, Susana Cardoso, Paula I. Moreira (Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease)
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - May 16, 2019 Category: Molecular Biology Source Type: research

Hemin-induced platelet activation and ferroptosis is mediated through ROS-driven proteasomal activity and inflammasome activation: Protection by Melatonin
In this study, we demonstrate that hemin-induced ferroptosis in platelets is mediated through ROS-driven proteasome activity and inflammasome activation, which were mitigated by Melatonin (MLT). Although inflammasome activation is linked with pyroptosis, it is still not clear whether ferroptosis is associated with inflammasome activation. Our study for the first time demonstrates an association of platelet activation/ferroptosis with proteasome activity and inflammasome activation. Although, high-throughput screening has recognized ferrostatin-1 (Fer-1) and liproxstatin-1 (Lip-1) as potent ferroptosis inhibitors, having an...
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - May 16, 2019 Category: Molecular Biology Source Type: research

Activation of NFKB-JMJD3 signaling promotes bladder fibrosis via boosting bladder smooth muscle cell proliferation and collagen accumulation
Publication date: Available online 16 May 2019Source: Biochimica et Biophysica Acta (BBA) - Molecular Basis of DiseaseAuthor(s): Junyu Lai, Manqing Ge, Sikui Shen, Tao Jin, Kunjie Wang, Qiang Wei, Hong Li, Jianzhong AiAbstractChronic cystitis is characterized by the hyperplasia and fibrosis of the bladder wall as well as attenuated compliance of the bladder. To further unravel its underlying molecular mechanism, the role of NFκB-JMJD3 signaling pathway in cystitis induced bladder fibrosis was investigated. Jmjd3 and Col1/3 expression was detected in a cystitis mouse model that was developed by intraperitoneal injecti...
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - May 16, 2019 Category: Molecular Biology Source Type: research

Activated NF-κB/Nrf2 and Wnt/β-catenin pathways are associated with lipid metabolism in CKD patients with microalbuminuria and macroalbuminuria
This study first demonstrates the association of plasma inflammation, oxidative stress, Wnt/β-catenin and lipid metabolism in CKD patients with microalbuminuria and macroalbuminuria.Graphical abstract (Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease)
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - May 16, 2019 Category: Molecular Biology Source Type: research

Mitophagy and mitochondrial integrity in cardiac ischemia-reperfusion injury
Publication date: Available online 14 May 2019Source: Biochimica et Biophysica Acta (BBA) - Molecular Basis of DiseaseAuthor(s): Mingjie Yang, Becky S. Linn, Yingmei Zhang, Jun RenAbstractIschemia-reperfusion injury (IR injury), produced by initial interruption and subsequent restoration of organ blood flow, is an important clinical dilemma accompanied by various cardiac reperfusion strategies following acute myocardial infarction (AMI). Although the restored blood flow is necessary for oxygen and nutrient supply, reperfusion often results in pathological sequelae leading to elevated ischemic damage. Among various theories...
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - May 16, 2019 Category: Molecular Biology Source Type: research

Asparaginyl endopeptidase induces endothelial permeability and tumor metastasis via downregulating zonula occludens protein ZO-1
Publication date: Available online 13 May 2019Source: Biochimica et Biophysica Acta (BBA) - Molecular Basis of DiseaseAuthor(s): Lichun Kang, Long Shen, Liqing Lu, Dekun Wang, Yong Zhao, Chuan'ai Chen, Lingfang Du, Junbo Gong, Yuying Zhang, Xue Mi, Rong Xiang, Mianzhi Zhang, Xiaoyue TanAbstractZona occludens-1 (ZO-1) is a key component of tight junctions that govern the function of the endothelial barrier against tumor metastasis. Factors secreted by tumor cells contribute to the maintenance of tumor vascular networks. How tumor cell-derived protein signals regulate ZO-1 expression is unclear. Here, we explored the effect ...
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - May 15, 2019 Category: Molecular Biology Source Type: research

d-Ribose contributes to the glycation of serum protein
Publication date: Available online 11 May 2019Source: Biochimica et Biophysica Acta (BBA) - Molecular Basis of DiseaseAuthor(s): Yao Chen, Lexiang Yu, Yujing Wang, Yan Wei, Yong Xu, Tao He, Rongqiao HeAbstractd-Ribose is active in glycation and rapidly produces advanced glycation end products, leading to cell death and to cognitive impairment in mice. Glycated serum protein (GSP) is a relatively short-term biomarker for glycemic control in diabetes mellitus. However, whether d-ribose is related to GSP is unclear. The aim of this work was to identify the contribution of d-ribose to GSP compared to d-glucose. Here, we showed...
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - May 12, 2019 Category: Molecular Biology Source Type: research

