bluuebunny/crossref_metadata_2025_split huggingface dataset:
Aldosterone-producing adenoma and other surgically correctable forms of primary aldosteronism
Authors: Amar Laurence , Plouin Pierre-François , Steichen Olivier 
Published: May 2010

Abstract
Surgically correctable forms of primary aldosteronism are characterized by unilateral aldosterone hypersecretion and renin suppression, associated with varying degrees of hypertension and hypokalemia. Unilateral aldosterone hypersecretion is caused by an aldosterone-producing adenoma (also known as Conn's adenoma and aldosteronoma), primary unilateral adrenal hyperplasia and rare cases of aldosterone-producing adrenocortical carcinoma. In these forms, unilateral adrenalectomy can cure aldosterone excess and hypokalemia, but not necessarily hypertension. The prevalence of primary aldosteronism in the general population is not known. Its prevalence in referred hypertensive populations is estimated to be between 6 and 13%, of which 1.5 to 5% have an aldosterone-producing adenoma or primary unilateral adrenal hyperplasia. Taking into account referral biases, the prevalence of surgically correctable primary aldosteronism is probably less than 1.5% in the hypertensive population and less than 0.3% in the general adult population. Surgically correctable primary aldosteronism is sought in patients with hypokalemic, severe or resistant forms of hypertension. Recent recommendations suggest screening for primary aldosteronism using the aldosterone to renin ratio. Patients with a raised ratio then undergo confirmatory suppression tests. The differential diagnosis of hypokalemic hypertension with low renin includes mineralocorticoid excess, with the mineralocorticoid being cortisol or 11-deoxycorticosterone, apparent mineralocorticoid excess, pseudo-hypermineralocorticoidism in Liddle syndrome or exposure to glycyrrhizic acid. Once the diagnosis is confirmed, adrenal computed tomography is performed for all patients. If surgery is considered, taking into consideration the clinical context and the desire of the patient, adrenal vein sampling is performed to detect whether or not aldosterone hypersecretion is unilateral. Laparoscopic surgery for unilateral aldosterone hypersecretion is associated with a morbidity of about 8%, with most complications being minor. It generally results in the normalization of aldosterone secretion and kalemia, and in a large decrease in blood pressure, but normotension without treatment is only achieved in half of all cases. Normotension following adrenalectomy is more frequent in young patients with recent hypertension than in patients with long-standing hypertension or a family history of hypertension.
Kidney function of HIV‐infected children in Lagos, Nigeria: using Filler's serum cystatin C‐based formula
Authors: Esezobor Christopher I , Iroha Edna , Oladipo Olajumoke , Onifade Elizabeth , Soriyan Oyetunji O , Akinsulie Adebola O , Temiye Edamisan O , Ezeaka Chinyere 
Published: May 2010

BackgroundLimited data is available on kidney function in HIV‐infected children in sub‐Saharan Africa. In addition, malnutrition in these children further reduces the utility of diagnostic methods such as creatinine‐based estimates of glomerular filtration rate. We determined the serum cystatin C level and estimated glomerular filtration rate of 60 antiretroviral‐naïve, HIV‐infected children and 60 apparently healthy age and sex matched children.MethodsSerum cystatin C level was measured using enzyme‐linked immunosorbent assay technique, while glomerular filtration rate was estimated using Filler's serum cystatin C formula. Student t test, Mann Whitney U test, Pearson chi square and Fisher's exact test were used, where appropriate, to test difference between groups.ResultsCompared to the controls, the HIV‐infected group had significantly higher median (interquartile range) serum cystatin C levels {0.77 (0.29) mg/l versus 0.66 (0.20) mg/l; p = 0.025} and a higher proportion of children with serum cystatin C level >1 mg/l {10 (16.7%) versus one (1.7%); p = 0.004}. The HIV‐infected children had a mean (± SD) eGFR of 96.8 (± 36.1) ml/min/1.73 m2 compared with 110.5 (± 27.8) ml/min/1.73 m2 in the controls (p = 0.021). After controlling for age, sex and body mass index, only the study group (HIV infected versus control) remained a significant predictor of serum cystatin C level (β = ‐0.216, p = 0.021). The proportion of HIV‐infected children with eGFR <60 ml/min/1.73 m2 was eight (13.3%) versus none (0%) in the control group (p = 0.006). However, the serum cystatin C level, eGFR and proportions of children with serum cystatin C level >1 mg/l and eGFR <60 ml/min/1.73 m2 were not significantly different between the HIV‐infected children with advanced disease and those with milder disease.ConclusionsHIV‐infected children in Nigeria have higher serum cystatin C level and lower eGFR compared to age and sex matched controls.
Hepatic failure, neonatal hemochromatosis and porto-pulmonary hypertension in a newborn with trisomy 21 - a case report
Authors: Neil Erin , Cortez Josef , Joshi Aparna , Bawle Erawati V , Poulik Janet , Zilberman Mark , El-Baba Mohammad F , Sood Beena G 
Published: May 2010

