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https://www.readbyqxmd.com/read/28214184/-systemic-lupus-erythematosus-and-lymphopenia-clinical-and-pathophysiological-features
#1
M Martin, A Guffroy, X Argemi, T Martin
Lymphopenia is frequent in systemic lupus erythematosus (SLE) and profound (<500/mm(3)) in 10% of cases. T lymphocytes, especially CD4+, are more affected than B cells. The pathophysiological mechanisms are complex, involving lymphocytotoxic antibodies, excess of apoptosis, increased susceptibility of T cells to complement mediated cytolysis, as well as lymphopoiesis impairment and lymphocyte sequestration. Lymphopenia in SLE is independent from other cytopenia and immunosuppressive drug regiments, and associated with disease activity, risk of flare and damage scores...
February 14, 2017: La Revue de Médecine Interne
https://www.readbyqxmd.com/read/28213698/rice-grassy-stunt-virus-p5-interacts-with-two-protein-components-of-the-plant-specific-cbl-cipk-ca-2-signaling-network-of-rice
#2
Guihong Xiong, Xiaojuan Liu, Ping Qiu, Xiaoyong Wu, Zhenguo Du, Jie Zhang, Liang Yang, Zujian Wu
Rice grassy stunt virus (RGSV) is a tenuivirus posing a threat to rice production in many South, Southeast, and East Asian countries. To date, no host factor interacting with RGSV has been reported. In this study, we screened a rice cDNA library with the GAL4-based yeast two-hybrid system using RGSV p5 as the bait. One of the candidate host factors interacting with RGSV p5 was found to be CBL-interacting protein kinase 25 (OsCIPK25), a member of the plant-specific CBL-CIPK Ca(2+) signaling network. The interaction between RGSV p5 and OsCIPK25, as well as OsCIPK5, which is closely related to OsCIPK25, was confirmed by their cellular co-localization and by a bimolecular fluorescence complementation assay in Nicotiana benthamiana cells...
February 17, 2017: Virus Genes
https://www.readbyqxmd.com/read/28210839/thrombotic-microangiopathy-caused-by-methionine-synthase-deficiency-diagnosis-and-treatment-pitfalls
#3
Maria Helena Vaisbich, Andressa Braga, Maria Gabrielle, Clarissa Bueno, Flávia Piazzon, Fernando Kok
BACKGROUND: Inborn errors of cobalamin (Cbl) metabolism form a large group of rare diseases. One of these, Cbl deficiency type C (CblC), is a well-known cause of thrombotic microangiopathy (TMA), especially in infants. However, there has only been a single published case of TMA associated to Cbl deficiency type G (CblG), also known as methionine synthase deficiency (MSD). CASE DIAGNOSIS/TREATMENT: A 21-month-old boy presented with pallor and oral ulcers during episodes of upper respiratory infection (URI)...
February 16, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28205538/complement-component-5-promotes-lethal-thrombosis
#4
Tomohiro Mizuno, Kengo Yoshioka, Masashi Mizuno, Mie Shimizu, Fumihiko Nagano, Tomoyuki Okuda, Naotake Tsuboi, Shoichi Maruyama, Tadashi Nagamatsu, Masaki Imai
Extracellular histones promote platelet aggregation and thrombosis; this is followed by induction of coagulation disorder, which results in exhaustion of coagulation factors. Complement component 5 (C5) is known to be associated with platelet aggregation and coagulation system activation. To date, the pathological mechanism underlying liver injury has remained unclear. Here, we investigated whether C5 promotes liver injury associated with histone-induced lethal thrombosis. C5-sufficient and C5-deficient mice received single tail vein injections of purified, unfractionated histones obtained from calf thymus (45-75 μg/g)...