Molecular and cellular bases of diabetes: Focus on type 2 diabetes mouse model-TallyHo
This article summarizes various molecular mechanisms involved in the developments of diabetes, including biochemical characteristics, genetic and molecular links with Alzheimer's disease, β-cell function, and factors associated with diabetes. This will help us in the development of novel therapeutic strategies targeting AD in future. (Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease)
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - May 12, 2019 Category: Molecular Biology Source Type: research

Lipopolysaccharide and interferon-γ team up to activate HIF-1α via STAT1 in normoxia and exhibit sex differences in human aortic valve interstitial cells
Publication date: Available online 26 April 2019Source: Biochimica et Biophysica Acta (BBA) - Molecular Basis of DiseaseAuthor(s): Iván Parra-Izquierdo, Irene Castaños-Mollor, Javier López, Cristina Gómez, J. Alberto San Román, Mariano Sánchez Crespo, Carmen García-RodríguezAbstractIn early stages of calcific aortic valve disease (CAVD), immune cells infiltrate into the valve leaflets and release cytokines such as interferon (IFN)-γ. IFN-γ has context-dependent direct effects, and also regulates other immune pathways. The purpose of this study was addressi...
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - May 9, 2019 Category: Molecular Biology Source Type: research

Missense variant in TPI1 (Arg189Gln) causes neurologic deficits through structural changes in the triosephosphate isomerase catalytic site and reduced enzyme levels in vivo
Publication date: Available online 7 May 2019Source: Biochimica et Biophysica Acta (BBA) - Molecular Basis of DiseaseAuthor(s): Bartholomew P. Roland, Kristen R. Richards, Stacy L. Hrizo, Samantha Eicher, Zackery J. Barile, Tien-Chien Chang, Grace Savon, Paola Bianchi, Elisa Fermo, Bianca Maria Ricerca, Luca Tortorolo, Jerry Vockley, Andrew P. VanDemark, Michael J. PalladinoAbstractMutations in the gene triosephosphate isomerase (TPI) lead to a severe multisystem condition that is characterized by hemolytic anemia, a weakened immune system, and significant neurologic symptoms such as seizures, distal neuropathy, and intell...
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - May 9, 2019 Category: Molecular Biology Source Type: research

DNA methylation changes in genes coding for leptin and insulin receptors during metabolic-altered pregnancies
Publication date: Available online 7 May 2019Source: Biochimica et Biophysica Acta (BBA) - Molecular Basis of DiseaseAuthor(s): Francisca Stolzenbach, Sharin Valdivia, Patricia Ojeda-Provoste, Fernando Toledo, Luis Sobrevía, Bredford KerrAbstractThe overwhelming rates of obesity worldwide are a major concern due to the elevated medical costs associated and the poor quality of life of obese patients. In the recent years, it has become evident that the intrauterine milieu can have a long-term impact on the foetus health. The placenta is a highly dynamic organ; whose primary function is to carry nutrients from the moth...
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - May 9, 2019 Category: Molecular Biology Source Type: research

CXCR7 contributes to the aggressive phenotype of cholangiocarcinoma cells
In conclusion, CXCR7 is widely expressed in CCA, and contributes to the aggressive phenotype of CCA cells, inducing cell migration, invasion, adhesion, survival, growth and stem cell-like features. Cell migration induced by CXCR7 requires interaction with β-arrestin 2. (Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease)
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - May 4, 2019 Category: Molecular Biology Source Type: research

Editorial Board
Publication date: 1 June 2019Source: Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease, Volume 1865, Issue 6Author(s): (Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease)
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - May 4, 2019 Category: Molecular Biology Source Type: research

COL1A1 C-propeptide mutations cause ER mislocalization of procollagen and impair C-terminal procollagen processing
Publication date: Available online 2 May 2019Source: Biochimica et Biophysica Acta (BBA) - Molecular Basis of DiseaseAuthor(s): Aileen M. Barnes, Aarthi Ashok, Elena N. Makareeva, Marina Brusel, Wayne A. Cabral, MaryAnn Weis, Catherine Moali, Emmanuel Bettler, David R. Eyre, John P. Cassella, Sergey Leikin, David J.S. Hulmes, Efrat Kessler, Joan C. MariniAbstractMutations in the type I procollagen C-propeptide occur in ~6.5% of Osteogenesis Imperfecta (OI) patients. They are of special interest because this region of procollagen is involved in α chain selection and folding, but is processed prior to fibril assembly a...
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - May 2, 2019 Category: Molecular Biology Source Type: research

