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[PMID]: 29511689
[Au] Autor:Park KH; Chung WH; Kwon H; Lee JM
[Ad] Address:Department of Otorhinolaryngology-Head and Neck Surgery, Soonchunhyang University College of Medicine, Cheonan, Republic of Korea.
[Ti] Title:Evaluation of Cerebral White Matter in Prelingually Deaf Children Using Diffusion Tensor Imaging.
[So] Source:Biomed Res Int;2018:6795397, 2018.
[Is] ISSN:2314-6141
[Cp] Country of publication:United States
[La] Language:eng
[Ab] Abstract:This study compared white matter development in prelingually deaf and normal-hearing children using a tract-based spatial statistics (TBSS) method. Diffusion tensor imaging (DTI) was performed in 21 prelingually deaf (DEAF group) and 20 normal-hearing (HEAR group) subjects aged from 1.7 to 7.7 years. Using TBSS, we evaluated the regions of significant difference in fractional anisotropy (FA) between the groups. Correlations between FA values and age in each group were also analyzed using voxel-wise correlation analyses on the TBSS skeleton. Lower FA values of the white matter tract of Heschl's gyrus, the inferior frontooccipital fasciculus, the uncinate fasciculus, the superior longitudinal fasciculus, and the forceps major were evident in the DEAF group compared with those in the HEAR group below 4 years of age, while the difference was not significant in older subjects. We also found that age-related development of the white matter tracts may continue until 8 years of age in deaf children. These results imply that development of the cerebral white matter tracts is delayed in prelingually deaf children.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1803
[Cu] Class update date: 180311
[Lr] Last revision date:180311
[St] Status:In-Process
[do] DOI:10.1155/2018/6795397

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[PMID]: 29360619
[Au] Autor:Whyte MP; Coburn SP; Ryan LM; Ericson KL; Zhang F
[Ad] Address:Center for Metabolic Bone Disease and Molecular Research, Shriners Hospital for Children, St. Louis, MO 63110, USA; Division of Bone and Mineral Diseases, Department of Internal Medicine, Washington University School of Medicine at Barnes-Jewish Hospital, St. Louis, MO 63110, USA. Electronic address
[Ti] Title:Hypophosphatasia: Biochemical hallmarks validate the expanded pediatric clinical nosology.
[So] Source:Bone;110:96-106, 2018 Jan 31.
[Is] ISSN:1873-2763
[Cp] Country of publication:United States
[La] Language:eng
[Ab] Abstract:Hypophosphatasia (HPP) is the inborn-error-of-metabolism due to loss-of-function mutation(s) of the ALPL (TNSALP) gene that encodes the tissue non-specific isoenzyme of alkaline phosphatase (TNSALP). TNSALP represents a family of cell-surface phosphohydrolases differing by post-translational modification that is expressed especially in the skeleton, liver, kidney, and developing teeth. Thus, the natural substrates of TNSALP accumulate extracellularly in HPP including inorganic pyrophosphate (PPi), a potent inhibitor of mineralization, and pyridoxal 5'-phosphate (PLP), the principal circulating form of vitamin B . The superabundance of extracellular PPi regularly causes tooth loss, and when sufficiently great can lead to rickets or osteomalacia. Sometimes diminished hydrolysis of PLP engenders vitamin B -dependent seizures in profoundly affected babies. Autosomal dominant and autosomal recessive inheritance from among >340 ALPL mutations identified to date, typically missense and located throughout the gene, largely explains the remarkably wide-ranging severity of HPP, greatest of all skeletal diseases. In 2015, our demographic, clinical, and DXA findings acquired over 25 years from 173 children and adolescents with HPP validated and expanded the clinical nosology for pediatric patients to include according to increasing severity "odonto" HPP, "mild childhood" HPP, "severe childhood" HPP, "infantile" HPP, and "perinatal" HPP. Herein, we assessed this expanded nosology using biochemical hallmarks of HPP. We evaluated exclusively data from the 165 preteenage HPP patients in this cohort to exclude potential effects from physiological changes in TNSALP levels across puberty. All patients had subnormal serum total and bone-specific ALP and elevated plasma PLP, and nearly all had excessive urinary PPi excretion. Only the PLP levels were unchanged across puberty. Mean levels of all four biomarkers correlated with HPP severity ranked according to the HPP nosology, but the data overlapped among all four patient groups. Hence, these four biochemical hallmarks represent both a sensitive and reliable tool for diagnosing children with HPP. Furthermore, the hallmarks validate our expanded clinical nosology for pediatric HPP that, with limitations, is an improved framework for conceptualizing and working with this disorder's remarkably broad-ranging severity.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1801
[Cu] Class update date: 180310
[Lr] Last revision date:180310
[St] Status:Publisher