AbstractLiver failure in neonates is a rare but often fatal disease. Trisomy 21 is not usually associated with significant infantile liver disease. If present, hepatic dysfunction in an infant with Trisomy 21 is likely to be attributed to transient myeloproliferative disorder with hepatic infiltration by hematopoietic elements and may be associated with secondary hemosiderosis. A less commonly recognized cause of liver failure in neonates with Trisomy 21 is neonatal hemochromatosis (NH); this association has been reported in nine cases of Trisomy 21 in literature. NH is a rare, severe liver disease of intra-uterine onset that is characterized by neonatal liver failure and hepatic and extrahepatic iron accumulation that spares the reticuloendothelial system. NH is the most frequently recognized cause of liver failure in neonates and the commonest indication for neonatal liver transplantation. Although porto-pulmonary hypertension (PPH) has been reported as a complication of liver failure in adults and older children, this has not been reported in neonates with liver failure of any etiology. This is probably due to the rarity of liver failure in newborns, delayed diagnosis and high mortality. The importance of recognizing PPH is that it is reversible with liver transplantation but at the same time increases the risk of post-operative mortality. Therefore, early diagnosis of PPH is critical so that early intervention can improve the chances of successful liver transplantation. We report for the first time the association of liver failure with porto-pulmonary hypertension secondary to NH in an infant with Trisomy 21.
Increased inflammation and impaired resistance to Chlamydophila pneumoniae infection in Dusp1-/- mice: critical role of IL-6
Authors: Rodriguez Nuria , Dietrich Harald , Mossbrugger Ilona , Weintz Gabriele , Scheller Jürgen , Hammer Michael , Quintanilla-Martinez Leticia , Rose-John Stefan , Miethke Thomas , Lang Roland 
Published: May 2010

Abstract
Dendritic cells interact with T cells in intestinal mucosa in an MHCII-dependent manner, suggesting presentation outside organized lymphoid tissue may be important in determining T cell phenotype.
The MAPK phosphatase DUSP1 is an essential negative regulator of TLR-triggered innate immune activation. Here, we have investigated the impact of DUSP1 on inflammatory and antimicrobial host responses to the intracellular pathogen Chlamydophila pneumoniae. Following nasal infection, DUSP1-deficient mice mounted an enhanced pulmonary cytokine (IL-1β, IL-6) and chemokine response (CCL3, CCL4, CXCL1, CXCL2), leading to increased leukocyte infiltration. Of interest, the increased inflammatory response, in the absence of DUSP1, was associated with higher bacterial numbers in the lungs, although the expression of IFN-γ and critical antichlamydial effector molecules, such as iNOS, was intact. Blockade of IL-6 trans-signaling by injection of a soluble gp130-Fc fusion protein corrected the overshooting chemokine production as well as the increased chlamydial load in Dusp1−/− mice. Furthermore, IL-6 enhanced the replication of C. pneumoniae in embryonic fibroblasts in vitro. These data show that DUSP1 is required to achieve a balanced response to chlamydial infection and identify IL-6 as critical for amplifying inflammation and benefiting chlamydial growth through direct effects on infected cells.
The bone marrow: a site of neutrophil clearance
Authors: Rankin Sara M 
Published: May 2010

Abstract
Review discusses evidence that the bone marrow is an important site of neutrophil clearance under homeostatic conditions.
Abstract
Neutrophils, an essential component of the innate immune system, are produced at a rate 1011 cells/day and are consequently the most abundant leukocyte in the blood. These granulocytes have a relatively short half-life (6–8 h) in the blood, and as a result of the cytotoxic nature of their contents, it is critical that there are mechanisms in place for the efficient clearance of these effete cells from the circulation. Although macrophages of the reticuloendothelial system are positioned ideally to recognize and phagocytose apoptotic neutrophils in the circulation, this review challenges the current dogma that neutrophils are cleared primarily in the liver and spleen, presenting the evidence that the bone marrow is also an important site of neutrophil clearance under homeostatic conditions. Furthermore, a role for the CXCL12/CXCR4 chemokine axis in the trafficking of senescent neutrophils back to the bone marrow for clearance is described.
CCR1- and CCR5-mediated inactivation of leukocytes by a nonglycosaminoglycan (non-GAG)-binding variant of n-Nonanoyl-CCL14 (NNY-CCL14)
Authors: Gupta Shipra , Rieder Sebastian , Richter Rudolf , Schulz-Maronde Sandra , Manns Johanna , Escher Sylvia E , Heitland Aleksandra , Mack Matthias , Forssmann Wolf-Georg , Elsner Jörn , Forssmann Ulf 
Published: May 2010