February 16, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28205080/assessment-of-cu-zn-edta-parenteral-toxicity-in-calves
#5
Luis Emilio Fazzio, Diana Esther Rosa, Sebastian Julio Picco, Guillermo Alberto Mattioli
Copper (Cu) parenteral administration is used in a beef cow-calf operations to prevent or correct Cu deficiency in bovines. At present, Zinc (Zn) salts have been incorporated to complement Cu antioxidant effect. A risk of hepatotoxicity generated by overdose is a negative consequence of injectable Cu application. Cu-Zn EDTA appears as an alternative; however, data about its toxicity is unknown. The aim of this study was to assess toxicity risk of different doses of Cu-Zn EDTA in calves. Thirty two Aberdeen Angus calves of 162 (±20) kg BW were assigned to 4 groups (n = 8), homogeneous in weight, sex, and age...
February 15, 2017: Biological Trace Element Research
https://www.readbyqxmd.com/read/28202800/discovery-of-novel-pneumococcal-serotype-35d-a-natural-wcig-deficient-variant-of-serotype-35b
#6
K Aaron Geno, Jamil S Saad, Moon H Nahm
The pneumococcus (Streptococcus pneumoniae) remains a significant cause of morbidity and mortality, especially among those at the extremes of age. Its capsular polysaccharide is essential for systemic virulence. Over ninety serologically distinct pneumococcal capsular polysaccharides (serotypes) are recognized, but they are unequal in prevalence. Because antibodies against the capsule are protective, polysaccharide conjugate vaccines, which are constructed against the most prevalent serotypes, have made great reductions in pneumococcal disease caused by these serotypes...
February 15, 2017: Journal of Clinical Microbiology
https://www.readbyqxmd.com/read/28202596/the-3-hydroxyacyl-acp-dehydratase-component-of-the-plant-mitochondrial-fatty-acid-synthase-system
#7
Xin Guan, Yozo Okazaki, Andrew Lithio, Ling Li, Xuefeng Zhao, Huanan Jin, Dan Nettleton, Kazuki Saito, Basil J Nikolau
We report the characterization of the Arabidopsis 3-hydroxyacyl-acyl carrier protein (ACP) dehydratase (mtHD) component of the mitochondrial fatty acid synthase (mtFAS) system, encoded by AT5G60335. The mitochondrial localization and catalytic capability of mtHD were demonstrated with a green fluorescent protein (GFP) transgenesis experiment, and by in vivo complementation and in vitro enzymatic assays. RNAi knockdown lines with reduced mtHD expression exhibit traits typically associated with mtFAS mutants, namely a miniaturized morphological appearance, reduced lipoylation of lipoylated proteins, and altered metabolomes consistent with the reduced catalytic activity of lipoylated enzymes...
February 15, 2017: Plant Physiology
https://www.readbyqxmd.com/read/28195315/genetic-testing-facilitates-prepubertal-diagnosis-of-congenital-hypogonadotropic-hypogonadism
#8
Cheng Xu, Mariarosaria Lang-Muritano, Franziska Phan-Hug, Andrew A Dwyer, Gerasimos P Sykiotis, Daniele Cassatella, James Acierno, Moosa Mohammadi, Nelly Pitteloud
Neonatal micropenis and cryptorchidism raise the suspicion of congenital hypogonadotropic hypogonadism (CHH), a rare genetic disorder caused by GnRH deficiency. Low plasma testosterone levels and low gonadotropins during minipuberty provide a clinical diagnostic clue, yet these tests are seldomly performed in general practice. We report a male neonate with no family history of reproductive disorders who was born with micropenis and cryptorchidism. Hormonal testing at age 2.5 months showed low testosterone (0...
February 14, 2017: Clinical Genetics
https://www.readbyqxmd.com/read/28192899/comparison-of-the-mismatch-repair-system-between-primary-and-metastatic-colorectal-cancers-using-immunohistochemistry
#9
Jiyoon Jung, Youngjin Kang, Yoo Jin Lee, Eojin Kim, Bokyung Ahn, Eunjung Lee, Joo Young Kim, Jeong Hyeon Lee, Youngseok Lee, Chul Hwan Kim, Yang-Seok Chae
Background: Colorectal cancer (CRC) is one of the most common malignancies worldwide. Approximately 10%-15% of the CRC cases have defective DNA mismatch repair (MMR) genes. Although the high level of microsatellite instability status is a predictor of favorable outcome in primary CRC, little is known about its frequency and importance in secondary CRC. Immunohistochemical staining (IHC) for MMR proteins (e.g., MLH1, MSH2, MSH6, and PMS2) has emerged as a useful technique to complement polymerase chain reaction (PCR) analyses...