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[PMID]: 29523871
[Au] Autor:Guo S; Zhang Y; Zhou T; Wang D; Weng Y; Chen Q; Ma J; Li YP; Wang L
[Ad] Address:Jiangsu Key Laboratory of Oral Diseases, Nanjing Medical University, 140 Hanzhong Road, Nanjing, 210029, China.
[Ti] Title:GATA4 as a novel regulator involved in the development of the neural crest and craniofacial skeleton via Barx1.
[So] Source:Cell Death Differ;, 2018 Mar 09.
[Is] ISSN:1476-5403
[Cp] Country of publication:England
[La] Language:eng
[Ab] Abstract:The role of GATA-binding protein 4 (GATA4) in neural crest cells (NCCs) is poorly defined. Here we showed that mouse NCCs lacking GATA4 exhibited developmental defects in craniofacial bone, teeth, and heart. The defects likely occurred due to decreased cell proliferation at the developmental stage. The in vitro results were consistent with the mouse model. The isobaric tags for relative and absolute quantitation assay revealed that BARX1 is one of the differentially expressed proteins after GATA4 knockdown in NCCs. On the basis of the results of dual-luciferase, electro-mobility shift, and chromatin immunoprecipitation assays, Barx1 expression is directly regulated by GATA4 in NCCs. In zebrafish, gata4 knockdown affects the development of NCCs derivatives. However, the phenotype in zebrafish could be partly rescued by co-injection of gata4 morpholino oligomers and barx1 mRNA. This study identified new downstream targets of GATA4 in NCCs and uncovered additional evidence of the complex regulatory functions of GATA4 in NCC development.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1803
[Cu] Class update date: 180310
[Lr] Last revision date:180310
[St] Status:Publisher
[do] DOI:10.1038/s41418-018-0083-x

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Caldas, Eloisa Dutra
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[PMID]: 29522798
[Au] Autor:da Motta LG; de Morais JA; Tavares ACAM; Vianna LMS; Mortari MR; Amorim RFB; Carvalho RR; Paumgartten FJR; Pic-Taylor A; Caldas ED
[Ad] Address:Laboratory of Toxicology, Faculty of Health Sciences, University of Brasilia, Brasilia, DF, Brazil.
[Ti] Title:Maternal and developmental toxicity of the hallucinogenic plant-based beverage ayahuasca in rats.
[So] Source:Reprod Toxicol;, 2018 Mar 06.
[Is] ISSN:1873-1708
[Cp] Country of publication:United States
[La] Language:eng
[Ab] Abstract:Rats were treated orally with ayahuasca (AYA) on gestation days (GD) 6-20 at doses corresponding to one-(1X) to eight-fold (8X) the average dose taken by a human adult in a religious ritual, and the pregnancy outcome evaluated on GD21. Rats treated with 4X and 8X doses died during the treatment period (44 and 52%), and those that survived showed kidney injury. Rats surviving the 8X dose showed neuronal loss in hippocampal regions and in the raphe nuclei, and those from the 2X dose neuronal loss in CA1. Delayed intrauterine growth, induced embryo deaths and increased occurrence of foetal anomalies were observed at the 8X dose. At non-lethal doses, AYA enhanced embryolethality and the incidence of foetal soft-tissue and skeleton anomalies. This study suggested that AYA is developmentally toxic and that its daily use by pregnant women may pose risks for the conceptus.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1803
[Cu] Class update date: 180309
[Lr] Last revision date:180309
[St] Status:Publisher