Reduction in urinary oxalate excretion in mouse models of Primary Hyperoxaluria by RNA interference inhibition of liver lactate dehydrogenase activity
Publication date: Available online 2 May 2019Source: Biochimica et Biophysica Acta (BBA) - Molecular Basis of DiseaseAuthor(s): Kyle D. Wood, Ross P. Holmes, David Erbe, Abigail Liebow, Sonia Fargue, John KnightAbstractThe Primary Hyperoxaluria's (PH) are rare autosomal recessive disorders characterized by elevated oxalate production. PH patients suffer recurrent calcium oxalate kidney stone disease, and in severe cases end stage renal disease. Recent evidence has shown that RNA interference may be a suitable approach to reduce oxalate production in PH patients by knocking down key enzymes involved in hepatic oxalate synth...
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - May 2, 2019 Category: Molecular Biology Source Type: research

Astaxanthin exerts protective effects similar to bexarotene in Alzheimer's disease by modulating amyloid beta and cholesterol homeostasis in blood-brain barrier endothelial cells
Publication date: Available online 2 May 2019Source: Biochimica et Biophysica Acta (BBA) - Molecular Basis of DiseaseAuthor(s): Elham Fanaee-Danesh, Chaitanya Chakravarthi Gali, Jelena Tadic, Martina Zandl-Lang, Alexandra Kober, Vicente Roca Agujetas, Cristina de Dios, Carmen Tam-Amersdorfer, Anika Stracke, Nicole Maria Albrecher, Anil Paul Chirackal Manavalan, Marielies Reiter, Yidan Sun, Anna Colell, Frank Madeo, Ernst Malle, Ute PanzenboeckAbstractThe pathogenesis of Alzheimer's disease (AD) is characterized by overproduction, impaired clearance, and deposition of amyloid-β peptides (Aβ) and connected to chole...
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - May 2, 2019 Category: Molecular Biology Source Type: research

Blood-brain barrier dysfunction underlying Alzheimer's disease is induced by an SSAO/VAP-1-dependent cerebrovascular activation with enhanced Aβ deposition
Publication date: Available online 30 April 2019Source: Biochimica et Biophysica Acta (BBA) - Molecular Basis of DiseaseAuthor(s): Montse Solé, María Esteban-Lopez, Biel Taltavull, Cristina Fábregas, Rut Fadó, Núria Casals, Jose Rodríguez-Álvarez, Alfredo J. Miñano-Molina, Mercedes UnzetaAbstractDysfunctions of the vascular system directly contribute to the onset and progression of Alzheimer's disease (AD). The blood-brain barrier (BBB) shows signs of malfunction at early stages of the disease. When Abeta peptide (Aβ) is deposited on brain vessels, it induces vas...
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - April 30, 2019 Category: Molecular Biology Source Type: research

Lipopolysaccharide and interferon-γ team up to activate HIF-1 via STAT1 in normoxia and exhibit sex differences in human aortic valve interstitial cells
Publication date: Available online 26 April 2019Source: Biochimica et Biophysica Acta (BBA) - Molecular Basis of DiseaseAuthor(s): Iván Parra-Izquierdo, Irene Castaños-Mollor, Javier López, Cristina Gómez, J. Alberto San Román, Mariano Sánchez Crespo, Carmen García-RodrígueAbstractIn early stages of calcific aortic valve disease (CAVD), immune cells infiltrate into the valve leaflets and release cytokines such as interferon (IFN)-γ. IFN-γ has context-dependent direct effects, and also regulates other immune pathways. The purpose of this study was addressin...
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - April 27, 2019 Category: Molecular Biology Source Type: research

Cholesterol metabolism, pancreatic β-cell function and diabetes
Publication date: Available online 26 April 2019Source: Biochimica et Biophysica Acta (BBA) - Molecular Basis of DiseaseAuthor(s): Carla Perego, Lorenzo Da Dalt, Angela Pirillo, Alessandra Galli, Alberico L. Catapano, Giuseppe D. NorataAbstractCholesterol plays an essential role in determining cell membrane physico-chemical characteristics and functions. A proper membrane structure is critical in pancreatic β-cells for glucose-mediated insulin secretion, and alterations in cellular cholesterol content may negatively affect this process, leading to β-cell dysfunction. The low density lipoprotein receptor (LDL-R) a...
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - April 26, 2019 Category: Molecular Biology Source Type: research