February 14, 2017: Journal of Pathology and Translational Medicine
https://www.readbyqxmd.com/read/28192254/effect-of-dietary-phosphorus-deficiency-on-the-growth-immune-function-and-structural-integrity-of-head-kidney-spleen-and-skin-in-young-grass-carp-ctenopharyngodon-idella
#10
Kang Chen, Wei-Dan Jiang, Pei Wu, Yang Liu, Sheng-Yao Kuang, Ling Tang, Wu-Neng Tang, Yong-An Zhang, Xiao-Qiu Zhou, Lin Feng
This study evaluates the effects of dietary phosphorus on the growth, immune function and structural integrity (head kidney, spleen and skin) of young grass carp (Ctenopharyngodon idella) that were fed graded levels of available phosphorus (0.95-8.75 g/kg diet). Results indicated that phosphorus deficiency decreased the growth performance of young grass carp. In addition, the results first demonstrated that compared with the optimal phosphorus level, phosphorus deficiency depressed the lysozyme (LZ) and acid phosphatase (ACP) activities and the complement 3 (C3), C4 and immunoglobulin M (IgM) contents, and down-regulated the mRNA levels of antimicrobial peptides, anti-inflammatory cytokines, inhibitor of κBα (IκBα) and target of rapamycin (TOR), whereas it up-regulated pro-inflammatory cytokines, nuclear factor kappa B (NF-κB) p65 and NF-κB p52 mRNA levels to decrease fish head kidney and spleen immune functions...
February 10, 2017: Fish & Shellfish Immunology
https://www.readbyqxmd.com/read/28192236/diagnosis-of-primary-antibody-and-complement-immunodeficiencies-in-young-adults-after-a-first-invasive-bacterial-infection
#11
Sébastien Sanges, Frédéric Wallet, Nicolas Blondiaux, Didier Theis, Isabelle Verin, Anne Vachée, Rodrigue Dessein, Karine Faure, Nathalie Viget, Eric Senneville, Olivier Leroy, Fleur Maury, Nicolas Just, Julien Poissy, Daniel Mathieu, Anne Prévotat, Cécile Chenivesse, Arnaud Scherpereel, Grégoire Smith, Benjamin Lopez, Jérémie Rosain, Véronique Fremeaux-Bacchi, Eric Hachulla, Pierre-Yves Hatron, Mathilde Bahuaud, Frédéric Batteux, David Launay, Myriam Labalette, Guillaume Lefèvre
OBJECTIVES: Screening for primary immunodeficiencies (PIDs) in adults is recommended after two severe bacterial infections. We aimed to evaluate if screening should be performed after the first invasive infection in young adults. METHODS: Eligible patients were retrospectively identified using hospital discharge and bacteriology databases in 3 centers during a 3-year period. Eighteen to forty year-old patients were included if they had experienced an invasive infection with encapsulated bacteria commonly encountered in PIDs (Streptococcus pneumoniae (SP), Neisseria meningitidis (NM), Neisseria gonorrhoeae (NG), Haemophilus influenzae (HI) or group A Streptococcus (GAS))...