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[PMID]: 29522466
[Au] Autor:Jin J; Yang X; Liu T; Xiao H; Wang G; Zhou M; Liu F; Zhang Y; Liu D; Chen M; Cheng W; Yang D; Ma M
[Ad] Address:State Key Laboratory of Natural and Biomimetic Drugs, Department of Natural Medicines, School of Pharmaceutical Sciences, Peking University, 38 Xueyuan Road, Haidian District, Beijing 100191, China. jinjing@bjmu.edu.cn.
[Ti] Title:Fluostatins M-Q Featuring a 6-5-6-6 Ring Skeleton and High Oxidized A-Rings from Marine Streptomyces sp. PKU-MA00045.
[So] Source:Mar Drugs;16(3), 2018 Mar 09.
[Is] ISSN:1660-3397
[Cp] Country of publication:Switzerland
[La] Language:eng
[Ab] Abstract:Aromatic polyketides from marine actinomycetes have received increasing attention due to their unusual structures and potent bioactivities. Compared to their terrestrial counterparts, marine aromatic polyketides have been less discovered and their structural and biological diversities are far from being fully investigated. In this study, we employed a PCR-based genome mining method to discover aromatic polyketides in our marine bacteria collection. Five new atypical angucyclinones, fluostatins M-Q ( - ) featuring a unique 6-5-6-6 ring skeleton, were discovered from one "positive" sp. PKU-MA00045. The structures of fluostatins M-Q ( - ) were elucidated based on comprehensive spectroscopic analyses and the crystallographic structure of fluostatin P ( ), which contains the most oxidized A-ring, was solved by X-ray diffraction analysis with Cu Kα radiation. Compared to the published 16 fluostatin analogues, fluostatins M-Q ( - ) contained a different methoxy group attached at C-7 and hydroxy group attached at C-4, enriching the structural diversity of aromatic polyketides from marine actinomycetes. Genome sequencing of sp. PKU-MA00045 revealed the biosynthetic gene cluster of fluostatins M-Q ( - ), which contained different genes and gene organizations compared to known fluostatin gene clusters, facilitating the investigation of the biosynthesis of the unique 6-5-6-6 ring skeleton in all fluostatins.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1803
[Cu] Class update date: 180309
[Lr] Last revision date:180309
[St] Status:In-Process

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[PMID]: 29522365
[Au] Autor:Kusunoki M; Kohama T; Yamada Y; Fujita E; Okada S; Maeda A; Takeshima N
[Ad] Address:a Faculty Biology Oriented Science and Technology , Kindai University , Wakayama , Japan.
[Ti] Title:Evaluating activities of daily living using an infrared depth sensor: KINECT .
[So] Source:Disabil Rehabil Assist Technol;:1-11, 2018 Mar 09.
[Is] ISSN:1748-3115
[Cp] Country of publication:England
[La] Language:eng
[Ab] Abstract:PURPOSE: With a growing proportion of elderly people in the population, the maintenance of activities of daily living (ADLs) in elderly people is crucial to keep medical costs down. We investigated the ADL measurement accuracy of KINECT and Kinect Studio. To eliminate the subjectivity of conventional methods, we numerically assessed motions with computer analysis. METHODS: Eighteen actions that repeated "move" and "stationary" phases, including movement of arms, legs, head and torso were measured using KINECT . Errors and standard deviations of joint coordinates at the stationary points outputted from KINECT Studio were evaluated. Simultaneous measurements were performed with KINECT using conventional high-performance motion capture, and the output was treated as a true value for comparison. RESULTS: In most motions, errors of the joint coordinates were within 100 mm; however, there were two cases where errors due to the skeleton-model estimation by KINECT Studio increased. Firstly, when a part of the body unexpectedly moved out of the infrared measurable area, and secondly, when parts of the body overlapped each other on the KINECT image. CONCLUSIONS: KINECT and Kinect Studio are effective for ADL assessment when positions that cause large errors are excluded. Since KINECT has sufficient precision, it should also be possible to develop a more appropriate ADL evaluation system with a new algorithm of skeleton-model estimation that does not depend on KINECT Studio. Implications for Rehabilitation The KINECT and Kinect Studio are effective for ADL assessment when positions that cause large errors are excluded With an increasing proportion of elderly people in the population, the maintenance of activities of daily living (ADLs) in elderly people is crucial to keep medical costs down Systems such as the KINECT can support these goals.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1803
[Cu] Class update date: 180309
[Lr] Last revision date:180309
[St] Status:Publisher
[do] DOI:10.1080/17483107.2018.1449020

