Forward and reverse genetics approaches to uncover metabolic aging pathways in Caenorhabditis elegans
Publication date: Available online 15 September 2017 Source:Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease Author(s): Arwen W. Gao, Jelmi uit de Bos, Mark G. Sterken, Jan E. Kammenga, Reuben L. Smith, Riekelt H. Houtkooper Abstract. The biological mechanisms of aging have been studied in depth and prominent findings in this field promote the development of new therapies for age-associated disorders. Various model organisms are used for research on aging; among these, the nematode Caenorhabditis elegans has been widely used and has provided valuable knowledge in determining the regulatory mechanisms drivi...
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - September 15, 2017 Category: Molecular Biology Source Type: research

Netrins & amp; Semaphorins: Novel regulators of the immune response
Publication date: Available online 14 September 2017 Source:Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease Author(s): Jordyn Feinstein, Bhama Ramkhelawon Netrins and semaphorins, members of the neuronal guidance cue family, exhibit a rich biology with significant roles that extend beyond chemotactic guidance of the axons to build the neuronal patterns of the body. Screening of adult tissues and specific cellular subsets have illuminated that these proteins are also abundantly expressed under both steady state and pathological scenarios. This observation suggests that, in addition to their role in the dev...
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - September 14, 2017 Category: Molecular Biology Source Type: research

Regulation of mitochondrial bioenergetics by the non-canonical roles of mitochondrial dynamics proteins in the heart
Publication date: Available online 14 September 2017 Source:Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease Author(s): Wang Wang, Celia Fernandez-Sanz, Shey-Shing Sheu Recent advancement in mitochondrial research has significantly extended our knowledge on the role and regulation of mitochondria in health and disease. One important breakthrough is the delineation of how mitochondrial morphological changes, termed mitochondrial dynamics, are coupled to the bioenergetics and signaling functions of mitochondria. In general, it is believed that fusion leads to an increased mitochondrial respiration efficiency...
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - September 14, 2017 Category: Molecular Biology Source Type: research

Differential effects of FXR or TGR5 activation in cholangiocarcinoma progression
Conclusion: Activation of FXR inhibits, whereas TGR5 activation may promote, CCA progression by regulating proliferation, migration and mitochondrial energy metabolism. Modulation of FXR or TGR5 activities may represent potential therapeutic strategies for CCA. (Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease)
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - September 13, 2017 Category: Molecular Biology Source Type: research

The translocator protein ligand XBD173 improves clinical symptoms and neuropathological markers in the SJL/J mouse model of multiple sclerosis
Publication date: Available online 9 September 2017 Source:Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease Author(s): Géraldine Leva, Christian Klein, Jérémie Benyounes, François Hallé, Frédéric Bihel, Nicolas Collongues, Jérôme De Seze, Ayikoe-Guy Mensah-Nyagan, Christine Patte-Mensah Multiple sclerosis (MS) is a severe autoimmune disease characterized by inflammatory, demyelinating and neurodegenerative components causing motor, sensory, visual and/or cognitive symptoms. The relapsing-remitting MS affecting 85% of patients is reliably mimicke...
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - September 10, 2017 Category: Molecular Biology Source Type: research

MiR-525-3p mediates antiviral defense to rotavirus infection by targeting nonstructural protein 1
This study was undertaken to demonstrate the importance of cellular miRNAs during RV (human Wa RV or Rhesus RV) strain infection. Twenty-nine differentially regulated miRNAs were identified during RV infection, and miR-525-3p was downregulated and validated by quantitative real-time polymerase chain reaction (qRT-PCR). MiR-525-3p mimic inhibited RV replication in dose-dependent manner. Correspondingly, the miR-525-3p inhibitors enhanced RV replication. We confirmed that miR-525-3p was complementary to the 3′ untranslated region (UTR) of nonstructural protein 1(NSP1) of RV (Wa or Rhesus) strains. Interestingly, miR-52...
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - September 8, 2017 Category: Molecular Biology Source Type: research