METTL14 is essential for β-cell survival and insulin secretion
Publication date: Available online 26 April 2019Source: Biochimica et Biophysica Acta (BBA) - Molecular Basis of DiseaseAuthor(s): Jun Liu, Guanzheng Luo, Juan Sun, Lili Men, Honggang Ye, Chuan He, Decheng RenAbstractDefects in the development, maintenance or expansion of β-cell mass can result in impaired glucose metabolism and diabetes. N6-methyladenosine affects mRNA stability and translation efficiency, and impacts cell differentiation and stress response. To determine if there is a role for m6A in β-cells, we investigated the effect of Mettl14, a key component of the m6A methyltransferase complex, on β-...
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - April 26, 2019 Category: Molecular Biology Source Type: research

The db mutation improves memory in younger mice in a model of Alzheimer's disease
Publication date: Available online 26 April 2019Source: Biochimica et Biophysica Acta (BBA) - Molecular Basis of DiseaseAuthor(s): Le Zhang, Sun-Ok Fernandez-Kim, Tina L. Beckett, Dana M. Niedowicz, Katharina Kohler, Kalavathi Dasuri, Annadora J. Bruce-Keller, M. Paul Murphy, Jeffrey N. KellerAbstractAlzheimer's disease (AD) is the most common age-related neurodegenerative disease, while obesity is a major global public health problem associated with the metabolic disorder type 2 diabetes mellitus (T2DM). Chronic obesity and T2DM have been identified as invariant risk factors for dementia and late-onset AD, while their imp...
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - April 26, 2019 Category: Molecular Biology Source Type: research

H+-dependent inorganic phosphate transporter in breast cancer cells: Possible functions in the tumor microenvironment
In this study, we characterized the H+-dependent Pi transport in breast cancer cell (MDA-MB-231) and around the cancer tissue. MDA-MB-231 cell line presented higher levels of H+-dependent Pi transport as compared to other breast cell lines, such as MCF-10A, MCF-7 and T47-D. The Pi transport was linear as a function of time and exhibited a Michaelis-Menten kinetic of Km = 1.387 ± 0.1674 mM Pi and Vmax = 198.6 ± 10.23 Pi × h−1 × mg protein−1 hence reflecting a low affinity Pi transport. H+-dependent Pi uptake was higher at acidic pH. FCCP, Bafilomycin A1 and SCH28080, w...
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - April 26, 2019 Category: Molecular Biology Source Type: research

Cardiac myocyte KLF5 regulates body weight via alteration of cardiac FGF21
Publication date: Available online 26 April 2019Source: Biochimica et Biophysica Acta (BBA) - Molecular Basis of DiseaseAuthor(s): Christine J. Pol, Nina M. Pollak, Michael J. Jurczak, Effimia Zacharia, Iordanes Karagiannides, Ioannis D. Kyriazis, Panagiotis Ntziachristos, Diego A. Scerbo, Brett R. Brown, Iannis Aifantis, Gerald I. Shulman, Ira J. Goldberg, Konstantinos DrosatosAbstractCardiac metabolism affects systemic energetic balance. Previously, we showed that Krüppel-like factor (KLF)-5 regulates cardiomyocyte PPARα and fatty acid oxidation-related gene expression in diabetes. We surprisingly found that c...
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - April 26, 2019 Category: Molecular Biology Source Type: research

Editorial Board
Publication date: 1 May 2019Source: Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease, Volume 1865, Issue 5Author(s): (Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease)
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - April 19, 2019 Category: Molecular Biology Source Type: research

MICAL2 is expressed in cancer associated neo-angiogenic capillary endothelia and it is required for endothelial cell viability, motility and VEGF response
Publication date: Available online 18 April 2019Source: Biochimica et Biophysica Acta (BBA) - Molecular Basis of DiseaseAuthor(s): Ivana Barravecchia, Sara Mariotti, Angela Pucci, Francesca Scebba, Chiara De Cesari, Silvio Bicciato, Enrico Tagliafico, Elena Tenedini, Carla Vindigni, Marco Cecchini, Gabriele Berti, Marianna Vitiello, Laura Poliseno, Chiara Maria Mazzanti, Debora AngeloniAbstractThe capacity of inducing angiogenesis is a recognized hallmark of cancer cells. The cancer microenvironment, characterized by hypoxia and inflammatory signals, promotes proliferation, migration and activation of quiescent endothelial...
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - April 19, 2019 Category: Molecular Biology Source Type: research

