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Alpha-Hydroxy acid

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141. Identification of Seven Novel Mutations in the Acid Alpha-glucosidase Gene in Five Chinese Patients with Late-onset Pompe Disease (PubMed)

Identification of Seven Novel Mutations in the Acid Alpha-glucosidase Gene in Five Chinese Patients with Late-onset Pompe Disease Pompe disease is a rare lysosomal glycogen storage disorder linked to the acid alpha-glucosidase gene (GAA). A wide clinical and genetic variability exists between patients from different ethnic populations, and the genotype-phenotype correlations are still not well understood. The aim of this study was to report the clinicopathological and genetic characteristics (...) in the proximal and axial muscles, while one patient developed respiratory insufficiency that required artificial ventilation. In muscle biopsies, vacuoles with variable sizes and shapes appeared inside muscle fibers, and they stained positive for both periodic acid-Schiff and acid phosphatase staining. Ten GAA gene mutations, including seven novel ones (c.796C>A, c.1057C>T, c.1201C>A, c.1780C>T, c.1799G>C, c.2051C>A, c.2235dupG), were identified by genetic tests.The seven novel GAA gene mutations revealed

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2018 Chinese medical journal

142. Design and synthesis of a potent, highly selective, orally bioavailable, retinoic acid receptor alpha agonist (PubMed)

Design and synthesis of a potent, highly selective, orally bioavailable, retinoic acid receptor alpha agonist A ligand-based virtual screening exercise examining likely bioactive conformations of AM 580 (2) and AGN 193836 (3) was used to identify the novel, less lipophilic RARα agonist 4-(3,5-dichloro-4-ethoxybenzamido)benzoic acid 5, which has good selectivity over the RARβ, and RARγ receptors. Analysis of the medicinal chemistry parameters of the 3,5-substituents of derivatives of template 5 (...) enabled us to design a class of drug-like molecules with lower intrinsic clearance and higher oral bioavailability which led to the novel RARα agonist 4-(3-chloro-4-ethoxy-5-isopropoxybenzamido)-2-methylbenzoic acid 56 that has high RARα potency and excellent selectivity versus RARβ (2 orders of magnitude) and RARγ (4 orders of magnitude) at both the human and mouse RAR receptors with improved drug-like properties. This RARα specific agonist 56 has high oral bioavailability (>80%) in both mice

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2018 Bioorganic & medicinal chemistry

143. Early alpha-lipoic acid therapy protects from degeneration of the inner retinal layers and vision loss in an experimental autoimmune encephalomyelitis-optic neuritis model (PubMed)

Early alpha-lipoic acid therapy protects from degeneration of the inner retinal layers and vision loss in an experimental autoimmune encephalomyelitis-optic neuritis model In multiple sclerosis (MS), neurodegeneration is the main reason for chronic disability. Alpha-lipoic acid (LA) is a naturally occurring antioxidant which has recently been demonstrated to reduce the rate of brain atrophy in progressive MS. However, it remains uncertain if it is also beneficial in the early, more inflammatory

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2018 Journal of neuroinflammation

144. Exercise and Redox Status Responses Following Alpha-Lipoic Acid Supplementation in G6PD Deficient Individuals (PubMed)

Exercise and Redox Status Responses Following Alpha-Lipoic Acid Supplementation in G6PD Deficient Individuals G6PD deficiency renders cells more susceptible to oxidative insults, while antioxidant dietary supplementation could restore redox balance and ameliorate exercise-induced oxidative stress. To examine the effects of alpha-lipoic acid (ALA) supplementation on redox status indices in G6PD deficient individuals, eight male adults with G6PD deficiency (D) participated in this randomized (...) double-blind placebo-controlled crossover trial. Participants were randomly assigned to receive ALA (600 mg/day) or placebo for 4 weeks separated by a 4-week washout period. Before and at the end of each treatment period, participants exercised following an exhaustive treadmill exercise protocol. Blood samples were obtained before (at rest), immediately after and 1h after exercise for later analysis of total antioxidant capacity (TAC), uric acid, bilirubin, thiobarbituric acid reactive substances

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2018 Antioxidants

145. γ-Aminobutyric acid receptor alpha 1 subunit loss of function causes genetic generalized epilepsy by impairing inhibitory network neurodevelopment. (PubMed)

γ-Aminobutyric acid receptor alpha 1 subunit loss of function causes genetic generalized epilepsy by impairing inhibitory network neurodevelopment. In humans, mutations of the γ-aminobutyric acid receptor subunit 1 (GABRA1) cause either mild or severe generalized epilepsy. Although these epilepsy-causing mutations have been shown to disrupt the receptor activity in vitro, their in vivo consequences on brain development and activity are not known. Here, we aim at unraveling the epileptogenesis