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[PMID]: 29268132
[Au] Autor:Xu XJ; Wang F; Zeng T; Lin J; Liu J; Chang YQ; Sun PH; Chen WM
[Ad] Address:College of Pharmacy, Jinan University, Guangzhou, 510632, PR China.
[Ti] Title:4-arylamidobenzyl substituted 5-bromomethylene-2(5H)-furanones for chronic bacterial infection.
[So] Source:Eur J Med Chem;144:164-178, 2018 Jan 20.
[Is] ISSN:1768-3254
[Cp] Country of publication:France
[La] Language:eng
[Ab] Abstract:Bacterial quorum-sensing (QS) can cause bacterial biofilm formation, thus induce antibiotic resistance and inflammation in chronic bacterial infections. A series of novel 4-arylamidobenzyl substituted 5-bromomethylene-2(5H)-furanones were designed by introducing of brominated furanones into rosiglitazone skeleton, and their potential application in the treatment of chronic bacterial infection was evaluated with regard to their disruption of quorum sensing and anti-inflammatory activities in vitro as well as in animal infection model. Compound 2e displayed both potent QS inhibitory activity and anti-inflammatory activity. Further mechanism studies revealed that the biological effects of 2e and 2k could be attributed, at least in part, to their interaction with PPARγ, and consequent suppression of the activation of NF-κB and MAPK cascades. Importantly, pretreatment with 2e significantly protects mice from lethal-dose LPS challenge. Thus, these data suggest that the dual effective derivative 2e may serve as a valuable candidate for the treatment of chronic bacterial infection.
[Mh] MeSH terms primary: 4-Butyrolactone/analogs & derivatives
Anti-Bacterial Agents/chemistry
Anti-Bacterial Agents/pharmacology
Anti-Inflammatory Agents/chemistry
Anti-Inflammatory Agents/pharmacology
Pseudomonas aeruginosa/drug effects
[Mh] MeSH terms secundary: 4-Butyrolactone/chemistry
4-Butyrolactone/pharmacology
Animals
Anti-Bacterial Agents/therapeutic use
Anti-Inflammatory Agents/therapeutic use
Chronic Disease
Humans
Lipopolysaccharides/immunology
Male
Mice
Molecular Docking Simulation
NF-kappa B/immunology
Nitric Oxide/immunology
PPAR gamma/immunology
Pseudomonas Infections/drug therapy
Pseudomonas Infections/immunology
Pseudomonas Infections/microbiology
Pseudomonas aeruginosa/immunology
Pseudomonas aeruginosa/physiology
Quorum Sensing/drug effects
RAW 264.7 Cells
[Pt] Publication type:JOURNAL ARTICLE
[Nm] Name of substance:0 (5-bromomethylene-2(5H)-furanone); 0 (Anti-Bacterial Agents); 0 (Anti-Inflammatory Agents); 0 (Lipopolysaccharides); 0 (NF-kappa B); 0 (PPAR gamma); 31C4KY9ESH (Nitric Oxide); OL659KIY4X (4-Butyrolactone)
[Em] Entry month:1803
[Cu] Class update date: 180309
[Lr] Last revision date:180309
[Js] Journal subset:IM
[Da] Date of entry for processing:171222
[St] Status:MEDLINE

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[PMID]: 29193820
[Au] Autor:Steyn C; Soley JT; Crole MR
[Ad] Address:Department of Anatomy and Physiology, Faculty of Veterinary Science, University of Pretoria, Onderstepoort, South Africa.
[Ti] Title:Osteology and Radiological Anatomy of the Thoracic Limbs of Temminck's Ground Pangolin (Smutsia temminckii).
[So] Source:Anat Rec (Hoboken);301(4):624-635, 2018 Apr.
[Is] ISSN:1932-8494
[Cp] Country of publication:United States
[La] Language:eng
[Ab] Abstract:Temminck's ground pangolin is the only pangolin present in South Africa. It is a myrmecophagous mammal with a bipedal gait. The thoracic limbs are used to break open ant nests, dig for food, and expand previously occupied burrows. This study describes the osteology and radiological anatomy of the thoracic limbs of this threatened species. Thoracic limbs from four Temminck's ground pangolins, which succumbed from electrocution or natural causes, were digitally radiographed in situ. The individual bones were then cleaned, described and digitally radiographed. The skeleton of the thoracic limbs revealed a similar number and arrangement of bones compared to that of domestic carnivores. The bones were robust and displayed numerous open epiphyseal lines. The latter provide an estimate of sexual maturity and should not be confused with fractures in injured ground pangolins. The scapula was broad and triangular-shaped. The humerus displayed a massive medial epicondyle. The radius and ulna were similarly sized, and displayed a broad radial trochlea and large olecranon tuber, respectively. The manus was composed of seven carpal bones, five short metacarpal bones and five digits of which the three central digits were the best developed. The unguicular process of the distal phalanx was bifid and elongated. The osteological characteristics indicate that the thoracic limbs of Temminck's ground pangolin are specifically adapted for protraction and retraction, strong elbow extension, flexion of the carpus and digits as well as pronation and supination of the antebrachium, as opposed to weight-bearing. These functions are likewise documented for other scratch-digging species. Anat Rec, 301:624-635, 2018. © 2017 Wiley Periodicals, Inc.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1712
[Cu] Class update date: 180310
[Lr] Last revision date:180310
[St] Status:In-Data-Review
[do] DOI:10.1002/ar.23733