Regulated expression of the TP β isoform of the human T Prostanoid receptor by the tumour suppressors FOXP1 and NKX3.1: Implications for the role of Thromboxane in Prostate Cancer
Publication date: Available online 8 September 2017 Source:Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease Author(s): Aine G. O'Sullivan, Sarah B. Eivers, Eamon P. Mulvaney, B. Therese Kinsella The prostanoid thromboxane (TX)A2 signals through the TPα and TPβ isoforms of T Prostanoid receptor (TP) that are transcriptionally regulated by distinct promoters termed Prm1 and Prm3, respectively, within the TBXA2R gene. We recently demonstrated that expression of TPα and TPβ is increased in PCa, differentially correlating with Gleason grade and with altered CpG methylation of the individua...
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - September 8, 2017 Category: Molecular Biology Source Type: research

Novel patient missense mutations in the HSD17B10 gene affect dehydrogenase and mitochondrial tRNA modification functions of the encoded protein
This study therefore identified two distinctive molecular mechanisms to explain the biochemical defects for the novel missense patient mutations. Graphical abstract (Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease)
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - September 7, 2017 Category: Molecular Biology Source Type: research

Structural determinants in ApoA-I amyloidogenic variants explain improved cholesterol metabolism despite low HDL levels
Publication date: Available online 6 September 2017 Source:Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease Author(s): Rita Del Giudice, Joan Domingo-Espín, Ilaria Iacobucci, Oktawia Nilsson, Maria Monti, Daria Maria Monti, Jens O. Lagerstedt Twenty Apolipoprotein A-I (ApoA-I) variants are responsible for a systemic hereditary amyloidosis in which protein fibrils can accumulate in different organs, leading to their failure. Several ApoA-I amyloidogenic mutations are also associated with hypoalphalipoproteinemia, low ApoA-I and high-density lipoprotein (HDL)-cholesterol plasma levels; however, subjec...
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - September 6, 2017 Category: Molecular Biology Source Type: research

Drug-induced bile duct injury
Publication date: Available online 4 September 2017 Source:Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease Author(s): Michele Visentin, Daniela Lenggenhager, Zhibo Gai, Gerd A. Kullak-Ublick Drug-induced liver injury includes a spectrum of pathologies, some related to the mode of injury, some to the cell type primarily damaged. Among these, drug-induced bile duct injury is characterized by the destruction of the biliary epithelium following exposure to a drug. Most of the drugs associated with bile duct injury cause immune-mediated lesions to the epithelium of interlobular ducts. These share common histop...
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - September 5, 2017 Category: Molecular Biology Source Type: research

Epinecidin-1 protects mice from LPS-induced endotoxemia and cecal ligation and puncture-induced polymicrobial sepsis
Publication date: Available online 4 September 2017 Source:Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease Author(s): Bor-Chyuan Su, Han-Ning Huang, Tai-Wen Lin, Chwan-Deng Hsiao, Jyh-Yih Chen The antimicrobial peptide, epinecidin-1 (Epi), was identified from Epinephelus coioides and may have clinical application for treating sepsis. Epi has been shown to ameliorate antibiotic-resistant bacteria-induced sepsis in mice, but further evaluation in mixed-flora models and a description of the protective mechanisms are essential to establish this peptide as a potential therapeutic. Therefore, we first tested th...
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - September 4, 2017 Category: Molecular Biology Source Type: research

MDA-MB-231 breast cancer cells fuel osteoclast metabolism and activity: A new rationale for the pathogenesis of osteolytic bone metastases
Publication date: Available online 1 September 2017 Source:Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease Author(s): Silvia Lemma, Gemma Di Pompo, Paolo E. Porporato, Martina Sboarina, Shonagh Russell, Robert J. Gillies, Nicola Baldini, Pierre Sonveaux, Sofia Avnet Recent progress in dissecting the molecular paracrine circuits of cancer and stromal cells in bone metastases (BM) are offering new options to improve current merely palliative approach. The study of tumor-stroma metabolic interplay may further ameliorate this scenario. In this context, we demonstrated that highly glycolytic MDA-MB-231 cancer ...
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - September 2, 2017 Category: Molecular Biology Source Type: research

Wnt/ β-catenin signaling links embryonic lung development and asthmatic airway remodeling
Publication date: Available online 1 September 2017 Source:Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease Author(s): Musaddique Hussain, Chengyun Xu, Meiping Lu, Xiling Wu, Lanfang Tang, Ximei Wu Embryonic lung development requires reciprocal endodermal-mesodermal interactions; mediated by various signaling proteins. Wnt/β-catenin is a signaling protein that exhibits the pivotal role in lung development, injury and repair while aberrant expression of Wnt/β-catenin signaling leads to asthmatic airway remodeling: characterized by hyperplasia and hypertrophy of airway smooth muscle cells, alveolar...
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - September 2, 2017 Category: Molecular Biology Source Type: research