Abstract
The non-glycosaminoglycan binding NNY-CCL14(G,A) is a potential lead for an anti-inflammatory therapy exerting its effects via multiple chemokine receptors.
Intervention on chemokine receptors to prevent directional leukocyte migration is a potential therapeutic strategy. NNY-CCL14 is a CD26-resistant lead molecule, which exerts its effects on multiple chemokine receptors (CCR1, CCR2, CCR3, and CCR5). The inhibitory effects of NNY-CCL14 in murine models of allergic airway inflammation have been assigned to its interaction with CCR1 and CCR5. In this study, a non-GAG-binding variant of NNY-CCL14 was generated by mutating basic amino acids within the identified GAG-binding 49BBXB52 motif. This CD26-resistant, non-GAG binding variant, NNY-CCL14(G,A), does not promote CCR1-dependent cell arrest on modeled endothelium. Its biological activity tested on human and murine chemokine receptors revealed distinguishing properties to NNY-CCL14. As suggested by EC50 values for intracellular calcium mobilization, NNY-CCL14(G,A) demonstrated a reduced ability to activate hCCR1, but internalization and desensitization of hCCR1 were unperturbed. Surprisingly, its activity on hCCR3 was strongly reduced, and it did not internalize mCCR3. A significantly reduced chemotactic activity of eosinophils and monocytes was observed. All biological effects mediated by NNY-CCL14(G,A) via hCCR5 and mCCR5 showed no difference to NNY-CCL14. In mice treated i.v. with NNY-CCL14(G,A), a sustained in vivo down-modulation of CCR5 was achieved over 3 h. Therefore, NNY-CCL14(G,A) inactivates leukocytes by desensitizing and internalizing multiple chemokine receptors, thus rendering them unresponsive to further stimulation by natural ligands. When administered systemically, NNY-CCL14(G,A) may modulate leukocyte functions prior to their interaction with other endothelium-bound chemokines expressed under pathophysiological conditions, such as allergic inflammation.
The mitochondrial import gene tomm22 is specifically required for hepatocyte survival and provides a liver regeneration model
Authors: Curado Silvia , Ober Elke A. , Walsh Susan , Cortes-Hernandez Paulina , Verkade Heather , Koehler Carla M. , Stainier Didier Y. R. 
Published: May 2010

SUMMARY
Understanding liver development should lead to greater insights into liver diseases and improve therapeutic strategies. In a forward genetic screen for genes regulating liver development in zebrafish, we identified a mutant – oliver – that exhibits liver-specific defects. In oliver mutants, the liver is specified, bile ducts form and hepatocytes differentiate. However, the hepatocytes die shortly after their differentiation, and thus the resulting mutant liver consists mainly of biliary tissue. We identified a mutation in the gene encoding translocase of the outer mitochondrial membrane 22 (Tomm22) as responsible for this phenotype. Mutations in tomm genes have been associated with mitochondrial dysfunction, but most studies on the effect of defective mitochondrial protein translocation have been carried out in cultured cells or unicellular organisms. Therefore, the tomm22 mutant represents an important vertebrate genetic model to study mitochondrial biology and hepatic mitochondrial diseases. We further found that the temporary knockdown of Tomm22 levels by morpholino antisense oligonucleotides causes a specific hepatocyte degeneration phenotype that is reversible: new hepatocytes repopulate the liver as Tomm22 recovers to wild-type levels. The specificity and reversibility of hepatocyte ablation after temporary knockdown of Tomm22 provides an additional model to study liver regeneration, under conditions where most hepatocytes have died. We used this regeneration model to analyze the signaling commonalities between hepatocyte development and regeneration.
Ras controls melanocyte expansion during zebrafish fin stripe regeneration
Authors: Lee Yoonsung , Nachtrab Gregory , Klinsawat Pai W. , Hami Danyal , Poss Kenneth D. 
Published: May 2010

