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https://www.readbyqxmd.com/read/29166577/cell-cycle-related-kinase-regulates-mammalian-eye-development-through-positive-and-negative-regulation-of-the-hedgehog-pathway
#1
Floria I Lupu, Jacob B Burnett, Jonathan T Eggenschwiler
Cell cycle-related kinase (CCRK) is a conserved regulator of ciliogenesis whose loss in mice leads to a wide range of developmental defects, including exencephaly, preaxial polydactyly, skeletal abnormalities, and microphthalmia. Here, we investigate the role of CCRK in mouse eye development. Ccrk mutants show dramatic patterning defects, with an expansion of the optic stalk domain into the optic cup, as well as an expansion of the retinal pigment epithelium (RPE) into neural retina (NR) territory. In addition, Ccrk mutants display a shortened optic stalk...
November 19, 2017: Developmental Biology
https://www.readbyqxmd.com/read/29164817/regulation-of-hedgehog-signaling-in-cancer-by-natural-and-dietary-compounds
#2
REVIEW
Cheng Bao, Pavel Kramata, Hong Jin Lee, Nanjoo Suh
The aberrant Hedgehog (Hh) signaling induced by mutations or overexpression of the signaling mediators has been implicated in cancer, associated with processes including inflammation, tumor cell growth, invasion and metastasis, as well as cancer stemness. Small molecules targeting the regulatory components of the Hh signaling pathway, especially Smoothened (Smo), have been developed for the treatment of cancer. However, acquired resistance to a Smo inhibitor vismodegib observed in clinical trials suggests that other Hh signaling components need to be explored as potential anticancer targets...
November 21, 2017: Molecular Nutrition & Food Research
https://www.readbyqxmd.com/read/29163520/the-injury-related-activation-of-hedgehog-signaling-pathway-modulates-the-repair-associated-inflammation-in-liver-fibrosis
#3
REVIEW
Xin Shen, Yu Peng, Hanmin Li
Liver fibrosis is a wound healing response initiated by inflammation responding for different iterative parenchymal damages caused by diverse etiologies. Immune cells, which exert their ability of either inducing injury or promoting repair, have been regarded as crucial participants in the fibrogenic response. A characteristic feature of the fibrotic microenvironment associated with chronic liver injury is aberrant activation of hedgehog (Hh) signaling pathway. Growing evidence from a number of different studies in vivo and in vitro has indicated that immune-mediated events involved in liver fibrogenesis are regulated by Hh signaling pathway...
2017: Frontiers in Immunology
https://www.readbyqxmd.com/read/29159599/delayed-gastric-emptying-following-laparoscopic-repair-of-very-large-hiatus-hernias-impairs-quality-of-life
#4
David S Liu, Chek Tog, Hou K Lim, Peter Stiven, Sarah K Thompson, David I Watson, Ahmad Aly
BACKGROUND: Delayed gastric emptying (DGE) following hiatus hernia surgery may affect a substantial number of patients with adverse clinical consequences. Here, we aim to evaluate the impact of DGE following laparoscopic repair of very large hiatus hernias on patients' quality of life, gastrointestinal symptomatology, and daily function. METHODS: Analysis of data collected from a multicenter prospective randomised trial of patients who underwent laparoscopic mesh versus sutured repair of very large hiatus hernias (>50% of stomach in chest)...
November 20, 2017: World Journal of Surgery
https://www.readbyqxmd.com/read/29158915/parafoveolar-retinoblastoma-regression-with-foveal-preservation-following-intra-arterial-chemotherapy-documented-on-hand-held-optical-coherence-tomography-in-a-newborn
#5
Vera Yarovaya, Kareem Sioufi, Carol L Shields
Background: Optical coherence tomography (OCT) has become an invaluable tool in retinoblastoma management, providing submillimeter visualization of tumor control following treatment. Herein, we document OCT-detection of a subtle tumor recurrence, allowing early intervention and achieving foveal microanatomy preservation. Case presentation: A 3-week-old girl was diagnosed with bilateral familial retinoblastoma, classified as group D in the right eye (OD) and group B in the left eye (OS), and treated with intravenous chemoreduction...