February 10, 2017: Clinical Microbiology and Infection
https://www.readbyqxmd.com/read/28188176/lectin-pathway-effector-enzyme-mannan-binding-lectin-associated-serine-protease-2-can-activate-native-complement-c3-in-absence-of-c4-and-or-c2
#12
Sadam Yaseen, Gregory Demopulos, Thomas Dudler, Munehisa Yabuki, Christi L Wood, W Jason Cummings, Larry W Tjoelker, Teizo Fujita, Steven Sacks, Peter Garred, Peter Andrew, Robert B Sim, Peter J Lachmann, Russell Wallis, Nicholas Lynch, Wilhelm J Schwaeble
All 3 activation pathways of complement-the classic pathway (CP), the alternative pathway, and the lectin pathway (LP)- converge into a common central event: the cleavage and activation of the abundant third complement component, C3, via formation of C3-activating enzymes (C3 convertases). The fourth complement component, C4, and the second component, C2, are indispensable constituents of the C3 convertase complex, C4bC2a, which is formed by both the CP and the LP. Whereas in the absence of C4, CP can no longer activate C3, LP retains a residual but physiologically critical capacity to convert native C3 into its activation fragments, C3a and C3b...
February 10, 2017: FASEB Journal: Official Publication of the Federation of American Societies for Experimental Biology
https://www.readbyqxmd.com/read/28181435/selective-c-sp-2-h-halogenation-of-click-4-aryl-1-2-3-triazoles
#13
Asier Goitia, Enrique Gómez-Bengoa, Arkaitz Correa
Selective bromination reactions of "click compounds" are described. Electron-neutral and electron-deficient arenes selectively undergo unprecedented Pd-catalyzed C-H ortho-halogenations assisted by simple triazoles as modular directing groups, whereas electron-rich arenes are regioselectively halogenated following an electrophilic aromatic substitution reaction pathway. These C-H halogenation procedures exhibit a wide group tolerance, complement existing bromination procedures, and represent versatile synthetic tools of utmost importance for the late-stage diversification of "click compounds"...
February 9, 2017: Organic Letters
https://www.readbyqxmd.com/read/28181292/tumor-associated-ape1-variant-exhibits-reduced-complementation-efficiency-but-does-not-promote-cancer-cell-phenotypes
#14
Jennifer L Illuzzi, Daniel R McNeill, Paul Bastian, Boris Brenerman, Robert Wersto, Helen R Russell, Fred Bunz, Peter J McKinnon, Kevin G Becker, David M Wilson
Base excision repair (BER) is the major pathway for coping with most forms of endogenous DNA damage, and defects in the process have been associated with carcinogenesis. Apurinic/apyrimidinic endonuclease 1 (APE1) is a central participant in BER, functioning as a critical endonuclease in the processing of noncoding abasic sites in DNA. Evidence has suggested that APE1 missense mutants, as well as altered expression or localization of the protein, can contribute to disease manifestation. We report herein that the tumor-associated APE1 variant, R237C, shows reduced complementation efficiency of the methyl methanesulfonate hypersensitivity and impaired cell growth exhibited by APE1-deficient mouse embryonic fibroblasts...
February 9, 2017: Environmental and Molecular Mutagenesis
https://www.readbyqxmd.com/read/28177769/a-deletion-variant-partially-complements-a-porin-less-strain-of-i-neurospora-crassa-i
#15
Fraser G Ferens, Victor Spicer, Oleg V Krokhin, Anna Motnenko, William A T Summers, Deborah A Court
Mitochondrial porin, the voltage-dependent anion channel, plays an important role in metabolism and other cellular functions within eukaryotic cells. To further the understanding of porin structure and function, <i>Neurospora crassa</i> wild-type porin was replaced with a deletion variant lacking residues 238-242 (238porin). 238porin was assembled in the mitochondrial outer membrane, but the steady state levels were only about 3% of those of the wild-type protein. The strain harbouring 238porin displayed cytochrome deficiencies and expressed alternative oxidase...
