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https://www.readbyqxmd.com/read/28426184/alterations-in-mandibular-morphology-associated-with-glypican-1-and-glypican-3-gene-mutations
#1
M Mian, S Ranjitkar, G C Townsend, P J Anderson
OBJECTIVES: Glypican 1 (GPC1) and glypican 3 (GPC3) are bone co-regulators that act downstream in many of the signalling pathways associated with craniosynostosis. Morphometric data from GPC-knockout mice were analysed to determine whether elimination of GPC1 and GPC3 genes would alter mandibular morphology. SETTING AND SAMPLE POPULATION: The murine model included five male and five female mandibles in each of GPC1-knockout, GPC1/GPC3-knockout and wild-type (control) groups...
April 20, 2017: Orthodontics & Craniofacial Research
https://www.readbyqxmd.com/read/28420236/human-hepatocarcinoma-cell-targeting-by-glypican-3-ligand-peptide-functionalized-silica-nanoparticles-implications-for-ultrasound-molecular-imaging
#2
Marco Di Paola, Alessandra Quarta, Francesco Conversano, Enzo Antonio Sbenaglia, Simona Bettini, Ludovico Valli, Giuseppe Gigli, Sergio Casciaro
Silica nanoparticles (SiNPs) are widely studied nanomaterials for their potential employment in advanced biomedical applications, like selective molecular imaging and targeted drug delivery. SiNPs are generally low cost and high biocompatible, can be easily functionalized with a wide variety of functional ligands and have been demonstrated to be effective in enhancing ultrasound contrast at clinical diagnostic frequencies. Therefore, SiNPs might be used as contrast agents in echographic imaging. In this work, we have developed a SiNPs-based system for the in vitro molecular imaging of hepatocellular carcinoma cells that express high levels of Glypican-3 protein (GPC-3) on their surface...
April 18, 2017: Langmuir: the ACS Journal of Surfaces and Colloids
https://www.readbyqxmd.com/read/28418018/neurodevelopmental-changes-in-excitatory-synaptic-structure-and-function-in-the-cerebral-cortex-of-sanfilippo-syndrome-iiia-mice
#3
Chrissa A Dwyer, Samantha L Scudder, Ying Lin, Lara E Dozier, Dustin Phan, Nicola J Allen, Gentry N Patrick, Jeffrey D Esko
Sanfilippo syndrome, MPS IIIA-D, results from deficits in lysosomal enzymes that specifically degrade heparan sulfate, a sulfated glycosaminoglycan. The accumulation of heparan sulfate results in neurological symptoms, culminating in extensive neurodegeneration and early death. To study the impact of storage in postnatal neurodevelopment, we examined murine models of MPS IIIA, which lack the enzyme sulfamidase. We show that changes occur in excitatory postsynaptic structure and function in the somatosensory cortex prior to signs of neurodegeneration...
April 18, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28402954/chaperone-mediated-autophagy-compensates-for-impaired-macroautophagy-in-the-cirrhotic-liver-to-promote-hepatocellular-carcinoma
#4
Srinivas Chava, Christine Lee, Yucel Aydin, Partha K Chandra, Asha Dash, Milad Chedid, Swan N Thung, Krzysztof Moroz, Tong Wu, Nabeen C Nayak, Srikanta Dash
Macroautophagy and chaperone-mediated autophagy (CMA) represent two major lysosomal degradation processes and often compensate for one another to facilitate cell survival. The aim of this study was to determine whether these autophagy pathways could compensate for one another to promote HCC cell survival in the cirrhotic liver. Analysis of normal liver tissue showed no expression of glypican-3 or p62 proteins, suggesting that macroautophagy is the major contributor to autophagic flux under non-pathological conditions...
March 29, 2017: Oncotarget
https://www.readbyqxmd.com/read/28393221/chromosome-13q-deletion-syndrome-involving-13q31%C3%A2-qter-a-case-report
#5
Yue-Ping Wang, Da-Jia Wang, Zhi-Bin Niu, Wan-Ting Cui
Partial deletions on the long arm of chromosome 13 lead to a number of different phenotypes depending on the size and position of the deleted region. The present study investigated 2 patients with 13q terminal (13qter) deletion syndrome, which manifested as anal atresia with rectoperineal fistula, complex type congenital heart disease, esophageal hiatus hernia with gastroesophageal reflux, facial anomalies and developmental and mental retardation. Array comparative genomic hybridization identified 2 regions of deletion on chromosome 13q31‑qter; 20...