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2018 Epilepsia

146. Dietary Intake of Alpha-Linolenic Acid Is Not Appreciably Associated with the Risk of Ischemic Stroke among Middle-Aged Danish Men and Women. (PubMed)

Dietary Intake of Alpha-Linolenic Acid Is Not Appreciably Associated with the Risk of Ischemic Stroke among Middle-Aged Danish Men and Women. Intake of the plant-derived omega-3 (n-3) fatty acid α-linolenic acid (ALA) may reduce the risk of ischemic stroke.We have investigated the associations between dietary intake of ALA and the risk of ischemic stroke and ischemic stroke subtypes.This was a follow-up study. A total of 57,053 participants aged 50-64 y were enrolled into the Danish Diet

2018 Journal of Nutrition

147. Arachidonic acid mediates the formation of abundant alpha-helical multimers of alpha-synuclein (PubMed)

Arachidonic acid mediates the formation of abundant alpha-helical multimers of alpha-synuclein The protein alpha-synuclein (αS) self-assembles into toxic beta-sheet aggregates in Parkinson's disease, while it is proposed that αS forms soluble alpha-helical multimers in healthy neurons. Here, we have made αS multimers in vitro using arachidonic acid (ARA), one of the most abundant fatty acids in the brain, and characterized them by a combination of bulk experiments and single-molecule Fӧrster (...) work provides strong biophysical evidence for the formation of alpha-helical multimers of αS in the presence of a biologically relevant fatty acid, which may have a protective role with respect to the generation of beta-sheet toxic structures during αS fibrillation.

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2016 Scientific reports

148. Tetrahydro-iso-alpha Acids Antagonize Estrogen Receptor Alpha Activity in MCF-7 Breast Cancer Cells (PubMed)

Tetrahydro-iso-alpha Acids Antagonize Estrogen Receptor Alpha Activity in MCF-7 Breast Cancer Cells Tetrahydro-iso-alpha acids commonly called THIAA or Tetra are modified hop acids extracted from hop (Humulus lupulus L.) which are frequently used in brewing industry mainly in order to provide beer bitterness and foam stability. Interestingly, molecular structure of tetrahydro-iso-alpha acids is close to a new type of estrogen receptor alpha (ERα) antagonists aimed at disrupting the binding

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2016 International journal of endocrinology

149. Alpha-linolenic acid and risk of cardiovascular disease: a systematic review and meta-analysis

Alpha-linolenic acid and risk of cardiovascular disease: a systematic review and meta-analysis Untitled Document The CRD Databases will not be available from 08:00 BST on Friday 4th October until 08:00 BST on Monday 7th October for essential maintenance. We apologise for any inconvenience.

2012 DARE.

150. A systematic review and meta-analysis of alpha-lipoic acid in the treatment of diabetic peripheral neuropathy

A systematic review and meta-analysis of alpha-lipoic acid in the treatment of diabetic peripheral neuropathy Untitled Document The CRD Databases will not be available from 08:00 BST on Friday 4th October until 08:00 BST on Monday 7th October for essential maintenance. We apologise for any inconvenience.

2012 DARE.

151. Alpha lipoic acid for symptomatic peripheral neuropathy in patients with diabetes: a meta-analysis of randomized controlled trials

Alpha lipoic acid for symptomatic peripheral neuropathy in patients with diabetes: a meta-analysis of randomized controlled trials Untitled Document The CRD Databases will not be available from 08:00 BST on Friday 4th October until 08:00 BST on Monday 7th October for essential maintenance. We apologise for any inconvenience.

2012 DARE.

152. The lipid peroxidation product 4-hydroxy-2-nonenal induces tissue factor decryption via ROS generation and the thioredoxin system (PubMed)

The lipid peroxidation product 4-hydroxy-2-nonenal induces tissue factor decryption via ROS generation and the thioredoxin system Many pathophysiologic agents transform cryptic tissue factor (TF) on cells to prothrombotic TF, and one such stimulus is 4-hydroxy-2-nonenal (HNE), the most abundant aldehyde produced by the oxidation of ω-6 polyunsaturated fatty acids. HNE was shown to induce reactive oxygen species (ROS) generation and p38 MAPK activation, but the link between them and their role

