19 results match your criteria metabolism vks

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A generic emergency protocol for patients with inborn errors of metabolism causing fasting intolerance: a retrospective, single-center study and the generation of www.emergencyprotocol.net.

J Inherit Metab Dis 2021 Apr 12. Epub 2021 Apr 12.

Section of Metabolic Diseases, Beatrix Children's Hospital, University Medical Centre Groningen, University of Groningen, Groningen, The Netherlands.

Introduction: Patients with inborn errors of metabolism causing fasting intolerance can experience acute metabolic decompensations. Long-term data on outcomes using emergency letters are lacking.

Methods: This is a retrospective, observational, single-center study of the use of emergency letters based on a generic emergency protocol in patients with hepatic glycogen storage diseases (GSD) or fatty acid oxidation disorders (FAOD). Read More

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Consensus guidelines for the diagnosis and management of pyridoxine-dependent epilepsy due to α-aminoadipic semialdehyde dehydrogenase deficiency.

J Inherit Metab Dis 2021 Jan 1;44(1):178-192. Epub 2020 Dec 1.

Department of Pediatrics Emma Children's Hospital, Amsterdam University Medical Centre, Amsterdam, The Netherlands.

Pyridoxine-dependent epilepsy (PDE-ALDH7A1) is an autosomal recessive condition due to a deficiency of α-aminoadipic semialdehyde dehydrogenase, which is a key enzyme in lysine oxidation. PDE-ALDH7A1 is a developmental and epileptic encephalopathy that was historically and empirically treated with pharmacologic doses of pyridoxine. Despite adequate seizure control, most patients with PDE-ALDH7A1 were reported to have developmental delay and intellectual disability. Read More

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January 2021

Registries for orphan drugs: generating evidence or marketing tools?

Orphanet J Rare Dis 2020 09 3;15(1):235. Epub 2020 Sep 3.

Platform Medicine for Society at Amsterdam University Medical Centers, location Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands.

Independent disease registries for pre-and post-approval of novel treatments for rare diseases are increasingly important for healthcare professionals, patients, regulators and the pharmaceutical industry. Current registries for rare diseases to evaluate orphan drugs are mainly set up and owned by the pharmaceutical industry which leads to unacceptable conflicts of interest. To ensure independence from commercial interests, disease registries should be set up and maintained by healthcare professionals and patients. Read More

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September 2020

Characterization of an extracellular protein, Rv1076 from M. tuberculosis with a potential role in humoral response.

Int J Biol Macromol 2017 Aug 19;101:621-629. Epub 2017 Mar 19.

Department of Biotechnology, BMS Block-I, South Campus, Panjab University, Sector 25, Chandigarh, 160014, India. Electronic address:

Many mycobacterial proteins involved in lipid metabolism are reported to be essential for survival and pathogenesis of M. tuberculosis. Rv1076 of M. Read More

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Position statement on the role of healthcare professionals, patient organizations and industry in European Reference Networks.

Orphanet J Rare Dis 2016 Jan 25;11. Epub 2016 Jan 25.

Amsterdam Institute for Social Science Research, University of Amsterdam, Amsterdam, The Netherlands.

A call from the EU for the set-up of European Reference Networks (ERNs) is expected to be launched in the first quarter of 2016. ERNs are intended to improve the care for patients with low prevalent or rare diseases throughout the EU by, among other things, facilitating the pooling and exchange of experience and knowledge and the development of protocols and guidelines. In the past, for example where costly orphan drugs have been concerned, industry has played an important role in facilitating consensus meetings and publication of guidelines. Read More

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January 2016

Vitamin K and hepatocellular carcinoma: The basic and clinic.

World J Clin Cases 2015 Sep;3(9):757-64

Xia Jinghe, Iwata Ozaki, Division of Hepatology, Diabetology and Endocrinology, Department of Internal Medicine, Saga Medical School, Saga University, Saga 849-8501, Japan.

Vitamin K (VK), which was originally identified as a cofactor involved in the production of functional coagulation factors in the liver, has been shown to be involved in various aspects of physiological and pathological events, including bone metabolism, cardiovascular diseases and tumor biology. The mechanisms and roles of VK are gradually becoming clear. Several novel enzymes involved in the VK cycle were identified and have been shown to be linked to tumorigenesis. Read More

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September 2015

Anti-E1E2 antibodies do predict response to triple therapy in treatment-experienced Hepatitis C Virus-cirrhosis cases.

Clin Res Hepatol Gastroenterol 2015 Dec 18;39(6):699-704. Epub 2015 Apr 18.

Centre de recherche en cancérologie de Lyon (CRCL), UMR I 1052/CNRS 5286, 69424 Lyon cedex 03, France; Université Claude-Bernard Lyon 1, 69100 Villeurbanne, France; Hospices civils de Lyon, hôpital de la Croix-Rousse, service d'hépatologie et de gastroentérologie, 69004 Lyon, France.