2017: International Journal of Retina and Vitreous
https://www.readbyqxmd.com/read/29158396/emt-programs-promote-basal-mammary-stem-cell-and-tumor-initiating-cell-stemness-by-inducing-primary-ciliogenesis-and-hedgehog-signaling
#6
Vincent J Guen, Tony E Chavarria, Cornelia Kröger, Xin Ye, Robert A Weinberg, Jacqueline A Lees
Tissue regeneration relies on adult stem cells (SCs) that possess the ability to self-renew and produce differentiating progeny. In an analogous manner, the development of certain carcinomas depends on a small subset of tumor cells, called "tumor-initiating cells" (TICs), with SC-like properties. Mammary SCs (MaSCs) reside in the basal compartment of the mammary epithelium, and their neoplastic counterparts, mammary TICs (MaTICs), are thought to serve as the TICs for the claudin-low subtype of breast cancer...
November 20, 2017: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/29156747/lethality-of-inappropriate-plasma-exposure-on-chicken-embryonic-development
#7
Jiao Jiao Zhang, Jin Oh Jo, Do Luong Huynh, Mrinmoy Ghosh, Nameun Kim, Sang Baek Lee, Hak Kyo Lee, Young Sun Mok, Taeho Kwon, Dong Kee Jeong
In this study, we examined the effects of non-thermal dielectric barrier discharge plasma on embryonic development in chicken eggs in order to determine the optimal level of plasma exposure for the promotion of embryonic growth. We exposed developing chicken embryos at either Hamburger-Hamilton (HH) stage 04 or HH 20 to plasma at voltages of 11.7 kV to 27.6 kV. Our results show exposure at 11.7 kV for 1 min promoted chicken embryonic development, but exposure to more duration and intensity of plasma resulted in dose-dependent embryonic death and HH 20 stage embryos survive longer than those at stage HH 04...
October 17, 2017: Oncotarget
https://www.readbyqxmd.com/read/29152039/hepatic-hydrothorax-an-update-and-review-of-the-literature
#8
REVIEW
Dmitry Victorovich Garbuzenko, Nikolay Olegovich Arefyev
This review considers the modern concepts of pathogenesis, diagnostic methods, and treatment principles of hepatic hydrothorax (HH). HH is the excessive (> 500 mL) accumulation of transudate in the pleural cavity in patients with decompensated liver cirrhosis but without cardiopulmonary and pleural diseases. It causes respiratory failure which aggravates the clinical course of liver cirrhosis, and the emergence of spontaneous bacterial pleural empyema may be the cause of death. The information was collected from the PubMed database, the Google Scholar retrieval system, the Cochrane reviews, and the reference lists from relevant publications for 1994-2016 using the keywords: "liver cirrhosis", "portal hypertension", "hepatic hydrothorax", "pathogenesis", "diagnostics", and "treatment"...
November 8, 2017: World Journal of Hepatology
https://www.readbyqxmd.com/read/29151614/i-xe-systematics-of-the-impact-plume-produced-chondrules-from-the-cb-carbonaceous-chondrites-implications-for-the-half-life-value-of-129-i-and-absolute-age-normalization-of-129-i-129-xe-chronometer
#9
O Pravdivtseva, A Meshik, C M Hohenberg, A N Krot
It is inferred that magnesian non-porphyritic chondrules in the CB (Bencubbin-type) carbonaceous chondrites formed in an impact generated plume of gas and melt at 4562.49 ± 0.21 Ma (Bollard et al., 2015) and could be suitable for the absolute age normalization of relative chronometers. Here xenon isotopic compositions of neutron irradiated chondrules from the CB chondrites Gujba and Hammadah al Hamra (HH) 237 have been analyzed in an attempt to determine closure time of their I-Xe isotope systematics. One of the HH 237 chondrules, #1, yielded a well-defined I-Xe isochron that corresponds to a closure time of 0...
March 15, 2017: Geochimica et Cosmochimica Acta
https://www.readbyqxmd.com/read/29150303/oxytocin-intranasal-administration-as-a-new-hope-for-hypogonadotropic-hypogonadism-patients
#10
Mohammad Saied Salehi, Sareh Pandamooz, Homayoun Khazali
Hypogonadotropic hypogonadism (HH) is a form of hypogonadism which also known as secondary or central hypogonadism. Congenital HH can occur due to defect in gonadotropin releasing hormone (GnRH) neurons, upstream regulators of GnRH neurons or pituitary gonadotropic cells. Testosterone or gonadotropins therapy are widely used to treat HH patients, however both have undesirable effects and GnRH treatment for HH patients is time and cost consuming. Direct delivery of therapeutics to the brain via the nasal route is located in the center of attention during the last decade and trial application of intranasal oxytocin as a method of enhancing social interactions are reported...