November 2, 2016: Biochemistry and Cell Biology, Biochimie et Biologie Cellulaire
https://www.readbyqxmd.com/read/28174205/the-vdac2-bak-axis-regulates-peroxisomal-membrane-permeability
#16
Ken-Ichiro Hosoi, Non Miyata, Satoru Mukai, Satomi Furuki, Kanji Okumoto, Emily H Cheng, Yukio Fujiki
Peroxisomal biogenesis disorders (PBDs) are fatal genetic diseases consisting of 14 complementation groups (CGs). We previously isolated a peroxisome-deficient Chinese hamster ovary cell mutant, ZP114, which belongs to none of these CGs. Using a functional screening strategy, VDAC2 was identified as rescuing the peroxisomal deficiency of ZP114 where VDAC2 expression was not detected. Interestingly, knockdown of BAK or overexpression of the BAK inhibitors BCL-XL and MCL-1 restored peroxisomal biogenesis in ZP114 cells...
February 7, 2017: Journal of Cell Biology
https://www.readbyqxmd.com/read/28151980/lhx8-interacts-with-a-novel-germ-cell-specific-nuclear-factor-containing-an-nbl1-domain-in-rainbow-trout-oncorhynchus-mykiss
#17
Liyuan Fu, Prasanthi P Koganti, Jian Wang, Lei Wang, Cheng-Lun Wang, Jianbo Yao
Lhx8 is an important transcription factor that is preferentially expressed in germ cells. Lhx8 null mice are infertile due to lack of oocytes and impairment of the transition from primordial follicles to primary follicles. Lhx8 deficiency also affects the expression of many important oocyte-specific genes. In this study, we report the characterization of rainbow trout lhx8 genes and identification of a novel germ cell-specific nuclear factor that interacts with Lhx8. Two lhx8 genes, lhx8a and lhx8b, were identified, encoding proteins of 344 and 361 amino acids, respectively...
2017: PloS One
https://www.readbyqxmd.com/read/28147216/an-emerging-role-of-alu-rna-in-geographic-atrophy-pathogenesis-the-implication-for-novel-therapeutic-strategies
#18
Xiying Mao, Wangyi Fang, Qinghuai Liu
It is generally accepted that geographic atrophy (GA), a currently untreatable advanced form of age-related macular degeneration (AMD), is a multifactorial disease resulting in gradual and permanent blindness. Various risk factors are demonstrated to be responsible for its pathogenesis, such as aging, light exposure, and smoking. Molecular components associated with those risk factors form a complex and interwoven network at the confluence of inflammation, highlighting the significance of inflammasome activation in GA progression...
December 2016: Discovery Medicine
https://www.readbyqxmd.com/read/28142228/notch1-dependent-nitric-oxide-signaling-deficiency-in-hypoplastic-left-heart-syndrome-revealed-through-patient-specific-phenotypes-detected-in-bioengineered-cardiogenesis
#19
Sybil C L Hrstka, Xing Li, Timothy J Nelson
Hypoplastic left heart syndrome (HLHS) is a severe congenital heart defect (CHD) attributable to multifactorial molecular underpinnings. Multiple genetic loci have been implicated to increase the risk of disease, yet genotype-phenotype relationships remain poorly defined. Whole genome sequencing complemented by cardiac phenotype from five individuals in an HLHS-affected family enabled the identification of NOTCH1 as a prioritized candidate gene linked to CHD in three individuals with mutant allele burden significantly impairing Notch signaling in the HLHS-affected proband...
January 31, 2017: Stem Cells
https://www.readbyqxmd.com/read/28141495/mycobacterial-phenolic-glycolipid-synthesis-is-regulated-by-camp-dependent-lysine-acylation-of-fadd22
#20
Sintu Samanta, Albel Singh, Priyanka Biswas, Apoorva Bhatt, Sandhya Visweswariah
The mycobacterial cell envelope is unique in its chemical composition, and has an important role to play in pathogenesis. Phthiocerol dimycocerosates (PDIMs) and glycosylated phenolphthiocerol dimycocerosates, also known as phenolic glycolipids (PGLs), contribute significantly to the virulence of M. tuberculosis. FadD22 is essential for PGL biosynthesis. We have recently shown in vitro that FadD22 is a substrate for lysine acylation by a unique cAMP-dependent, protein lysine acyl transferase found only in mycobacteria...
January 28, 2017: Microbiology
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