Reversal of aberrant PI3K/Akt signaling by Salubrinal in a GalT-deficient mouse model
In this study, we compared the expression levels of the PI3K/Akt signaling pathway in normal and GalT-deficient mouse tissues. In mutant mouse ovaries, phospho-Akt [pAkt (Ser473)] and pGsk3β were reduced by 62.5% and 93.5%, respectively (p < 0.05 versus normal controls). In mutant cerebella, pAkt (Ser473) and pGsk3β were reduced by 62%, 50%, respectively (p < 0.05). To assess the role of ER stress in the down-regulation of PI3K/Akt signaling, we examined if administration of Salubrinal, a chemical compound that alleviates ER stress, to GalT-deficient fibroblasts and animals could normalize the pathw...
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - August 27, 2017 Category: Molecular Biology Source Type: research

Mitochondrial dysfunction in rat splenocytes following hemorrhagic shock
This article is part of a Special Issue entitled: Immune and Metabolic Alterations in Trauma and Sepsis edited by Dr. Raghavan Raju. (Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease)
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - August 27, 2017 Category: Molecular Biology Source Type: research

Endpoints in the design of clinical trials for primary sclerosing cholangitis
This article is part of a Special Issue entitled: Cholangiocytes in Health and Diseaseedited by Jesus Banales, Marco Marzioni, Nicholas LaRusso and Peter Jansen. (Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease)
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - August 26, 2017 Category: Molecular Biology Source Type: research

Omega-3 polyunsaturated fatty acids ameliorate ethanol-induced adipose hyperlipolysis: A mechanism for hepatoprotective effect against alcoholic liver disease
In this study, the fat-1 transgenic mice that synthesizes endogenous n-3 from n-6 PUFA and their wild type littermates with an exogenous n-3 PUFA enriched diet were subjected to a chronic ethanol feeding plus a single binge as model to induce liver injury with adipose lipolysis. Additionally, the differentiated adipocytes from 3T3-L1 cells were treated with docosahexaenoic acid or eicosapentaenoic acid for mechanism studies. Our results demonstrated that endogenous and exogenous n-3 PUFA enrichment ameliorates ethanol-stimulated adipose lipolysis by increasing PDE3B activity and reducing cAMP accumulation in adipocyte, whi...
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - August 26, 2017 Category: Molecular Biology Source Type: research

Mechanisms of endothelial cell dysfunction in cystic fibrosis
We examined cells from human umbilical cords (HUVEC) and pulmonary artery isolated from non-cystic fibrosis (PAEC) and CF human lungs (CF-PAEC), under static conditions or physiological shear. CFTR activity, clearly detected in HUVEC and PAEC, was markedly reduced in CF-PAEC. CFTR blockade increased endothelial permeability to macromolecules and reduced trans‑endothelial electrical resistance (TEER). Consistent with this, CF-PAEC displayed lower TEER compared to PAEC. Under shear, CFTR blockade reduced VE-cadherin and p120 catenin membrane expression and triggered the formation of paxillin- and vinculin-enriched membrane...
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - August 26, 2017 Category: Molecular Biology Source Type: research

Insulin-like growth factor-1 signaling in cardiac aging
Publication date: Available online 25 August 2017 Source:Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease Author(s): Wang-Soo Lee, Jaetaek Kim Cardiovascular disease (CVD) is the leading cause of death in most developed countries. Aging is associated with enhanced risk of CVD. Insulin-like growth factor-1 (IGF-1) binds to its cognate receptor, IGF-1 receptor (IGF-1R), and exerts pleiotropic effects on cell growth, differentiation, development, and tissue repair. Importantly, IGF-1/IGF-1R signaling is implicated in cardiac aging and longevity. Cardiac aging is an intrinsic process that results in cardiac dy...
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - August 26, 2017 Category: Molecular Biology Source Type: research

Cholemic nephropathy – Historical notes and novel perspectives
This article is part of a Special Issue entitled: Cholangiocytes in Health and Diseaseedited by Jesus Banales, Marco Marzioni, Nicholas LaRusso and Peter Jansen. (Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease)
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - August 26, 2017 Category: Molecular Biology Source Type: research

