The CLN6 gene (ceroid-lipofuscinosis, neuronal 6, late infantile, variant) is located on the long arm of chromosome 15 at position 23 (15q23). It provides instructions for making a protein that likely regulates the transport of certain proteins and fats from the endoplasmic reticulum to lysosomes. Based on this function, the CLN6 protein appears to help in the process of ridding cells of materials they no longer need. It is unclear how mutations in the CLN6 gene are involved in the buildup of lipopigments and the features of Kufs disease. Research suggests that CLN6 gene mutations that cause Kufs disease allow enough functional protein to be produced so that signs and symptoms of the disorder do not develop until adulthood. The presence of other proteins that compensate for the lack of CLN6 protein could also play a role in the appearance of symptoms in adulthood.. CLN6 gene; Sep 2013. Kufs disease, the major adult form of neuronal ceroid lipofuscinosis, caused by mutations in CLN6; May ...

PubMed Central Canada (PMC Canada) provides free access to a stable and permanent online digital archive of full-text, peer-reviewed health and life sciences research publications. It builds on PubMed Central (PMC), the U.S. National Institutes of Health (NIH) free digital archive of biomedical and life sciences journal literature and is a member of the broader PMC International (PMCI) network of e-repositories.

TY - JOUR. T1 - Paneth cell marker expression in intestinal villi and colon crypts characterizes dietary induced risk for mouse sporadic intestinal cancer. AU - Wang, Donghai. AU - Peregrina, Karina. AU - Dhima, Elena. AU - Lin, Elaine Y.. AU - Mariadason, John M.. AU - Augenlicht, Leonard H.. PY - 2011/6/21. Y1 - 2011/6/21. N2 - Nutritional and genetic risk factors for intestinal tumors are additive on mouse tumor phenotype, establishing that diet and genetic factors impact risk by distinct combinatorial mechanisms. In a mouse model of dietary-induced sporadic small and large intestinal cancer in WT mice in which tumor etiology, lag, incidence, and frequency reflect ,90% of intestinal cancer in Western societies, dietary-induced risk altered gene expression profiles predominantly in villus cells of the histologically normal mucosa, in contrast to targeting of crypt cells by inheritance of an Apc 1638N allele or homozygous inactivation of p21 Waf1/cip1, and profiles induced by each risk factor ...

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Adult-onset neuronal ceroid lipofuscinosis, also known as Kufs disease, is a neurodegenerative disorder without retinal involvement. There are 2 overlapping phenotypes: type A, characterized by progressive myoclonic epilepsy, and type B, characterized by dementia and a variety of motor-system signs (summary by {1:Arsov et al., 2011}). In general, the neuronal ceroid lipofuscinoses (NCL; CLN) are a clinically and genetically heterogeneous group of neurodegenerative disorders characterized by the intracellular accumulation of autofluorescent lipopigment storage material in different patterns ultrastructurally. The clinical course includes progressive dementia, seizures, and progressive visual failure ({8:Mole et al., 2005}). The ultrastructural pattern of lipopigment in CLN4 comprises a mixed pattern of granular, curvilinear, and fingerprint profiles. ({8:Mole et al., 2005}). For a general phenotypic description and a discussion of genetic heterogeneity of CLN, see CLN1 ({256730 ...

Adult-onset neuronal ceroid lipofuscinosis, also known as Kufs disease, is a neurodegenerative disorder without retinal involvement. There are 2 overlapping phenotypes: type A, characterized by progressive myoclonic epilepsy, and type B, characterized by dementia and a variety of motor-system signs (summary by {1:Arsov et al., 2011}). In general, the neuronal ceroid lipofuscinoses (NCL; CLN) are a clinically and genetically heterogeneous group of neurodegenerative disorders characterized by the intracellular accumulation of autofluorescent lipopigment storage material in different patterns ultrastructurally. The clinical course includes progressive dementia, seizures, and progressive visual failure ({8:Mole et al., 2005}). The ultrastructural pattern of lipopigment in CLN4 comprises a mixed pattern of granular, curvilinear, and fingerprint profiles. ({8:Mole et al., 2005}). For a general phenotypic description and a discussion of genetic heterogeneity of CLN, see CLN1 ({256730 ...