Background And Aims: We previously showed that pre-treatment serum anti-E1E2 predicted hepatitis C virus (HCV) RNA viral kinetics (VKs) and treatment outcome in patients with chronic hepatitis C receiving pegylated interferon/ribavirin (Peg-IFN/RBV) double therapy. Here, we determined whether baseline anti-E1E2 was correlated with the on-treatment VK and could predict virological outcome in treatment-experienced HCV-infected cirrhotic patients receiving protease inhibitor-based triple therapy.

Methods: Sera from 19 patients with HCV genotype 1 infection and compensated cirrhosis who failed to respond to a prior course of Peg-IFN/RBV were selected at time 0 before starting triple therapy with boceprevir or telaprevir. Read More

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December 2015

DHA-enriched high-oleic acid canola oil improves lipid profile and lowers predicted cardiovascular disease risk in the canola oil multicenter randomized controlled trial.

Am J Clin Nutr 2014 Jul 14;100(1):88-97. Epub 2014 May 14.

From the Richardson Centre for Functional Foods and Nutraceuticals, University of Manitoba, Winnipeg, Canada (PJHJ, VKS, and SP); the Risk Factor Modification Centre (DJAJ) and the Keenan Research Centre for Biomedical Science (PWC), St. Michaels Hospital, University of Toronto, Toronto, Canada; the Institute of Nutrition and Functional Foods, Laval University, Québec City, Canada (BL, PC, AC, and LB-G); the Department of Nutritional Sciences (SGW, XL, JAF, and PMK-E) and the Department of Biobehavioral Health (SGW and CEM), Pennsylvania State University, University Park, PA.

Background: It is well recognized that amounts of trans and saturated fats should be minimized in Western diets; however, considerable debate remains regarding optimal amounts of dietary n-9, n-6, and n-3 fatty acids.

Objective: The objective was to examine the effects of varying n-9, n-6, and longer-chain n-3 fatty acid composition on markers of coronary heart disease (CHD) risk.

Design: A randomized, double-blind, 5-period, crossover design was used. Read More

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Clinical pathways for inborn errors of metabolism: warranted and feasible.

Orphanet J Rare Dis 2013 Feb 25;8:37. Epub 2013 Feb 25.

Inborn errors of metabolism (IEMs) are known for their low prevalence and multidisciplinary care mostly founded on expert opinion. Clinical pathways are multidisciplinary tools to organise care which provide a clear route to the best care and improve communication. In 2010 the Dutch Society for Children and Adults with an Inborn Error of Metabolism (VKS) initiated development of clinical pathways for inborn errors of metabolism. Read More

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February 2013

Inhibition of repair-related DNA polymerases by vitamin Ks, their related quinone derivatives and associated inflammatory activity (Review).

Int J Oncol 2013 Mar 15;42(3):793-802. Epub 2013 Jan 15.

Laboratory of Food and Nutritional Sciences, Department of Nutritional Science, Kobe-Gakuin University, Kobe, Hyogo 651-2180, Japan.

Vitamin Ks (VKs) are fat-soluble quinone compounds known to have various bioactivities. This review describes the inflammatory effects of VKs and their related quinone derivatives based on DNA polymerase (pol) inhibition. VK3, but not VK1 or VK2 (=MK-4), inhibited the activity of human pol γ, which is the DNA replicative pol in mitochondria. Read More

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Effect of IL28B genotype on early viral kinetics during interferon-free treatment of patients with chronic hepatitis C.

Gastroenterology 2012 Apr 13;142(4):790-5. Epub 2012 Jan 13.

Roche, Nutley, New Jersey, USA.

Background & Aims: Although interleukin 28B (interferon, lambda 3) (IL28B) genotype affects the response of patients with chronic hepatitis C to peginterferon and ribavirin, little is known regarding its effect on response to direct-acting antivirals in interferon-free combinations. We analyzed the effects of IL28B genotype on the viral kinetic (VK) response to an interferon-free combination of the nucleoside polymerase inhibitor mericitabine (RG7128) and the hepatitis C virus (HCV) protease inhibitor danoprevir.

Methods: We performed a double-blind, dose-escalation study of patients with chronic HCV genotype 1 infection who were interferon treatment naive or had not responded to previous therapy with peginterferon and ribavirin. Read More

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Vitamin K has the potential to protect neurons from methylmercury-induced cell death in vitro.

J Neurosci Res 2011 Jul 12;89(7):1052-8. Epub 2011 Apr 12.

Department of Anatomy II, School of Veterinary Medicine, Azabu University, Sagamihara, Japan.

Vitamin K (VK) has a protective effect on neural cells. Methylmercury is a neurotoxicant that directly induces neuronal death in vivo and in vitro. Therefore, in the present study, we hypothesized that VK inhibits the neurotoxicity of methylmercury. Read More

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Vitamin K enhancement of sorafenib-mediated HCC cell growth inhibition in vitro and in vivo.

Int J Cancer 2010 Dec;127(12):2949-58

Department of Medical Oncology, Kimmel Cancer Center, Thomas Jefferson University, Philadelphia, PA 19107, USA.