Interleukin-6 deficiency facilitates myocardial dysfunction during high fat diet-induced obesity by promoting lipotoxicity and inflammation
Conclusion Our results suggest that IL-6 contributes to limit lipid metabolic disorder, cardiac hypertrophy, fibrosis, inflammation and myocardium lipotoxicity during HFD-induced obesity. Graphical abstract (Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease)
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - August 25, 2017 Category: Molecular Biology Source Type: research

How the biliary tree maintains immune tolerance?
This article is part of a Special Issue entitled: Cholangiocytes in Health and Diseaseedited by Jesus Banales, Marco Marzioni, Nicholas LaRusso and Peter Jansen. (Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease)
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - August 25, 2017 Category: Molecular Biology Source Type: research

Targeting cholangiocarcinoma
This article is part of a Special Issue entitled: Cholangiocytes in Health and Diseaseedited by Jesus Banales, Marco Marzioni, Nicholas LaRusso and Peter Jansen. (Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease)
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - August 25, 2017 Category: Molecular Biology Source Type: research

miR-4516, a MicroRNA downregulated in psoriasis inhibits keratinocyte motility by targeting fibronectin/Integrin α9 signaling
Publication date: Available online 24 August 2017 Source:Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease Author(s): Shruti Chowdhari, Kabir Sardana, Neeru Saini Psoriasis is recognized as a T cell mediated inflammatory hyperproliferative skin disorder. Several microRNAs (miRNAs) have been implicated in the pathogenesis of psoriasis however, understanding of their mechanistic involvement remains unclear. Previously, we have shown that PUVA induced miR-4516 downregulates signal transducer and activator of transcription 3 (STAT3) by direct binding to its 3′ untranslated region (3’UTR) and suppres...
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - August 25, 2017 Category: Molecular Biology Source Type: research

Growth arrest and DNA damage-inducible 45 α protects against nonalcoholic steatohepatitis induced by methionine- and choline-deficient diet
Publication date: Available online 24 August 2017 Source:Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease Author(s): Naoki Tanaka, Shogo Takahashi, Xiao Hu, Lu Yu, Naoyuki Fujimori, Srujana Golla, Zhong-Ze Fang, Toshifumi Aoyama, Kristopher W. Krausz, Frank J. Gonzalez Growth arrest and DNA damage-inducible 45 α (Gadd45α) is a stress-inducible protein that plays an important role in cell survival/death and DNA repair, but its contribution to the development of nonalcoholic steatohepatitis (NASH) has not been investigated. C57BL/6 Gadd45a-null and wild-type (WT) mice were treated with methionine...
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - August 25, 2017 Category: Molecular Biology Source Type: research

BRD4 has dual effects on the HMGB1 and NF- κB signalling pathways and is a potential therapeutic target for osteoarthritis
Publication date: Available online 24 August 2017 Source:Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease Author(s): Jiang Yafei, Zhu Libo, Zhang Tao, Lu Haiming, Wang Cong, Xue Bao, Xu Xun, Liu Yu, Cai Zhengdong, Sang Weilin, Hua Yingqi, Ma Jinzhong Osteoarthritis (OA) has traditionally been defined as a non-inflammatory disease. Recently, many studies have demonstrated that OA also has an inflammatory component. BRD4, a member of the Bromodomain and Extra-Terminal Domain family, has emerged as an important regulator of some chronic inflammatory diseases. JQ1, an antagonist of BRD4, modulates transcriptio...
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - August 25, 2017 Category: Molecular Biology Source Type: research

Inhibition of insulin/PI3K/AKT signaling decreases adipose Sortilin 1 in mice and 3T3-L1 adipocytes
In conclusion, this study revealed an important role of insulin signaling in regulating adipose Sort1 protein stability, and further suggests that impaired insulin signaling may underlie reduced adipose Sort1 in obesity. The cellular events downstream of insulin/PI3K/AKT signaling that mediates insulin regulation of Sort1 stability requires further investigation. (Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease)
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - August 25, 2017 Category: Molecular Biology Source Type: research