April 3, 2017: Molecular Medicine Reports
https://www.readbyqxmd.com/read/28382841/hepatocellular-carcinoma-in-fanconi-bickel-syndrome
#6
Jennifer Pogoriler, Allison F O'Neill, Stephan D Voss, Robert C Shamberger, Antonio R Perez-Atayde
Fanconi-Bickel syndrome is a rare autosomal recessive disorder due to mutations in the facilitative glucose transporter 2 ( GLUT2 or SLC2A2) gene resulting in excessive glycogen storage predominantly in the liver and kidney. Previous case reports of this condition have described liver biopsies with glycogen storage and variable steatosis and/or fibrosis. Unlike in other types of glycogen storage disease, hepatocellular adenomas and carcinomas have not been described to date in this syndrome. A 6-year-old boy with consanguineous parents had short stature, poorly controlled rickets, hepatosplenomegaly, and renal tubular dysfunction clinically consistent with Fanconi-Bickel Syndrome...
January 1, 2017: Pediatric and Developmental Pathology
https://www.readbyqxmd.com/read/28378832/glypican-3-as-a-serum-marker-for-hepatoblastoma
#7
Shengmei Zhou, Maurice R G O'Gorman, Fusheng Yang, Kevin Andresen, Larry Wang
Hepatoblastoma (HB) is the most common primary liver cancer in children. The conventional serum marker for HB, alpha-fetoprotein (AFP), has its limitations. Novel serum markers need to be explored. Glypican 3 (GPC3) has been reported to be an excellent histological immunomarker for HB. However, the clinical value of serum GPC3 in patients with HB is unknown. A total of 184 serum samples were tested for both GPC3 by ELISA, and AFP by immunometric assay. Of these, 134 were from 32 patients with HB at three treatment stages, 30 from age-matched patients with benign hepatobiliary disorders (BHD) and 20 from age-matched "normal controls"(NC)...
April 5, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28371070/molecular-analysis-of-a-novel-intragenic-deletion-in-gpc3-in-three-cousins-with-simpson-golabi-behmel-syndrome
#8
Julia Schmidt, Ronja Hollstein, Frank J Kaiser, Gabriele Gillessen-Kaesbach
Simpson-Golabi-Behmel syndrome (SGBS) is characterized by multiple congenital abnormalities, pre/postnatal overgrowth, distinctive craniofacial features intellectual disability (ID) of variable degree, and an increased risk for embryonal tumors. SGBS is X-linked recessive and caused by deletions, duplications, and point mutations in GPC3, encoding a membrane associated cell surface heparan sulfate proteoglycan named glypican 3. GPC3 plays essential roles in the regulation of cell growth signaling and cell division...
March 29, 2017: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/28351894/long-term-load-duration-induces-n-cadherin-down-regulation-and-loss-of-cell-phenotype-of-nucleus-pulposus-cells-in-a-disc-bioreactor-culture
#9
Pei Li, Ruijie Zhang, Liyuan Wang, Yibo Gan, Yuan Xu, Lei Song, Lei Luo, Chen Zhao, Chengmin Zhang, Bin Ouyang, Bing Tu, Qiang Zhou
Long-term exposure to a mechanical load causes degenerative changes in the disc nucleus pulposus (NP) tissue. A previous study demonstrated that N-cadherin (N-CDH)-mediated signaling can preserve the NP cell phenotype. However, N-cadherin expression and the resulting phenotype alteration in NP cells under mechanical compression remain unclear. This study investigated the effects of the compressive duration on N-CDH expression and on the phenotype of NP cells in an ex-vivo disc organ culture. Porcine discs were organ-cultured in a self-developed mechanically active bioreactor for 7 days...
March 28, 2017: Bioscience Reports
https://www.readbyqxmd.com/read/28321273/characterization-of-a-new-monoclonal-anti-glypican-3-antibody-specific-to-the-hepatocellular-carcinoma-cell-line-hepg2
#10
Preeyanat Vongchan, Robert J Linhardt
AIM: To characterize the antigen on HepG2 cell that is specifically recognized by a new monoclonal antibody raised against human liver heparan sulfate proteoglycan (HSPG), clone 1E4-1D9. METHODS: The antigen recognized by mAb 1E4-1D9 was immunoprecipitated and its amino acid sequence was analyzed LC/MS. The transmembrane domain, number of cysteine residues, and glycosylation sites were predicted from these entire sequences. Data from amino acid analysis was aligned with glypican-3 (https://www...