The multikinase inhibitor sorafenib is the first oral agent to show activity against human hepatocellular carcinoma (HCC). Apoptosis has been shown to be induced in HCC by several agents, including sorafenib as well as by the naturally occurring K vitamins (VKs). As few nontoxic agents have activity against HCC growth, we evaluated the activity of sorafenib and VKs, both independently and together on the growth of HCC cells in vitro and in vivo. Read More

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December 2010

Characterisation and potential diagnostic value of circulating matrix Gla protein (MGP) species.

Thromb Haemost 2010 Oct 5;104(4):811-22. Epub 2010 Aug 5.

VitaK and Cardiovascular Research Institute Maastricht (CARIM), Maastricht University, Maastricht, The Netherlands.

Matrix γ-carboxyglutamate (Gla) protein (MGP) is an important local inhibitor of vascular calcification, which can undergo two post-translational modifications: vitamin K-dependent γ-glutamate carboxylation and serine phosphorylation. While carboxylation is thought to have effects upon binding of calcium-ions, phosphorylation is supposed to affect the cellular release of MGP. Since both modifications can be exerted incompletely, various MGP species can be detected in the circulation. Read More

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October 2010

Sorafenib combined vitamin K induces apoptosis in human pancreatic cancer cell lines through RAF/MEK/ERK and c-Jun NH2-terminal kinase pathways.

J Cell Physiol 2010 Jul;224(1):112-9

Department of Medical Oncology, Kimmel Cancer Center, Thomas Jefferson University, Philadelphia, Pennsylvania 19107, USA.

Apoptosis has been shown to be induced by many agents, including the clinically useful Sorafenib and K vitamins (VKs). Since few agents have activity against pancreas cancer cell growth, we evaluated the role of naturally occurring K vitamins and Sorafenib both independently and together on the growth in culture of pancreas adenocarcinoma cell lines, including PL-5, PANC-1, and MIA PaCa-2. We found that when a K vitamin was combined with Sorafenib, the dose of Sorafenib required for growth inhibition was substantially reduced. Read More

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Does clofazimine (B663) reach Mycobacterium leprae persisting in Schwann cells and endothelial cells of endoneurial blood vessels in peripheral nerves?

V Kumar

Microsc Res Tech 2008 Aug;71(8):614-8

National JALMA Institute for Leprosy and Other Mycobacterial Diseases (ICMR), Taj Ganj, Agra 282001, India.

Peripheral nerve biopsies from 10 Lepromatous leprosy (LL) patients who were on multidrug treatment (MDT) were investigated by light and electron microscopy. Clofazimine (CLF) has been included as an essential component of MDT, which is the standard WHO regimen for treatment of leprosy. The patients receiving continuous MDT for a long period had viable bacilli in Schwann cells (SCs) of peripheral nerves whereas they had disappeared from the skin. Read More

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A novel and efficient synthesis of bicyclo[2.2.2]octenones and sigmatropic shifts in ground and excited states: stereoselective route to cis-decalins and diquinane frameworks.

J Org Chem 2005 Feb;70(3):973-81

Department of Chemistry, National Single Crystal X-ray Diffraction Facility, Indian Institute of Technology-Bombay, Mumbai 400 076, India.

A new and efficient synthesis of a variety of highly embellished bicyclooctenones having an endo-vinyl moiety and their sigmatropic shifts in ground and excited states leading to a stereoselective route to substituted cis-decalins and diquinane frameworks have been described. Functionalized bicyclo[2.2. Read More

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February 2005

Simultaneous determination of vitamin K analogs in human serum by sensitive and selective high-performance liquid chromatography with electrochemical detection.

Nutrition 2003 Jul-Aug;19(7-8):661-5

Department of Clinical Pharmacotherapy, Faculty of Pharmaceutical Sciences, Niigata University of Pharmacy and Applied Life Sciences, Niigata, Japan.

Objectives: We report a sensitive and selective method for the simultaneous determination of vitamin K1 and K2 analogs (VKs) with high-performance liquid chromatography in which a platinum catalyst reduction column and an electrochemical detector operated in the oxidation mode are incorporated into the detection system. We also applied this trace analysis method to the simultaneous determination of VKs in human serum to investigate the physiologic and pathophysiologic roles of VKs in the bone metabolism.

Methods: Our high-performance liquid chromatographic method with postcolumn catalyst reduction and electrochemical detection was applied for the simultaneous determination of VKs in human serum samples. Read More

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January 2004

Identification of sequences within the gamma-carboxylase that represent a novel contact site with vitamin K-dependent proteins and that are required for activity.

J Biol Chem 2001 Dec 8;276(50):46878-86. Epub 2001 Oct 8.

Department of Molecular Cardiology, Lerner Research Institute, Cleveland Clinic Foundation, Cleveland, OH 44195, USA.

The vitamin K-dependent (VKD) carboxylase converts clusters of Glu residues to gamma-carboxylated Glu residues (Glas) in VKD proteins, which is required for their activity. VKD precursors are targeted to the carboxylase by their carboxylase recognition site, which in most cases is a propeptide. We have identified a second tethering site for carboxylase and VKD proteins that is required for carboxylase activity, called the vitamin K-dependent protein site of interaction (VKS). Read More

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December 2001
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