Role of membrane GM1 on early neuronal membrane actions of A β during onset of Alzheimer's disease
In this study, we examined if the association and subsequent membrane perforation induced by Aβ was dependent on GM1 levels. Pretreatment of hippocampal neurons with D-PDMP decreased GM1 and Aβ clustering at the membrane (Aβ fluorescent-punctas/20μm, control=16.2±1.1 vs. D-PDMP =6.4±0.4, p <0.001). Interestingly, membrane perforation with Aβ occurred with a slower time course when the GM1 content was diminished (time to establish perforated configuration (TEPC) (min): control=7.8±2 vs. low GM1=12.1±0.5, p <0.01), suggesting that the presence of GM1 in the mem...
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - August 25, 2017 Category: Molecular Biology Source Type: research

Adiponectin attenuates NADPH oxidase-mediated oxidative stress and neuronal damage induced by cerebral ischemia-reperfusion injury
In conclusion, APN-P inhibits neuronal apoptosis and alleviates oxidative stress in neurons subjected to I/R, suggesting that it may be beneficial for the treatment of brain damage following ischemic stroke. (Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease)
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - August 25, 2017 Category: Molecular Biology Source Type: research

Optimising the clinical strategy for autoimmune liver diseases: Principles of value-based medicine
Conclusions We have generated a comprehensive set of COIs which sequentially capture different clinical outcome of the three AILDs explored. These indicators represent a critical tool to implement a value-based approach to patients with these conditions, to monitor, compare and improve quality through benchmarking of clinical performance and to assess the significance of novel drugs and technologies. This article is part of a Special Issue entitled: Cholangiocytes in Health and Diseaseedited by Jesus Banales, Marco Marzioni, Nicholas LaRusso and Peter Jansen. (Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease)
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - August 25, 2017 Category: Molecular Biology Source Type: research

Bile acid receptors in the biliary tree: TGR5 in physiology and disease
This article is part of a Special Issue entitled: Cholangiocytes in Health and Diseaseedited by Jesus Banales, Marco Marzioni, Nicholas LaRusso and Peter Jansen. (Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease)
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - August 25, 2017 Category: Molecular Biology Source Type: research

Cholangiocytes in the pathogenesis of primary sclerosing cholangitis and development of cholangiocarcinoma
This article is part of a Special Issue entitled: Cholangiocytes in Health and Diseaseedited by Jesus Banales, Marco Marzioni, Nicholas LaRusso and Peter Jansen. (Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease)
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - August 25, 2017 Category: Molecular Biology Source Type: research

The HRH4 rs11662595 Mutation is Associated with Histamine H4 Receptor Dysfunction and with Increased Epithelial-to-Mesenchymal Transition Progress in Non-Small Cell Lung Cancer
In conclusion, these findings reveal that rs11662595 is a loss-of-function polymorphism that results in dysfunction of HRH4 and attenuates the anti-EMT function of HRH4 in NSCLC, which provides a promising biomarker for prognosis and therapy of NSCLC. (Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease)
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - August 25, 2017 Category: Molecular Biology Source Type: research

Immune and metabolic alterations following trauma and sepsis – An overview
Publication date: Available online 24 August 2017 Source:Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease Author(s): Raghavan Raju (Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease)
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - August 24, 2017 Category: Molecular Biology Source Type: research

A novel PMCA3 mutation in an ataxic patient with hypomorphic phosphomannomutase 2 (PMM2) heterozygote mutations: Biochemical characterization of the pump defect
Publication date: Available online 12 August 2017 Source:Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease Author(s): Mattia Vicario, Tito Calì, Domenico Cieri, Francesca Vallese, Raissa Bortolotto, Raffaele Lopreiato, Francesco Zonta, Marta Nardella, Alessia Micalizzi, Dirk J. Lefeber, Enza Maria Valente, Enrico Bertini, Giuseppe Zanotti, Ginevra Zanni, Marisa Brini, Ernesto Carafoli The neuron-restricted isoform 3 of the plasma membrane Ca2+ ATPase plays a major role in the regulation of Ca2+ homeostasis in the brain, where the precise control of Ca2+ signaling is a necessity. Several function-affe...
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - August 12, 2017 Category: Molecular Biology Source Type: research