SUMMARYRegenerative medicine for complex tissues like limbs will require the provision or activation of precursors for different cell types, in the correct number, and with the appropriate instructions. These strategies can be guided by what is learned from spectacular events of natural limb or fin regeneration in urodele amphibians and teleost fish. Following zebrafish fin amputation, melanocyte stripes faithfully regenerate in tandem with complex fin structures. Distinct populations of melanocyte precursors emerge and differentiate to pigment regenerating fins, yet the regulation of their proliferation and patterning is incompletely understood. Here, we found that transgenic increases in active Ras dose-dependently hyperpigmented regenerating zebrafish fins. Lineage tracing and marker analysis indicated that increases in active Ras stimulated the in situ amplification of undifferentiated melanocyte precursors expressing mitfa and kita. Active Ras also hyperpigmented early fin regenerates of kita mutants, which are normally devoid of primary regeneration melanocytes, suppressing defects in precursor function and survival. By contrast, this protocol had no noticeable impact on pigmentation by secondary regulatory melanocyte precursors in late-stage kita regenerates. Our results provide evidence that Ras activity levels control the repopulation and expansion of adult melanocyte precursors after tissue loss, enabling the recovery of patterned melanocyte stripes during zebrafish appendage regeneration.
J-Aggregation of Zinc 31,131-Dihydroxychlorins by Exclusive Coordination Bonding between 31-Hydroxy Group and Central Zinc Atom
Authors: Kunieda Michio , Tamiaki Hitoshi 
Published: May 2010

Abstract
Zinc 31,131-dihydroxychlorin lacking an exo-five-membered E-ring was prepared as a model of natural bacteriochlorophyll-d possessing 31-OH and 13-C=O groups. The synthetic zinc complex has the 13-CH2OH instead of the 13-C=O and self-aggregated in a nonpolar organic solvent, giving slipped and π-overlapped molecular stackings (J-aggregates) with red-shifted and broadened electronic absorption bands. For the intermolecular interactions among the J-aggregates, coordination bonding of the 31-OH (not 131-OH) with the central Zn was utilized.
Optical Resolution of Chiral Buckybowls by Chiral HPLC
Authors: Tsuruoka Ryoji , Higashibayashi Shuhei , Ishikawa Takeharu , Toyota Shinji , Sakurai Hidehiro 
Published: May 2010

Abstract
Optical resolution of chiral buckybowls, C3 symmetric trimethylsumanene derivatives, was achieved by chiral HPLC. Enantiomeric excess (ee) of enatioselectively prepared trimethyltris(trimethylsilyl)sumanene was determined by the chiral HPLC analysis. The enantiomers of racemic trimethylsumanenetrione were separated, and the bowl inversion energy barrier was determined by the CD spectra.
Silver Oxide as a Novel Catalyst for Carbon–Carbon Bond-forming Reactions in Aqueous Media
Authors: Ueno Masaharu , Tanoue Arata , Kobayashi Shu 
Published: May 2010

Abstract
Silver oxide was found to be an excellent catalyst for allylation reactions of allyltributyltins with aldehydes in aqueous media. Despite the very low solubility of silver oxide in the media, the reactions proceeded smoothly, and the catalyst was recovered and reused. When α-methyl-substituted allyltributyltin was used as the nucleophile, the corresponding α-adducts were obtained exclusively in high yields with good anti-selectivity. A reaction mechanism including transmetalation from tin to silver is proposed.
The rhodanese RhdA helps Azotobacter vinelandii in maintaining cellular redox balance
Authors: Remelli William , Cereda Angelo , Papenbrock Jutta , Forlani Fabio , Pagani Silvia 
Published: May 2010

AbstractThe tandem domain rhodanese-homology protein RhdA ofAzotobacter vinelandiishows an active-site loop structure that confers structural peculiarity in the environment of its catalytic cysteine residue. Thein vivoeffects of the lack of RhdA were investigated using anA. vinelandiimutant strain (MV474) in which therhdAgene was disrupted by deletion. Here, by combining analytical measurements and transcript profiles, we show that deletion of therhdAgene generates an oxidative stress condition to whichA. vinelandiiresponds by activating defensive mechanisms. In conditions of growth in the presence of the superoxide generator phenazine methosulfate, a stressor-dependent induction ofrhdAgene expression was observed, thus highlighting that RhdA is important forA. vinelandiito sustain oxidative stress. The potential of RhdA to buffer general levels of oxidants inA. vinelandiicells via redox reactions involving its cysteine thiol is discussed.
Blunting the knife: development of vaccines targeting digestive proteases of blood-feeding helminth parasites
Authors: Pearson Mark S. , Ranjit Najju , Loukas Alex 
Published: May 2010