November 2017: Medical Hypotheses
https://www.readbyqxmd.com/read/29144824/17%C3%AE-hydroxylase-deficiency-is-an-underdiagnosed-disease-high-frequency-of-misdiagnoses-in-a-large-cohort-of-brazilian-patients
#11
Rafaela Fontenele, Marivânia Costa-Santos, Claudio E Kater
OBJECTIVE: 17α-Hydroxylase deficiency (P450c17D) is characterized by hypogonadism and mineralocorticoid hypertension. We aimed to estimate the relative incidence and spectrum of preliminary misdiagnoses in Brazilian P450c17D patients. DESIGN: Cross-sectional study. METHODS: We reviewed, updated, and analyzed data of 40 P450c17D patients (21 XY, 19 XX). RESULTS: Complete data were unavailable for two patients. Seven patients were relatives of an index case...
November 16, 2017: Endocrine Practice
https://www.readbyqxmd.com/read/29143041/joint-experimental-and-theoretical-studies-of-the-surprising-stability-of-the-aryl-pentazole-upon-noncovalent-binding-to-%C3%AE-cyclodextrin
#12
Yu-Zhang Yang, Xiao-Feng Liu, Ru-Bo Zhang, Si-Ping Pang
Herein, binding of the β-cyclodextrin (β-CD) host to the unstable aryl pentazole guest has been confirmed experimentally and theoretically. After the confinement of aryl pentazole, electron density reorganization was studied by M06-2X dispersion-corrected DFT and further reflected in the characteristic shift in the NMR spectra. Among the host-guest complexes, the inclusion complex is favored with the phenyl ring expectedly encapsulated within the cavity through noncovalent interactions such as hydrogen bonding, C-Hπ, and the special Csp(2)-HH-Csp(3) bonding discovered by the NBO, QTAIM, and NCI-RDG theories...
November 16, 2017: Physical Chemistry Chemical Physics: PCCP
https://www.readbyqxmd.com/read/29142600/co-activation-of-hedgehog-and-wnt-signaling-pathways-is-associated-with-poor-outcomes-in-triple-negative-breast-cancer
#13
Kimberly M Arnold, Ryan T Pohlig, Jennifer Sims-Mourtada
Hedgehog (HH) and Wnt pathway activation have been implicated in poor prognosis of breast cancer. Crosstalk between these two pathways has been demonstrated to be important in breast cancer progression, however the association between these two pathways and breast cancer survival rate is unknown. The present study comprised a cohort of 36 patients with triple negative breast cancer (TNBC) to investigate co-activation of HH and canonical Wnt pathway in association to patient outcome. All patients had varying degrees of cytoplasmic sonic HH and glioma-associated oncogene homolog (Gli)-1 staining, which positively correlated with tumor stage...
November 2017: Oncology Letters
https://www.readbyqxmd.com/read/29142103/an-intracellular-activation-of-smoothened-independent-of-hedgehog-stimulation-in-drosophila
#14
Kai Jiang, Yajuan Liu, Jie Zhang, Jianhang Jia
Smoothened (Smo), a GPCR family protein, plays a critical role in the reception and transduction of Hedgehog (Hh) signal. Smo is phosphorylated and activated on the cell surface, however, it is unknown whether Smo can be intracellularly activated. Here, we demonstrate that inactivation of the ESCRT-III causes dramatic accumulation of Smo, and subsequent activation of Hh signaling. In contrast, inactivation of ESCRTs 0-II induces mild Smo accumulation. We provide evidence that Kurtz (Krz), the Drosophila β-arrestin2, acts in parallel with the ESCRTs 0-II pathway to sort Smo to the multivesicular bodies and lysosome-mediated degradation...
November 15, 2017: Journal of Cell Science
https://www.readbyqxmd.com/read/29141375/-relationship-between-the-severity-of-reflux-esophagitis-and-the-esophageal-motility-features-on-high-resolution-manometry
#15
Z W Hu, Z G Wang, J M Wu, S R Tian, Y Zhang, X L Zhan, X Du, F Wang, R H Xin, H Xu
Objective: To analyze esophageal motility dysfunction in gastroesophageal reflux disease (GERD) with different severity of esophagitis, and the relationship between the esophageal motility dysfunction and the severity of esophagitis. Methods: GERD patients simultaneously undergone endoscopy and high-resolution manometry were divided into four groups: Non-esophagitis (Non-erosive gastroesophageal reflux disease, NERD) group, mild esophagitis group, moderate esophagitis group and severe esophagitis group. The gender and age were matched for each group, and every group consisted of 80 cases...