Metformin ameliorates endotoxemia-induced endothelial pro-inflammatory responses via AMPK-dependent mediation of HDAC5 and KLF2
Publication date: Available online 16 April 2019Source: Biochimica et Biophysica Acta (BBA) - Molecular Basis of DiseaseAuthor(s): Rui Tian, Ranran Li, Yiyun Liu, Jialin Liu, Tingting Pan, Ruyuan Zhang, Bingya Liu, Erzhen Chen, Yaoqing Tang, Hongping QuAbstractExaggerated endothelial pro-inflammatory response is a hallmark in the early stage of sepsis and contributes to the subsequent tissue injury and organ failure. The anti-inflammatory effects of AMP-activated protein kinase (AMPK) activator metformin in sepsis has been revealed. However, the underlying mechanisms remain not fully understood. In the present study, the p...
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - April 17, 2019 Category: Molecular Biology Source Type: research

The metastasis suppressor, NDRG1, differentially modulates the ER stress response in the presence and absence of the anti-cancer agent, Dp44mT
Publication date: Available online 11 April 2019Source: Biochimica et Biophysica Acta (BBA) - Molecular Basis of DiseaseAuthor(s): A.M. Merlot, G.M. Porter, S. Sahni, E.G.W. Lim, P. Peres, D.R. RichardsonAbstractThe metastasis suppressor, N-myc downstream regulated gene-1 (NDRG1), is a stress response protein that is involved in the inhibition of multiple oncogenic signaling pathways. Initial studies have linked NDRG1 and the endoplasmic reticulum (ER) stress response. Considering this, we extensively examined the mechanism by which NDRG1 regulates the ER stress response in pancreatic and colon cancer cells. We also examin...
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - April 13, 2019 Category: Molecular Biology Source Type: research

Dietary cholesterol is essential to mast cell activation and associated obesity and diabetes in mice
This study reproduced the obesity and diabetes inhibitory activities of DSCG and ketotifen from mice on a Western diet. Yet, such inhibitory effects were diminished in mice on the HFD. DSCG and ketotifen MC inhibitory activities were recovered from mice on the HFD supplemented with the same amount of cholesterol (1.25%) as that in the Western diet. DSCG and ketotifen effectively blunted the high-cholesterol diet-induced elevations of blood histamine and adipose tissue MC degranulation. Pearson's correlation test demonstrated significant and positive correlations between plasma histamine and total cholesterol or low-density...
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - April 10, 2019 Category: Molecular Biology Source Type: research

Epigenetic silencing of genes enhanced by collective role of reactive oxygen species and MAPK signaling downstream ERK/Snail axis: Ectopic application of hydrogen peroxide repress CDH1 gene by enhanced DNA methyltransferase activity in human breast cancer
We report that hydrogen peroxide (H2O2) treatment modulate CDH1 gene expression by epigenetic modification(s). Sublethal dosage of H2O2 treatment decrease of E-cadherin, increase DNMT1, HDAC1, Snail, Slug and enrich H3K9me3 and H3K27me3 in the CDH1 promoter. The effect of H2O2 was attenuated by ROS scavengers; NAC, lupeol and β-sitosterol. DNMT inhibitor, AZA prevented the H2O2 induced promoter-CpG-island methylation of CDH1. Treatment of cells with U0126 (inhibitor of ERK) reduced the expression of DNMT1, Snail and Slug, increased CDH1. This implicates that CDH1 is synergistically repressed by histone methylation, DN...
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - April 5, 2019 Category: Molecular Biology Source Type: research

DNA repair fidelity in stem cell maintenance, health, and disease
Publication date: Available online 4 April 2019Source: Biochimica et Biophysica Acta (BBA) - Molecular Basis of DiseaseAuthor(s): Chinnadurai Mani, P. Hemachandra Reddy, Komaraiah PalleAbstractStem cells are a sub population of cell types that form the foundation of our body, and have the potential to replicate, replenish and repair limitlessly to maintain the tissue and organ homeostasis. Increased lifetime and frequent replication set them vulnerable for both exogenous and endogenous agents-induced DNA damage compared to normal cells. To counter these damages and preserve genetic information, stem cells have evolved with...
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - April 5, 2019 Category: Molecular Biology Source Type: research