Targeting EMT with a small-molecule inhibitor may be important in therapeutics, since it is increasingly being proven that EMT plays an integral role in cell migration, invasion, and metastasis (10, 11). Cat K and Cat S inhibitors have been tried in clinical trials, with many side effects and little progress (12, 13). However, no Cat L inhibitor has been evaluated in clinical trials. Our study was a preclinical mechanistic study to evaluate the feasibility of using the Cat L small-molecule inhibitor Z-FY-CHO in prostate and breast cancer cells that display EMT and are thus more aggressive. In this study, we showed that mesenchymal cells with high Snail expression displayed increased nuclear Cat L expression as well as increased expression of nuclear p110 and p90 isoforms of CUX1, which indicates higher Cat L activity within the nucleus since CUX1 is a substrate of Cat L in the nucleus (6). So far, only few substrates of nuclear cysteine peptidases have been identified, namely, CUX1, the histones ...

The Ustilago maydis lipase UM03410 belongs to the mostly unexplored Candida antarctica lipase (CAL-A) subfamily. The two lipases with […] the highest identity are a lipase from Sporisorium reilianum and the prototypic CAL-A. In contrast to the other CAL-A-type lipases, this hypothetical U. maydis lipase is annotated to possess a prolonged N-terminus of unknown function. Here, we show for the first time the recombinant expression of two versions of lipase UM03410: the full-length form (lipUMf) and an N-terminally truncated form (lipUMs). For comparison to the prototype, the expression of recombinant CAL-A in E. coli was investigated. Although both forms of lipase UM03410 could be expressed functionally in E. coli, the N-terminally truncated form (lipUMs) demonstrated significantly higher activities towards p-nitrophenyl esters. The functional expression of the N-terminally truncated lipase was further optimized by the appropriate choice of the E. coli strain, lowering the cultivation ...

MO-DC generated in vitro serve as a model type of DC to unravel the complex interactions among DC maturation, MHC class II peptide loading, and endocytic transport (3, 33, 34). The emerging picture suggests that endocytic protease activity is regulated by differential activity of the lysosomal ATPase during DC maturation (3). By analyzing lysosomal MBP processing at pH 5.0 in vitro, we have here mimicked the conditions present in the lysosomal compartment of DC in the activated state in vivo.. The MHC class II-associated proteolytic machinery is characterized by a hierarchical proteolytic cascade, where the initial step controls the efficiency of Ag processing, peptide presentation, and T cell activation (14). Different types of APC as well as primary cells and immortalized cell lines contain distinct activity patterns of endocytic proteases (11, 17, 31, 35, 36, 37) which might result in different processing pathways for a given Ag and hence in different selections of peptides presented. We here ...

Squamous cell carcinoma (SCC) is a treatment-refractory subtype of human cancer arising from stratified epithelium of the skin, lung, esophagus, oropharynx and other tissues. A unifying feature of SCC is high-level expression of the p53 related protein p63 (TP63) in 80% of cases. The major p63 isoform expressed in SCC is ΔNp63α, an N-terminally truncated form which functions as a key SCC cell survival factor by mechanisms that are unclear. In this study we demonstrate that ΔNp63α associates with HDAC1 and HDAC2 to form an active transcriptional repressor complex that can be targeted to therapeutic advantage. Repression of pro-apoptotic Bcl-2 family member genes including PUMA by p63/HDAC is required for survival of SCC cells. Cisplatin chemotherapy, a mainstay of SCC treatment, promotes dissociation of p63 and HDAC from the PUMA promoter, leading to increased histone acetylation, PUMA activation and apoptosis. These effects are recapitulated upon targeting the p63/HDAC complex selectively ...

Firstly, we analyzed hAFC samples for the expression of germ cell markers; in agreement to previous reports [11], we observed a consistently high expression of OCT4 and CD133. However, when we examined additional markers we did not observe expression of genes specific to germ cell differentiation. In this regard, it was reported that hAFCs had the ability to form embryoid bodies (EBs) when cultured under conditions without anti-differentiation factors and without contact to feeder cells. Moreover, the formation of such three-dimensional multicellular aggregates was accompanied by a decrease in stem cell marker expression and by the induction of differentiation into different cell lineages [23]. Hence herein hAFCs kept under a stem cell culture system yielded a high number of clones that when cultured in suspension without anti-differentiation factors, spontaneously formed multicellular EBs and harbored high differentiation potential. Moreover, when we cultured EBs in the presence of germ cell ...