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[PMID]: 29522281
[Au] Autor:Tamplen M; Fowler T; Markey J; Knott PD; Suva LJ; Alliston T
[Ad] Address:Department of Otolaryngology - Head and Neck Surgery, University of California, San Francisco, California.
[Ti] Title:Treatment with anti-Sclerostin antibody to stimulate mandibular bone formation.
[So] Source:Head Neck;, 2018 Mar 09.
[Is] ISSN:1097-0347
[Cp] Country of publication:United States
[La] Language:eng
[Ab] Abstract:BACKGROUND: Anti-Sclerostin antibody (Scl-Ab) is a promising new bone anabolic therapy. Although anti-Scl-Ab stimulates bone formation and repair in the appendicular and axial skeleton, its efficacy in the craniofacial skeleton is still poorly understood. METHODS: Using an established model of Down syndrome-dependent bone deficiency, 10 Ts65Dn mice and 10 wild-type mice were treated weekly via i.v. tail vein injection with vehicle or anti-Sclerostin for 3 weeks and euthanized 1 week after. RESULTS: Wild-type mice treated with the anti-Scl-Ab had increased mandibular bone, trabecular thickness, and alveolar height compared with controls. Anti-Scl-Ab increased Ts65Dn mandibular bone parameters such that they were statistically indistinguishable from those in vehicle-treated wild-type mandibles. CONCLUSION: Treatment with anti-Scl-Ab significantly increased mandibular bone mass and alveolar height in wild type mice and normalized mandibular bone mass and alveolar height in Ts65Dn mice. The anti-Scl-Ab therapy represents a novel method for increasing mandibular bone formation.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1803
[Cu] Class update date: 180309
[Lr] Last revision date:180309
[St] Status:Publisher
[do] DOI:10.1002/hed.25128

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[PMID]: 29522194
[Au] Autor:Roser-Page S; Vikulina T; Yu K; McGee-Lawrence ME; Weitzmann MN
[Ad] Address:Atlanta VA Medical Center, Decatur, GA, USA.
[Ti] Title:Neutralization of CD40 ligand costimulation promotes bone formation and accretion of vertebral bone mass in mice.
[So] Source:Rheumatology (Oxford);, 2018 Mar 07.
[Is] ISSN:1462-0332
[Cp] Country of publication:England
[La] Language:eng
[Ab] Abstract:Objective: Immunosuppressive biologics are used in the management of RA and additional immunomodulators are under investigation including modulators of the CD40/CD40 ligand (CD40L) costimulation pathway. Tampering with immune function can have unanticipated skeletal consequences due to disruption of the immuno-skeletal interface, a nexus of shared cells and cytokine effectors serving discrete functions in both immune and skeletal systems. In this study, we examined the effect of MR1, a CD40L neutralizing antibody, on physiological bone remodelling in healthy mice. Methods: Female C57BL6 mice were treated with MR1 and BMD was quantified by dual energy X-ray absorptiometry and indices of trabecular bone structure were quantified by micro-CT. Serum biochemical markers were used to evaluate bone turnover and formation indices by histomorphometry. Results: Unexpectedly, MR1 stimulated significant accretion of BMD and trabecular bone mass in the spine, but not in long bones. Surprisingly, bone accretion was accompanied by a significant increase in bone formation, rather than suppression of bone resorption. Mechanistically, MR1-induced bone accrual was associated with increased Treg development and elevated production of cytotoxic T lymphocyte antigen 4, a costimulation inhibitor that promotes T cell anergy and CD8+ T cell expression of the bone anabolic ligand Wnt-10b. Conclusion: Our studies reveal an unexpected bone anabolic activity of pharmacological CD40L suppression. Therapeutic targeting of the CD40L pathway may indeed have unforeseen consequences for the skeleton, but may also constitute a novel strategy to promote bone formation to ameliorate osteoporotic bone loss and reduce fracture risk in the axial skeleton.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1803
[Cu] Class update date: 180309
[Lr] Last revision date:180309
[St] Status:Publisher
[do] DOI:10.1093/rheumatology/kex525


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