TRPC channels: Regulation, dysregulation and contributions to chronic kidney disease
Publication date: Available online 4 April 2019Source: Biochimica et Biophysica Acta (BBA) - Molecular Basis of DiseaseAuthor(s): Stuart E. Dryer, Hila Roshanravan, Eun Young KimAbstractMutations in the gene encoding canonical transient receptor potential-6 (TRPC6) channels result in severe nephrotic syndromes that typically lead to end-stage renal disease. Many but not all of these mutations result in a gain in the function of the resulting channel protein. Since those observations were first made, substantial work has supported the hypothesis that TRPC6 channels can also contribute to progression of acquired (non-genetic...
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - April 5, 2019 Category: Molecular Biology Source Type: research

Novel cell line models to study mechanisms and overcoming strategies of proteasome inhibitor resistance in multiple myeloma
Publication date: Available online 4 April 2019Source: Biochimica et Biophysica Acta (BBA) - Molecular Basis of DiseaseAuthor(s): Daniela Brünnert, Marianne Kraus, Thorsten Stühmer, Stefanie Kirner, Robin Heiden, Pankaj Goyal, Christoph Driessen, Ralf C. Bargou, Manik ChatterjeeAbstractExperimental data on resistance mechanisms of multiple myeloma (MM) to ixazomib (IXA), a second-generation proteasome inhibitor (PI), are currently lacking. We generated MM cell lines with a 10-fold higher resistance to IXA as their sensitive counterparts, and observed cross-resistance towards the PIs carfilzomib (CFZ) and bortezom...
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - April 5, 2019 Category: Molecular Biology Source Type: research

Reduction of SIRT1 blunts the protective effects of ischemic post-conditioning in diabetic mice by impairing the Akt signaling pathway
Publication date: Available online 4 April 2019Source: Biochimica et Biophysica Acta (BBA) - Molecular Basis of DiseaseAuthor(s): Mingge Ding, Lang Hu, Hongyan Yang, Chao Gao, Ke Zeng, Mingzhe Yu, Jiahao Feng, Jihuan Qiu, Chaoyang Liu, Feng Fu, Yan LiAbstractIschemic post-conditioning (IPO) activates Akt signaling to confer cardioprotection. The responsiveness of diabetic hearts to IPO is impaired. We hypothesized that decreased cardiac SIRT1, a positive regulator of Akt, may be responsible for the impaired responsiveness of diabetic hearts to IPO-mediated cardioprotection. High-fat diet and streptozotocin-induced diabetic...
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - April 5, 2019 Category: Molecular Biology Source Type: research

Role of proteases in dysfunctional placental vascular remodelling in preeclampsia
Publication date: Available online 5 April 2019Source: Biochimica et Biophysica Acta (BBA) - Molecular Basis of DiseaseAuthor(s): Jaime A. Gutiérrez, Isabel Gómez, Delia I. Chiarello, Rocío Salsoso, Andrés D. Klein, Enrique Guzmán-Gutiérrez, Fernando Toledo, Luis SobreviaAbstractPreeclampsia is a syndrome characterised by vascular dysfunction, impaired angiogenesis, and hypertension during pregnancy. Even when the precise pathophysiology of preeclampsia remains elusive, impaired vascular remodelling and placental angiogenesis in the placental villi and defective trophoblast invasio...
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - April 5, 2019 Category: Molecular Biology Source Type: research

Corrigendum to “Activation of bradykinin B2 receptor induced the inflammatory responses of cytosolic phospholipase A2 after the early traumatic brain injury.” [Biochem. Biophys. Acta Mol. Basis Dis. 1864/9 Pt B (2018) 2957–2971]
Publication date: Available online 3 April 2019Source: Biochimica et Biophysica Acta (BBA) - Molecular Basis of DiseaseAuthor(s): Honglu Chao, Liang Fan, Lin Zhao, Yinlong Liu, Chao Lin, Xiupeng Xu, Zheng Li, Zhongyuan Bao, Yan Liu, Xiaoming Wang, Yongping You, Ning Liu, Jing Ji (Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease)
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - April 4, 2019 Category: Molecular Biology Source Type: research

Notch-1 decreased expression contributes to leptin receptor downregulation in nasal epithelium from allergic turbinates
ConclusionThus, both the leptin and Notch-1 pathways appear to represent markers for epithelial homeostasis in allergic rhinitis. (Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease)
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - April 2, 2019 Category: Molecular Biology Source Type: research