Cancer stem cells (CSCs) are considered as the origin of tumor relapse. Here, we investigated the effects of Fructus Viticis total flavonoids (FVTF) on the characteristics of lung cancer stem-like cells (LCSLCs) derived from human small cell lung cancer NCI-H446 cell line and its potential mechanism. Human small cell lung cancer NCI-H446 cell line was cultured in vitro. The CD133+ cells were sorted from NCI-H446 cell line by magnetic separation. The suspended culture with stem cell-conditioned medium was used to amplify CD133+ sphere-forming cells (SFCs). The stem cell characteristics of CD133+ SFCs were evaluated using cell self-renewal capacity by tumor sphere formation assay, migration and invasion capacity by Transwell assay, tumorigenicity by xenograft model in nude mouse and cancer stem cell markers expression levels by western blot. The effects of FVTF on the properties of LCSLCs were examined by tumorsphere formation assay and transwell chamber assay. The expression level of p-Akt was determined

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Purpose: Diabetic corneas have ultrastructural changes that can lead to dry eye, abnormal healing after trauma. The literature is sparse on the topic of Asian diabetics corneas with good Va and automated refractive keratometry readings. At our retina clinic in California, we retrospectively evaluated the keratometry readings of corneas of Diabetic patients in an Asian population. Are there external refractive or superficial keratometry differences in Asian Diabetic vs Asian Control eyes?. Methods: Corneal topography was performed using Topcon KR-8000PA Auto Kerato- Refractometer (Oakland, NJ) on patients as part of the refractive screening prior to dilation for evaluation of diabetic retinopathy ...

Quantification of the 5 noncoding region of the genomic and antigenomic HCV RNA strands was done using a strand-specific real-time RT-PCR, with use of the thermostable enzyme Tth for the synthesis of cDNA at a high temperature. Thus, for the amplification of the genomic HCV RNA strand, cDNA was generated in 20 L of reaction mixture that contained the total RNA extracted from 200 uL of serum or 0.5 ug of total RNA from liver specimens or PBMC samples, 50 pmol/L antisense primer UTRLC2 (5-CAAGCACCCTATCAGGCAGT-3), 1 mmol/L MnCl2, 200 umol/L each deoxynucleotide triphosphate, 1X RT buffer (Applied Biosystems), and 5 U of Tth (Applied Biosystems). After 20 min at 65 C, the RT activity was inactivated by Mn2+ chelation with 8 uL of the 10X chelating buffer (Applied Biosystems), followed by heating at 95 C for 30 min. For amplification of antigenomic HCV RNA, cDNA was synthesized under the same conditions by the addition of 50 pmol/L sense primer UTRLC1 (5-CTTCACGCAGAAAGCGTCTA-3). Real-time PCR was ...

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The neuronal ceroid lipofuscinoses (NCLs) are inherited neurodegenerative lysosomal storage disorders caused by the accumulation of ceroid and lipofuscin in various cell types, mainly cells of the cerebral cortex, cerebellar cortex, and retina (Dyken et al. 1988; Williams and Mole 2012). Characteristic features at onset include clumsiness; deterioration of vision and psychomotor functions; seizures; and behavioral changes. Progression of clinical features results ultimately in total disability, blindness and premature death. Although NCL affects primarily children, age of onset of symptoms varies from infancy to adulthood. The incidence of NCL is variable and ranges from 1.3 to 7 per 100,000 (Mole and Williams 2013). However, it is more common in northern European populations, particularly Finland where the incidence may reach 1 in 12,500 individuals and a carrier frequency of 1 in 70 (Rider and Rider 1988). NCLs are clinically and genetically heterogeneous. A nomenclature and classification ...

Pyroglutamate amyloid-β peptides (pGlu-Aβ) are particularly pernicious forms of amyloid-β peptides (Aβ) present in Alzheimers disease (AD) brains. pGlu-Aβ peptides are N-terminally truncated forms of full-length Aβ peptides (flAβ(1-40/42) ) in which

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I am trying to find if there is anyone in Brisbane, QLD, Australia who has the human osteosarcoma cell line U-2 OS in their lab. Any suggestions on how to get this information would be helpful Thank you Helen ...