Autophagy regulates accumulation and functional activity of granulocytic myeloid-derived suppressor cells via STAT3 signaling in endotoxin shock
Publication date: Available online 9 August 2017 Source:Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease Author(s): Guanjun Dong, Chuanping Si, Qingqing Zhang, Fenglian Yan, Chunxia Li, Hui Zhang, Qun Ma, Jun Dai, Zhihua Li, Hui Shi, Bo Wang, Junfeng Zhang, Jiankuo Ming, Yuan Hu, Shuo Geng, Yao Zheng, Liwu Li, Huabao Xiong Autophagy extensively participates in immune responses and inflammatory diseases. Myeloid-derived suppressor cells (MDSCs) are derived from CD11b+ Gr1+ cells under pathological conditions and play an immunosuppressive role in the pathogenesis of cancer and inflammatory diseases. However,...
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - August 9, 2017 Category: Molecular Biology Source Type: research

Melanocortin Receptors
Publication date: Available online 8 August 2017 Source:Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease Author(s): Ya-Xiong Tao (Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease)
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - August 8, 2017 Category: Molecular Biology Source Type: research

Ablation of systemic SIRT1 activity promotes nonalcoholic fatty liver disease by affecting liver-mesenteric adipose tissue fatty acid mobilization
Publication date: Available online 5 August 2017 Source:Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease Author(s): Junrui Cheng, Chun Liu, Kangquan Hu, Andrew Greenberg, Dayong Wu, Lynne M. Ausman, Michael W. McBurney, Xiang-Dong Wang Sirtuin 1 (SIRT1) has been reported to protect against nonalcoholic fatty liver disease (NAFLD) development. The mechanism of how SIRT1 deacetylase activity affects NAFLD has not been well investigated. The current investigation addressed the causal effect of systemic SIRT1 activity on NAFLD development and the underlying mechanism involved in both liver and mesenteric adipo...
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - August 6, 2017 Category: Molecular Biology Source Type: research

The search for novel diagnostic and prognostic biomarkers in cholangiocarcinoma
This article is part of a Special Issue entitled: Cholangiocytes in Health and Diseaseedited by Jesus Banales, Marco Marzioni, Nicholas LaRusso and Peter Jansen. (Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease)
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - August 4, 2017 Category: Molecular Biology Source Type: research

Genetics and mechanisms of hepatic cystogenesis
Publication date: Available online 4 August 2017 Source:Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease Author(s): L.F.M. van de Laarschot, J.P.H. Drenth Polycystic liver disease (PLD) is a heterogeneous genetic condition. PKD1 and PKD2 germline mutations are found in patients with autosomal dominant polycystic kidney disease (ADPKD). Autosomal dominant polycystic liver disease (ADPLD) is associated with germline mutations in PRKCSH, SEC63, LRP5, and recently ALG8, and SEC61. GANAB mutations are found in both patient groups. Loss of heterozygosity of PLD-genes in cyst epithelium contributes to the develop...
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - August 4, 2017 Category: Molecular Biology Source Type: research

MiR-485-5p modulates mitochondrial fission through targeting mitochondrial anchored protein ligase in cardiac hypertrophy
In this study, we found that hypertrophic agonist phenylephrine (PE) enhanced the expression of MAPL and promoting mitochondrial fission, while it decreased the expression of mitochondrial fusion protein (Mfn2) in hypertrophic cardiomyocytes. Silencing expression of MAPL by siRNA attenuated PE-induced depletion of Mfn2 and increase of mitochondrial fission as well as hypertrophic response in cultured primary cardiomyocytes. MiR-485-5p is screened as a candidate inhibitor of MAPL. Overexpression of miR-485-5p blocked mitochondrial fission and hypertrophy induced by PE through inhibiting MAPL expression and increasing the le...
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - August 4, 2017 Category: Molecular Biology Source Type: research

On the role of IgG4 in inflammatory conditions: lessons for IgG4-related disease
This article is part of a Special Issue entitled: Cholangiocytes in Health and Diseaseedited by Jesus Banales, Marco Marzioni, Nicholas LaRusso and Peter Jansen. (Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease)
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - August 4, 2017 Category: Molecular Biology Source Type: research

Repopulating the Biliary Tree from the Peribiliary Glands
This article is part of a Special Issue entitled: Cholangiocytes in Health and Diseaseedited by Jesus Banales, Marco Marzioni, Nicholas LaRusso and Peter Jansen. (Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease)
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - August 2, 2017 Category: Molecular Biology Source Type: research