Mitochondrial ALDH2 protects against lipopolysaccharide-induced myocardial contractile dysfunction by suppression of ER stress and autophagy
This study examined the effect of ALDH2 on endotoxemia-induced cardiac anomalies. Echocardiographic, cardiac contractile and intracellular Ca2+ properties were examined. Our results indicated that LPS impaired cardiac contractile function (reduced fractional shortening, LV end systolic diameter, peak shortening, maximal velocity of shortening/relengthening, prolonged relengthening duration, oxidation of SERCA, and intracellular Ca2+ mishandling), associated with ER stress, inflammation, O2− production, increased autophagy, CAMKKβ, phosphorylated AMPK and suppressed phosphorylation of mTOR, the effects of which w...
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - April 1, 2019 Category: Molecular Biology Source Type: research

Mitochondrial fusion and maintenance of mitochondrial homeostasis in diabetic retinopathy
This study aims to investigate the role of mitochondrial fusion in the development of diabetic retinopathy, and illustrate the molecular mechanism responsible for Mfn2 suppression. Using human retinal endothelial cells, manipulated for Mfn2, we investigated the role of fusion in mitochondrial structural and functional damage in diabetes. The molecular mechanism of its suppression in diabetic milieu was determined by investigating Mfn2 promoter DNA methylation, and confirmed using molecular and pharmacological inhibitors of DNA methylation. Similar studies were performed in the retinal microvasculature (prepared by hypotoni...
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - March 27, 2019 Category: Molecular Biology Source Type: research

Chemokine (C-C motif) ligand 2 gene ablation protects low-density lipoprotein and paraoxonase-1 double deficient mice from liver injury, oxidative stress and inflammation
This study demonstrates that CCL2 is a key molecule for the development of metabolic and histological alterations in the liver of mice sensitive to the development of hyperlipidemia and hepatic steatosis, a finding with potential to identify new therapeutic targets in liver diseases. (Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease)
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - March 22, 2019 Category: Molecular Biology Source Type: research

Intestinal vitamin D receptor modulates lipid metabolism, adipose tissue inflammation and liver steatosis in obese mice
ConclusionOur study suggests a VDR-mediated metabolic cross-talk between gut and adipose tissue, which significantly contributes to systemic lipid homeostasis. These results have important implications for use of the intestinal VDR as a therapeutic target for obesity and associated disorders. (Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease)
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - March 22, 2019 Category: Molecular Biology Source Type: research

LRP1 promotes synthetic phenotype of pulmonary artery smooth muscle cells in pulmonary hypertension
In conclusion, our results indicate that LRP1 promotes the dedifferentiation of PASMC from a contractile to a synthetic phenotype thus suggesting its contribution to vascular remodeling in PH. (Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease)
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - March 22, 2019 Category: Molecular Biology Source Type: research

Cartilage biomechanics: A key factor for osteoarthritis regenerative medicine
Publication date: Available online 22 March 2019Source: Biochimica et Biophysica Acta (BBA) - Molecular Basis of DiseaseAuthor(s): D. Martínez-Moreno, G. Jiménez, P. Gálvez-Martín, G. Rus, J.A. MarchalAbstractOsteoarthritis (OA) is a joint disorder that is highly extended in the global population. Several researches and therapeutic strategies have been probed on OA but without satisfactory long-term results in joint replacement. Recent evidences show how the cartilage biomechanics plays a crucial role in tissue development. This review describes how physics alters cartilage and its extracellular...
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - March 22, 2019 Category: Molecular Biology Source Type: research

Dystrobrevin is required postsynaptically for homeostatic potentiation at the Drosophila NMJ
Publication date: Available online 21 March 2019Source: Biochimica et Biophysica Acta (BBA) - Molecular Basis of DiseaseAuthor(s): Salinee Jantrapirom, Wutigri Nimlamool, Piya Temviriyanukul, Somaieh Ahmadian, Cody J. Locke, Graeme W. Davis, Masamitsu Yamaguchi, Jasprina N. Noordermeer, Lee G. Fradkin, Saranyapin PotikanondAbstractEvolutionarily conserved homeostatic systems have been shown to modulate synaptic efficiency at the neuromuscular junctions of organisms. While advances have been made in identifying molecules that function presynaptically during homeostasis, limited information is currently available on how post...
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - March 21, 2019 Category: Molecular Biology Source Type: research