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Introduction to Brain Tumor Stem Cells -- Isolation and Culture of Glioblastoma Brain Tumor Stem Cells -- Establishment and Culture of Patient-Derived Primary Medulloblastoma Cell Lines -- Bioinformatic Strategies for the Genomic and Epigenomic Characterization of Brain Tumors -- Detecting Stem Cell Marker Expression Using the NanoString nCounter System -- Flow Cytometric Analysis of Brain Tumor Stem Cells -- In Vitro Self-Renewal Assays for Brain Tumor Stem Cells -- Differentiation of Brain Tumor Initiating Cells -- The Study of Brain Tumor Stem Cell Migration -- The Study of Brain Tumor Stem Cell Invasion -- Cell Cycle Dynamics in Glioma Cancer Stem Cells -- Embryonic Stem Cell Models of Human Brain Tumors -- Chromatin Immunoprecipitation (ChIP) Protocols for the Cancer and Developmental Biology Laboratory -- EPH Profiling of BTIC Populations in Glioblastoma Multiforme Using CyTOF -- Pooled Lentiviral CRISPR-Cas9 Screens for Functional Genomics in Mammalian Cells -- In Vitro Assays for ...

We describe a comprehensive and practical protocol for fluorescence recovery after photobleaching experiments with live cells. Although ...

Ravina berei Drav Huna (499 or 421 CE). Rosh Metivta of Sura. He, together with his teacher, Rav Ashi, collected and commented upon the Gemara of what would henceforth be known as the Talmud Bavli.. Rav Azariah min Haadumim, author of Meor Einayim (1577).. Rav Shlomo Zalman Yosef of Vyelpol (1857).. Rav Dov Ber of Levo, son on Rav Yisrael of Ruzhin (1875).. Rav Yisrael Aryeh of Premishlan (1890).. Rav Dov Ber Livshitz, Rav of Sardnik (1900). Rav Yisrael Taub of Modzhitz, author of Divrei Yisrael (1849-1920). He was the son of Rav Shmuel Eliyahu Taub of Zvolin (1888) and the grandson of Rav Yechezkel Taub of Kuzmir (1856), who was one of the students of the Chozeh of Lublin. He became the first Rebbe of Modzhitz and was succeeded by his son, Shaul Yedidya Elazer. Legend has it that in 1913 Taub composed a 30-minute negun while having his leg amputated without anesthesia.. Rav Yisrael Friedman, the second Tchortkover Rebbe (1934, 1933, or 1932). Rav Yechiel Michel Hager of Horodenka (1941). One ...

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2018 Toyota RAV4 LEMarca: Toyota Modelo: RAV4 Año: 2018 Millas: 35409 Color: Exterior- Red Interior- Black Motor y Transmision: 2.5L 4-Cylin 18086983

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Background: Osteosarcoma (OS) is the most predominant bone tumor in individuals between 10-25 yrs of age. Cisplatin is the most widely used chemotherapeutic agent for OS management, it significantly enhances the survival rate. Resistance to the drug, toxicities of high doses and metastasis are the major concerns in the treatment. Objective: Our primary objective is to investigate effects of calcitriol in combination with cisplatin on the osteosarcoma cell apoptosis, invasion and migration. Our hypothesis is pretreatment of OS cells with calcitriol would sensitize them for cisplatin therapy leading to decrease in concentration for IC50. Secondary objective was to evaluate the effect of calcitriol on migration and invasion of OS cell lines, and the role of matrix metalloproteins (MMPs) in migration/invasion. Design: Previous findings in our lab suggests, calcitriol act as differentiation agent in human osteosarcoma cell lines 143B and SaOS-2. The dose response of cisplatin with and without ...