AbstractProteases are pivotal to parasitism, mediating biological processes crucial to worm survival including larval migration through tissue, immune evasion/modulation and nutrient acquisition by the adult parasite. In haematophagous parasites, many of these proteolytic enzymes are secreted from the intestine (nematodes) or gastrodermis (trematodes) where they act to degrade host haemoglobin and serum proteins as part of the feeding process. These proteases are exposed to components of the immune system of the host when the worms ingest blood, and therefore present targets for the development of anti-helminth vaccines. The protective effects of current vaccine antigens against nematodes that infect humans (hookworm) and livestock (barber's pole worm) are based on haemoglobin-degrading intestinal proteases and act largely as a result of the neutralisation of these proteases by antibodies that are ingested with the blood-meal. In this review, we survey the current status of helminth proteases that show promise as vaccines and describe their vital contribution to a parasitic existence.
Detection of breast cancer-related antigens through cDNA phage-displayed protein microarray
Authors: Bi Qun , Tan Taochao , Xiang Xi , Lu Aiping , Zhu Shenggeng 
Published: May 2010

AbstractHigh-throughput molecular profiling techniques are helpful in the diagnosis of multifactorial disease. In this study, a cDNA-phage-displayed protein microarray using phage particles spotted directly onto it as sensors was used to detect related antigens in breast tumor sera. cDNA sequences from 17 positive clones were determined, which included some sequences encoding known breast cancer-related antigens and proteins related to other diseases, as well as proteins with unknown functions. Our results not only provide some useful information for breast cancer research, but also suggest that the strategy used here would be efficient to search for disease-related proteins and other functional target proteins.
The XPD helicase: XPanDing archaeal XPD structures to get a grip on human DNA repair
Authors: Wolski Stefanie C. , Kuper Jochen , Kisker Caroline 
Published: May 2010

Abstract
Xeroderma pigmentosum complementation group D protein (XPD) is an iron-sulfur cluster containing 5′-3′ helicase and, in humans, part of the transcription factor TFIIH. TFIIH is involved in nucleotide excision repair as well as in transcription initiation. Recently, three different groups have reported the structures of archaeal XPDs. All structures revealed a four-domain organization with two RecA-like domains, an Arch domain and an iron-sulfur cluster domain. It was possible to rationalize several of the mutations in the human XPD gene that lead to one of the three severe diseases xeroderma pigmentosum, Cockayne syndrome and trichothiodystrophy. The different structures are compared and disease-related mutations are discussed.
The membrane-bound bile acid receptor TGR5 (Gpbar-1) is localized in the primary cilium of cholangiocytes
Authors: Keitel Verena , Ullmer Christoph , Häussinger Dieter 
Published: May 2010

Abstract
Cholangiocyte cilia are sensory organelles that extend from the apical membrane into the bile duct lumen and detect changes in bile flow and osmolarity. Whether or not cholangiocyte cilia are responsive to bile acids is unknown. TGR5 (Gpbar-1) is a membrane-bound bile acid receptor which is expressed in biliary epithelial cells and promotes chloride secretion in gallbladder epithelial cells. As shown in the present study, TGR5 is localized in the primary cilium of mouse and human cholangiocytes. Here the receptor could play an important role in coupling biliary bile acid concentration and composition to ductular bile formation.
Proteolysis of platelet receptors in humans and other species
Authors: Qiao Jian L. , Shen Yang , Gardiner Elizabeth E. , Andrews Robert K. 
Published: May 2010

AbstractIn the past 5 years, metalloproteinase-mediated ectodomain shedding of platelet receptors has emerged as a new mechanism for modulating platelet function. By regulating surface expression of the platelet-specific receptors, glycoprotein (GP)VI that binds collagen, and GPIbα (the major ligand-binding subunit of the GPIb-IX-V complex) that binds von Willebrand factor (VWF) and other procoagulant and proinflammatory ligands, shedding not only irreversibly downregulates GPVI/GPIbα function, but generates proteolytic fragments that might be unique biomarkers or modulators in plasma. This is potentially significant because GPVI and GPIbα are involved in initiating thrombotic diseases such as heart attack and stroke, as well as autoimmune diseases where anti-platelet antibodies result in thrombocytopenia. Altered expression levels of GPIbα/GPVI are associated with both thrombotic propensity and platelet aging, suggesting an additional role in platelet clearance. Although emerging data are elucidating molecular mechanisms underlying GPIbα/GPVI shedding, evidence for the functional consequences of sheddingin vivo, either clinically or in animal models, is far more limited. Here we consider recent published evidence for GPVI or GPIbα shedding in humans, nonhuman primates and mice, and whether conservation of sheddase cleavage sites across species points to a functional role for metalloproteolytic sheddingin vivo.