Lysosome motility and distribution: Relevance in health and disease
Publication date: Available online 21 March 2019Source: Biochimica et Biophysica Acta (BBA) - Molecular Basis of DiseaseAuthor(s): Juan Esteban Oyarzún, Jonathan Lagos, Mary Carmen Vázquez, Cristian Valls, Catalina De la Fuente, María Isabel Yuseff, Alejandra R. Alvarez, Silvana ZanlungoAbstractLysosomes are dynamic organelles, which can fuse with a variety of targets and undergo constant regeneration. They can move along microtubules in a retrograde and anterograde fashion by using motor proteins, kinesin and dynein, being main players in extracellular secretion, intracellular components degradation a...
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - March 21, 2019 Category: Molecular Biology Source Type: research

PID1 regulates insulin-dependent glucose uptake by controlling intracellular sorting of GLUT4-storage vesicles
Publication date: Available online 21 March 2019Source: Biochimica et Biophysica Acta (BBA) - Molecular Basis of DiseaseAuthor(s): Alexander W. Fischer, Kirstin Albers, Christian Schlein, Frederike Sass, Lucia M. Krott, Hartwig Schmale, Philip L.S.M. Gordts, Ludger Scheja, Joerg HeerenAbstractThe phosphotyrosine interacting domain-containing protein 1 (PID1) serves as a cytosolic adaptor protein of the LDL receptor-related protein 1 (LRP1). By regulating its intracellular trafficking, PID1 controls the hepatic, LRP1-dependent clearance of pro-atherogenic lipoproteins. In adipose and muscle tissues, LRP1 is present in endos...
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - March 21, 2019 Category: Molecular Biology Source Type: research

Role of OCT4 in cancer stem-like cells and chemotherapy resistance
Publication date: Available online 21 March 2019Source: Biochimica et Biophysica Acta (BBA) - Molecular Basis of DiseaseAuthor(s): Ismail S. Mohiuddin, Sung-Jen Wei, Min H. KangAbstractCancer stem-like cells (CSCs) contribute to the tumorigenicity, progression, and chemoresistance of cancers. It is not known whether CSCs arise from normal stem cells or if they arise from differentiated cancer cells by acquiring self-renewal features. These CSCs share stem cell markers that normal stem cells express. There is a rising interest in octamer-binding transcription factor 4 (OCT4), one of the stem cell factors that are essential ...
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - March 21, 2019 Category: Molecular Biology Source Type: research

Using induced pluripotent stem cell neuronal models to study neurodegenerative diseases
We present evidence from previous works on the important phenotypic changes of various neuronal types in these neurological diseases. We also summarize efforts on conducting low- and high-throughput screening experiments with hiPSCs toward developing potential therapeutics for treatment of neurodegenerative diseases. Lastly, we discuss the limitations of hiPSCs culture system in studying neurodegenerative diseases and alternative strategies to overcome these drawbacks. (Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease)
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - March 18, 2019 Category: Molecular Biology Source Type: research

Corrigendum to “MLL3 enhances the transcription of PD-L1 and regulates anti-tumor immunity” [Biochim. Biophys. Acta, Mol. Basis Dis. 1865 (2) (Feb 1, 2019) 454–463]
Publication date: Available online 18 March 2019Source: Biochimica et Biophysica Acta (BBA) - Molecular Basis of DiseaseAuthor(s): Wei Xiong, Huanghao Deng, Changkun Huang, Cong Zeng, Chengzhu Jian, Kun Ye, Zhaohui Zhong, Xiaokun Zhao, Liang Zhu (Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease)
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - March 18, 2019 Category: Molecular Biology Source Type: research

GDF11 exhibits tumor suppressive properties in hepatocellular carcinoma cells by restricting clonal expansion and invasion
Publication date: Available online 16 March 2019Source: Biochimica et Biophysica Acta (BBA) - Molecular Basis of DiseaseAuthor(s): Monserrat Gerardo-Ramírez, Roberto Lazzarini-Lechuga, Sharik Hernández-Rizo, Javier Esteban Jiménez-Salazar, Arturo Simoni-Nieves, Carmen García-Ruiz, José Carlos Fernández-Checa, Jens U. Marquardt, Cedric Coulouarn, María Concepción Gutiérrez-Ruiz, Benjamín Pérez-Aguilar, Luis E. Gomez-QuirozAbstractGrowth differentiation factor 11 (GDF11) has been characterized as a key regulator of differentiation in cells that reta...
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - March 17, 2019 Category: Molecular Biology Source Type: research