November 14, 2017: Zhonghua Yi Xue za Zhi [Chinese medical journal]
https://www.readbyqxmd.com/read/29139542/temporal-and-spatial-expression-patterns-of-hedgehog-receptors-in-the-developing-inner-and-middle-ear
#16
Jeong-Oh Shin, Harinarayana Ankamreddy, Naga Mahesh Jakka, Seokwon Lee, Un-Kyung Kim, Jinwoong Bok
The mammalian inner ear is a complex organ responsible for balance and hearing. Sonic hedgehog (Shh), a member of the Hedgehog (Hh) family of secreted proteins, has been shown to play important roles in several aspects of inner ear development, including dorsoventral axial specification, cochlear elongation, tonotopic patterning, and hair cell differentiation. Hh proteins initiate a downstream signaling cascade by binding to the Patched 1 (Ptch1) receptor. Recent studies have revealed that other types of co-receptors can also mediate Hh signaling, including growth arrest-specific 1 (Gas1), cell-adhesion molecules-related/down-regulated by oncogenes (Cdon), and biregional Cdon binding protein (Boc)...
2017: International Journal of Developmental Biology
https://www.readbyqxmd.com/read/29139540/neurosphere-formation-potential-resides-not-in-the-caudal-cell-mass-but-in-the-secondary-neural-tube
#17
Ji Yeoun Lee, Eun Sun Lee, Saet Pyoul Kim, Myung-Sook Lee, Ji Hoon Phi, Seung-Ki Kim, Young-Il Hwang, Kyu-Chang Wang
The caudal cell mass (CCM) is known as the main player in secondary neurulation, forming the secondary neural tube (2NT). This suggests that the CCM may have the character of neural progenitor cells. The neural potential of the CCM and the 2NT (CCM + 2NT) was assessed by in vitro culture of neurospheres during Hamburger and Hamilton stages (HH) of secondary neurulation (HH16 to HH32). We also analyzed the neural potential of the developing central nervous system (CNS) by comparing the neurosphere culture from the brain, upper / lower spinal cord, and CCM + 2NT from various HH stages...
2017: International Journal of Developmental Biology
https://www.readbyqxmd.com/read/29138370/-development-of-holistic-cancer-treatment-centering-cancer-patients-from-the-standpoint-of-hypoxia-and-hedgehog-signaling
#18
Hideya Onishi, Toshitatsu Ogino, Takashi Morisaki, Mitsuo Katano
Recently, hypoxia that is one of cancer microenvironments, takes much attention. Because circumstance that we usually perform experiment is 20% O2 condition, it is likely that different signaling pathways may be activated in vivo cancer. We focused Hedgehog(Hh)signaling as one of activated pathways under hypoxia. It has been shown that Hh signaling is activated under hypoxia, followed by inducing malignant phenotypes in pancreatic cancer. Therefore, Hh signaling inhibitor should elicit anti-tumor effect. However, if we consider "whole-person therapy" we should confirm how Hh signaling affects the function of immune cells...
November 2017: Gan to Kagaku Ryoho. Cancer & Chemotherapy
https://www.readbyqxmd.com/read/29136303/chromosome-9-mutations-reported-absent-in-some-patients-with-basal-cell-carcinoma-nevus-syndrome
#19
S Shih, B A Urso, R Domozych, K M Updyke, A I Laughlin, J A Solomon
Basal Cell Carcinoma Nevus Syndrome (BCCNS), also known as Gorlin syndrome, is a rare autosomal dominant condition with a substantial disease burden, thought to occur secondary to chromosome 9 mutation evoking a Hedgehog (Hh) signaling aberration that leads to early development of numerous basal cell carcinomas (BCCs). Individuals with BCCNS also present with keratocystic odontogenic tumors, medulloblastomas, and palmar or plantar pits1. This condition demonstrates variable expressivity, leading to differing phenotypes, even within the same family2...
November 14, 2017: Journal of the European Academy of Dermatology and Venereology: JEADV
https://www.readbyqxmd.com/read/29134618/the-mechanisms-of-systemic-iron-homeostasis-and-etiology-diagnosis-and-treatment-of-hereditary-hemochromatosis
#20
REVIEW
Hiroshi Kawabata
Hereditary hemochromatosis (HH) is a group of genetic iron overload disorders that manifest with various symptoms, including hepatic dysfunction, diabetes, and cardiomyopathy. Classic HH type 1, which is common in Caucasians, is caused by bi-allelic mutations of HFE. Severe types of HH are caused by either bi-allelic mutations of HFE2 that encodes hemojuvelin (type 2A) or HAMP that encodes hepcidin (type 2B). HH type 3, which is of intermediate severity, is caused by bi-allelic mutations of TFR2 that encodes transferrin receptor 2...
November 13, 2017: International Journal of Hematology
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