Corrigendum to “Disruption of calpain reduces lipotoxicity-induced cardiac injury by preventing endoplasmic reticulum stress” [Biochim. Biophy.Acta 1862 (11): 2023-2033]
Publication date: September 2017 Source:Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease, Volume 1863, Issue 9 Author(s): S Li, L Zhang, R Ni, T Cao, D Zheng, S Xiong, PA Greer, GC Fan, T Peng (Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease)
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - July 28, 2017 Category: Molecular Biology Source Type: research

Corrigendum to “Relationship of angiotensin I-converting enzyme (ACE) and bradykinin B2 receptor (BDKRB2) polymorphism with diabetic nephropathy” [BBA - Molecular Basis of Disease Vol.1863/Iss.6(2017) 1264–1272]
Publication date: September 2017 Source:Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease, Volume 1863, Issue 9 Author(s): Honghong Zou, Guoqing Wu, Jinlei Lv, Gaosi Xu (Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease)
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - July 28, 2017 Category: Molecular Biology Source Type: research

Bezafibrate prevents mitochondrial dysfunction, antioxidant system disturbance, glial reactivity and neuronal damage induced by sulfite administration in striatum of rats: Implications for a possible therapeutic strategy for sulfite oxidase deficiency
Publication date: September 2017 Source:Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease, Volume 1863, Issue 9 Author(s): Mateus Grings, Alana Pimentel Moura, Belisa Parmeggiani, Julia Tauana Pletsch, Gabriela Miranda Fernandez Cardoso, Pauline Maciel August, Cristiane Matté, Angela T.S. Wyse, Moacir Wajner, Guilhian Leipnitz Sulfite accumulates in tissues of patients affected by sulfite oxidase (SO) deficiency, a neurometabolic disease characterized by seizures and progressive encephalopathy, often resulting in early death. We investigated the effects of sulfite on mitochondrial function, antioxida...
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - July 28, 2017 Category: Molecular Biology Source Type: research

Reelin protects from colon pathology by maintaining the intestinal barrier integrity and repressing tumorigenic genes
In conclusion, the results highlight a role for reelin in maintaining intestinal epithelial cell homeostasis and providing resistance against colon pathology. (Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease)
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - July 28, 2017 Category: Molecular Biology Source Type: research

Electroencephalographic and biochemical long –lasting abnormalities in animal model of febrile seizure
Publication date: September 2017 Source:Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease, Volume 1863, Issue 9 Author(s): Haitham S. Mohammed, Heba S. Aboul Ezz, Heba M. Sayed, Maha A. Ali Febrile seizures (FS) are convulsions associated with high body temperature. It has a high incidence in children from the age of 6months to 5years and may have adverse consequences in adulthood. The experimental model of FS could be induced in animals via hyperthermia. The present study was designed to investigate persistent electroencephalographic (EEG), neurochemical and behavioral alterations in adult animals that had...
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - July 28, 2017 Category: Molecular Biology Source Type: research

Targeted downregulation of dMyc restricts neurofibrillary tangles mediated pathogenesis of human neuronal tauopathies in Drosophila
Publication date: September 2017 Source:Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease, Volume 1863, Issue 9 Author(s): Soram Idiyasan Chanu, Surajit Sarkar Formation of Neurofibrillary Tangles (NFTs) in neuronal tissues has been implicated as the hallmark of disease pathogenesis and tau mediated toxicity in human and mammalian models. However, previous studies had failed to correlate NFT formation with pathogenesis of human neuronal tauopathies in Drosophila disease models. Though, a recent report suggests formation of tau mediated NFTs like structures confined to dopaminergic neurons in Drosophila adul...
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - July 28, 2017 Category: Molecular Biology Source Type: research

BAG-1M co-activates BACE1 transcription through NF- κB and accelerates Aβ production and memory deficit in Alzheimer’s disease mouse model
In this report, we found that over-expression of BAG-1M enhances BACE1-mediated cleavage of amyloid precursor protein (APP) and Aβ production by up-regulating BACE1 gene transcription. The regulation of BACE1 transcription by BAG-1M was dependent on NF-κB, as BAG-1M complexes NF-κB at the promoter of BACE1 gene and co-activates NF-κB-facilitated BACE1 transcription. Moreover, expression of BAG-1M by lentiviral vector in the hippocampus of AD transgenic model mice promotes Aβ generation and formation of neuritic plaque, and subsequently accelerates memory deficits of the mice. These results provi...
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - July 23, 2017 Category: Molecular Biology Source Type: research