March 8, 2017: World Journal of Hepatology
https://www.readbyqxmd.com/read/28315695/an-unusual-primary-malignant-tumor-of-the-stomach-fetal-gut-like-gastric-adenocarcinoma-with-blastoma-like-component
#11
Altaf Taher, Nebojsa Denic, Sangeetha N Kalimuthu, Runjan Chetty
An unusual case of a polypoid, malignant gastric tumor in a 62-year man is presented. Endoscopy and subsequent polypectomy revealed an 8.5 x 6.5 x 4.5cm lesion in the body of the stomach. Microscopy showed surface dysplasia with an invasive adenocarcinoma displaying prominent tubulopapillary areas composed of large vacuolated cells, pleomorphic nuclei and occasional cytoplasmic hyaline globules. This component then blended with tubular structures lined by more primitive appearing vacuolated cells embedded within a stroma made up of cellular primitive, high-grade blastema-like areas and, less cellular more pleomorphic foci with spindle and several bizarre, large cells...
March 15, 2017: Human Pathology
https://www.readbyqxmd.com/read/28300561/nucleolin-is-a-nuclear-target-of-heparan-sulfate-derived-from-glypican-1
#12
Fang Cheng, Mattias Belting, Lars-Åke Fransson, Katrin Mani
The recycling, S-nitrosylated heparan sulfate (HS) proteoglycan glypican-1 releases anhydromannose (anMan)-containing HS chains by a nitrosothiol-catalyzed cleavage in endosomes that can be constitutive or induced by ascorbate. The HS-anMan chains are then transported to the nucleus. A specific nuclear target for HS-anMan has not been identified. We have monitored endosome-to-nucleus trafficking of HS-anMan by deconvolution and confocal immunofluorescence microscopy using an anMan-specific monoclonal antibody in non-growing, ascorbate-treated, and growing, untreated, wild-type mouse embryonic fibroblasts and hypoxia-exposed Alzheimer mouse Tg2576 fibroblasts and human U87 glioblastoma cells...
March 12, 2017: Experimental Cell Research
https://www.readbyqxmd.com/read/28280721/tissue-biomarkers-in-hepatocellular-tumors-which-when-and-how
#13
REVIEW
Luca Di Tommaso, Massimo Roncalli
Few tissue markers are currently available to pathologists in the study of hepatocellular tumors. These markers should be used carefully taking into consideration not only morphology but also, and sometimes even more important, the clinical setting where the lesion to be diagnosed had developed. Glypican-3, heat shock protein 70, and glutamine synthetase (GS) are markers currently used, as a single panel, to discriminate the nature of a <2 cm hepatocellular lesion lacking radiological features of hepatocellular carcinoma (HCC) detected in a cirrhotic patient under surveillance...
2017: Frontiers in Medicine
https://www.readbyqxmd.com/read/28279206/mastalgia-as-an-atypical-presentation-of-hepatocellular-carcinoma-a-case-report
#14
AbdulAziz Mohammad Al-Sharydah, Abdulrhman Hamad Al-Abdulwahhab, Ibrahim Abobaker Alghnimi, Mohammed A El Shawarby, Faisal Ahmad Katbi
BACKGROUND: As the incidence of hepatocellular carcinoma (HCC) diagnoses in Saudi Arabia has recently increased due to better diagnostic techniques, the incidence of diagnosed HCC metastasis has also increased. Here, we report a case of HCC metastasis to the rib with an initially atypical presentation of mastalgia caused by extrahepatic metastasis. CASE PRESENTATION: A 31-year-old woman with a prior hepatitis B viral infection presented with a mass in the left breast accompanied by mastalgia for a 6-month duration...
March 9, 2017: World Journal of Surgical Oncology
https://www.readbyqxmd.com/read/28276470/simple-risk-score-for-prediction-of-early-recurrence-of-hepatocellular-carcinoma-within-the-milan-criteria-after-orthotopic-liver-transplantation
#15
Jiliang Feng, Jushan Wu, Ruidong Zhu, Dezhao Feng, Lu Yu, Yan Zhang, Dayu Bu, Chenlei Li, Yuyan Zhou, Lianghao Si, Yuhan Liu, Ziwei Liang, Jianing Xu, Tianjun Wu
Ten to twenty percent of the hepatocellular carcinoma (HCC) patients fulfilling the Milan criteria (MC) recurred within three years after orthotopic liver transplantation (OLT). We therefore utilize a training cohort to develop an improved prognostic model for predicting the recurrence in these patients. By univariate and multivariate analysis, AFP level [cut-off value: 321 ng/mL, area under the curve (AUC) = 0.724, 95% confidence interval (CI) = 0.604-0.843, P < 0.001] and cytokeratin-19 (CK19) and glypican-3 (GPC3) expression pattern from nine putative prognostic factors were entered in risk factor scoring model to conjecture the tumor recurrence...