Prion rods, i.e. insoluble infectious aggregates of the N-terminally truncated form of the prion protein, PrP 27-30, and the corresponding recombinant protein, rPrP(90-231), were autoclaved in water, bovine lipid or lipid-water mixtures for 20 min at temperatures from 100 to 170 °C. A protocol was developed for the quantitative precipitation of small amounts of protein from large excesses of lipid. PrP remaining undegraded after autoclaving was quantified by Western blot and degradation factors were calculated. The Arrhenius plot of the rate of degradation vs temperature yielded linear relationships for prion rods in water or lipid-water as well as for rPrP(90-231) in lipid-water. The presence of lipids increased the heat stability of prion rods, especially at lower temperatures. Prion rods had a much higher thermal stability compared to rPrP. Autoclaving of prion rods in pure lipid gave different results - not simple degradation but bands indicative of covalently linked dimers, tetramers and higher

Yamawaki H, Sato K, Hori M, Ozaki H, Nakamura S, Nakayama H, Doi K, Karaki H. Impairment of EDR by a long-term PDGF treatment in organ-cultured rabbit mesenteric artery. Am J Physiol. 1999 07; 277(1):H318-23 ...

Rav Shmuel Auerbach, rosh yeshiva of Yeshiva Maalos Hatorah and son of Rav Shlomo Zalman Auerbach ztl, was released today from the hospital after undergoing a complex catheterization. The procedure was performed yesterday at Hadassah Ein Kerem Medical Center in southwest Yerushalayim.. Rav Shmuel remained hospitalized for observation for 24 hours after the catheterization, and doctors seem to be pleased with his condition. Rav Shmuel will continue his recovery at home.. It is hoped that Rav Shmuel can resume his regular schedule by the end of this week.. Rav Shmuel was greeted by a crowd of neighbors and onlookers as he arrived to his home on Rechov Ibn Shaprut in the Shaarei Chesed neighborhood of Yerushalayim at about noon.. All are asked to daven for the complete recovery of Rav Shmuel ben Chaya Rivka, besoch shear cholei Yisroel.. {Matzav.com Israel New Bureau}. ...

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Thrombospondin-1 (TSP1) is known to possess tumor suppressor functions. In contradiction, TSP1 enhances the stromal vascularization and growth of certain cancers. A cell adhesion receptor, CD36, has been shown to interact with a ligand TSP1. We studied how CD36 affects the growth of the osteosarcoma cell line (HOS) expressing TSP1. We used the anti-CD36 ribozyme to specifically suppress CD36 gene expression in the HOS. The expression of the CD36 mRNA was significantly suppressed in the ribozyme-introduced cell line (HOS/Rz). The transformant HOS/Rz markedly decreased its growth. The growth of the osteosarcoma cell line HOS may be regulated by autocrine or paracrine loop TSP1 and CD36.

Osteosarcoma (OS) is the most common type of bone cancer in children and adolescents. With the current treatment approaches, the survival rate is 60-70%. Therapies for treating OS have remained the same over the past thirty years. There is a need for developing more effective therapies, which can significantly improve the survival of the patients who do not respond well to current therapeutic strategies. Epidemiological evidence indicates impaired regulation of cell proliferation and survival appears to be a key event in the etiology of the disease. Previous in vitro and in vivo studies suggest that 1α,25-dihydroxyvitamin D3 (calcitriol or 1,25D) has significant antineoplastic activity by inhibiting cell proliferation and inducing differentiation and apoptosis in breast, prostate, colon, skin, and brain cancer. The molecular mechanisms for calcitriol-mediated cancer prevention are still unknown. In particular, the role of calcitriol in modulating OS needs to be investigated. The primary ...

Esophageal cancer is consists of highly heterogeneous populations, which include cancer initiating cells, so-called cancer stem cells. Although histone modification is shown to be an essential factor for normal stem cell maintenance, the involvement of histone demethylase in cancer progression has been poorly understood. We investigated the functional roles of the Histone H3 lysine 4 (H3K4) demethylase KDM5B, a crucial epigenetic regulator that is required for normal embryonic development and cell growth. KDM5B knockdown resulted in the suppression of esophageal cancer cell growth, sphere formation and invasion ability and was associated with loss of epithelial cell marker expression. These tumor inhibitory effects were reverted subsequent to subcutaneous inoculation of these cells into immune-deficient mice. These results indicated that KDM5B plays an important role in maintaining cancer stem cells and justifies the rationale for studying the effects of continuous inhibition of this epigenetic ...

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