March 9, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28266898/hepatic-malignancy-in-an-infant-with-wolf-hirschhorn-syndrome
#16
Sara Rutter, Raffaella A Morotti, Steven Peterec, Patrick G Gallagher
INTRODUCTION: Wolf-Hirschhorn syndrome (WHS) is a contiguous gene syndrome involving deletions of the chromosome 4p16 region associated with growth failure, characteristic craniofacial abnormalities, cardiac defects, and seizures. CASE REPORT: This report describes a six-month-old girl with WHS with growth failure and typical craniofacial features who died of complex congenital heart disease. Genetic studies revealed a 9.8 Mb chromosome 4p-terminal deletion. At autopsy, the liver was grossly unremarkable...
March 7, 2017: Fetal and Pediatric Pathology
https://www.readbyqxmd.com/read/28263753/the-maternal-plasma-proteome-changes-as-a-function-of-gestational-age-in-normal-pregnancy-a-longitudinal-study
#17
Roberto Romero, Offer Erez, Eli Maymon, Piya Chaemsaithong, Zhonghui Xu, Percy Pacora, Tinnakorn Chaiworapongsa, Bogdan Done, Sonia S Hassan, Adi L Tarca
OBJECTIVE: Pregnancy is accompanied by dramatic physiological changes in maternal plasma proteins. Characterization of the maternal plasma proteome in normal pregnancy is an essential step for understanding changes to predict pregnancy outcome. The objective of this study was to describe maternal plasma proteins that change in abundance with advancing gestational age and determine biological processes that are perturbed in normal pregnancy. STUDY DESIGN: A longitudinal study included 43 normal pregnancies that had a term delivery of an infant who was appropriate for gestational age without maternal or neonatal complications...
March 3, 2017: American Journal of Obstetrics and Gynecology
https://www.readbyqxmd.com/read/28240019/differential-expression-of-glypican-3-and-insulin%C3%A2-like-growth-factor-ii-mrnas-and-alpha-fetoprotein-and-ki-67-markers-in-hcv-related-hepatocellular-carcinomas-in-egyptian-patients
#18
Mohamed A Saber, Samah MM AbdelHafiz, Fatma E Khorshed, Tarek S Aboushousha, Hussam EM Hamdy, Mohamed I Seleem, Amira H Soliman
Background: Increasing evidence indicates that in hepatocellular carcinomas (HCCs) abnormal gene expression, for example of glypican-3 (GPC-3) and insulin-like growth factor-II (IGF-II), are associated with the occurrence and progression of HCC. The objective of this study was to evaluate the differential expression of GPC-3 and IGF-II mRNAs in HCC tissues with a background of chronic hepatitis C virus (HCV) genotype 4 cirrhosis, in relation to Ki-67 and alpha-feto protein (AFP) tissue markers. Methods: One hundred and five patients with HCCs who had undergone hepatectomy, were included, after obtaining informed consent...
January 1, 2017: Asian Pacific Journal of Cancer Prevention: APJCP
https://www.readbyqxmd.com/read/28211170/endothelial-glycocalyx-as-a-critical-signalling-platform-integrating-the-extracellular-haemodynamic-forces-and-chemical-signalling
#19
REVIEW
Ye Zeng
The glycocalyx covers the human mammalian cells and plays important roles in stroke, inflammation and atherosclerosis. It has also been shown to be involved in endothelial mechanotransduction of shear stress. Shear stress induces the remodelling of the major component of the glycocalyx including glypican-1, a cell membrane heparan sulphate proteoglycan. Other factors, such as sphingosine-1-phosphate (S1P), protect the glycocalyx against syndecan-1 ectodomain shedding and induce the synthesis of heparan sulphate...
February 17, 2017: Journal of Cellular and Molecular Medicine
https://www.readbyqxmd.com/read/28207681/significance-of-glypican-3-gpc3-expression-in-hepatocellular-cancer-diagnosis
#20
Bin Sun, Zhi Huang, Bi Wang, Yanglong Yu, Shihai Lin, Lei Luo, Yuzhu Wang, Zheng Huang
BACKGROUND Primary hepatocellular carcinoma (HCC) is a malignant tumor that is common China. Early diagnosis is of great significance for improving treatment efficiency. GPC3 level is closely related to HCC occurrence. This study investigated GPC3 expression in HCC patient serum and tissue, and assessed the significance of GPC3 combined AFP detection in HCC diagnosis. MATERIAL AND METHODS A total of 76 HCC patients in our hospital were enrolled. Immunohistochemistry was applied to test GPC3 expression in cancer tissue and para-carcinoma tissue...
February 16, 2017: Medical Science Monitor: International Medical Journal of Experimental and Clinical Research
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