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2017 Blood advances

153. Circulating linoleic acid and alpha-linolenic acid and glucose metabolism: the Hoorn Study (PubMed)

Circulating linoleic acid and alpha-linolenic acid and glucose metabolism: the Hoorn Study Data on the relation between linoleic acid (LA) and alpha-linolenic acid (ALA) and type 2 diabetes mellitus (T2DM) risk are scarce and inconsistent. The aim of this study was to investigate the association of serum LA and ALA with fasting and 2 h post-load plasma glucose and glycated hemoglobin (HbA1c).This study included 667 participants from third examination (2000) of the population-based Hoorn study (...) in which individuals with glucose intolerance were overrepresented. Fatty acid profiles in serum total lipids were measured at baseline, in 2000. Diabetes risk markers were measured at baseline and follow-up in 2008. Linear regression models were used in cross-sectional and prospective analyses.In cross-sectional analyses (n = 667), serum LA was inversely associated with plasma glucose, both in fasting conditions (B = -0.024 [-0.045, -0.002]) and 2 h after glucose tolerance test (B = -0.099 [-0.158

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2016 European journal of nutrition

154. The Role of Hepatocyte Nuclear Factor 4-Alpha in Perfluorooctanoic Acid- and Perfluorooctanesulfonic Acid-Induced Hepatocellular Dysfunction (PubMed)

The Role of Hepatocyte Nuclear Factor 4-Alpha in Perfluorooctanoic Acid- and Perfluorooctanesulfonic Acid-Induced Hepatocellular Dysfunction Perfluorooctanoic acid (PFOA) and perfluorooctanesulfonic acid (PFOS), chemicals present in a multitude of consumer products, are persistent organic pollutants. Both compounds induce hepatotoxic effects in rodents, including steatosis, hepatomegaly and liver cancer. The mechanisms of PFOA- and PFOS-induced hepatic dysfunction are not completely understood (...) . We present evidence that PFOA and PFOS induce their hepatic effects via targeting hepatocyte nuclear factor 4-alpha (HNF4α). Human hepatocytes treated with PFOA and PFOS at a concentration relevant to occupational exposure caused a decrease in HNF4α protein without affecting HNF4α mRNA or causing cell death. RNA sequencing analysis combined with Ingenuity Pathway Analysis of global gene expression changes in human hepatocytes treated with PFOA or PFOS indicated alterations in the expression

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2016 Toxicology and applied pharmacology

155. Resolution of subchorionic hematoma and symptoms of threatened miscarriage using vaginal alpha lipoic acid or progesterone: clinical evidences. (PubMed)

Resolution of subchorionic hematoma and symptoms of threatened miscarriage using vaginal alpha lipoic acid or progesterone: clinical evidences. Alpha Lipoic Acid (ALA) is a safe natural molecule that exerts a selective immunomodulating activity with antioxidant and anti-inflammatory properties. This randomized controlled clinical trial (RCT) tested the effect of the vaginal administration with ALA or Progesterone, in subchorionic hematoma resorption in women with threatened miscarriage.400 mg

2017 European review for medical and pharmacological sciences

156. The Effect of Alpha Linolenic Acid (ALA) Supplementation During Pregnancy

The Effect of Alpha Linolenic Acid (ALA) Supplementation During Pregnancy The Effect of Alpha Linolenic Acid (ALA) Supplementation During Pregnancy - Full Text View - ClinicalTrials.gov Hide glossary Glossary Study record managers: refer to the if submitting registration or results information. Search for terms x × Study Record Detail Saved Studies Save this study Warning You have reached the maximum number of saved studies (100). Please remove one or more studies before adding more. The Effect (...) of Alpha Linolenic Acid (ALA) Supplementation During Pregnancy The safety and scientific validity of this study is the responsibility of the study sponsor and investigators. Listing a study does not mean it has been evaluated by the U.S. Federal Government. of clinical studies and talk to your health care provider before participating. Read our for details. ClinicalTrials.gov Identifier: NCT03040856 Recruitment Status : Not yet recruiting First Posted : February 2, 2017 Last Update Posted : February 15

2017 Clinical Trials

157. Dietary Intake of Alpha-linolenic Acid in Elderly

Dietary Intake of Alpha-linolenic Acid in Elderly Dietary Intake of Alpha-linolenic Acid in Elderly - Full Text View - ClinicalTrials.gov Hide glossary Glossary Study record managers: refer to the if submitting registration or results information. Search for terms x × Study Record Detail Saved Studies Save this study Warning You have reached the maximum number of saved studies (100). Please remove one or more studies before adding more. Dietary Intake of Alpha-linolenic Acid in Elderly (...) by (Responsible Party): Fathi Driss, Assistance Publique - Hôpitaux de Paris Study Details Study Description Go to Brief Summary: The purpose of this study is to evaluate the antiatherogenic and anti-inflammatory potential of an adequate intake of alpha-linolenic acid in elderly. Condition or disease Intervention/treatment Phase Cardiovascular Diseases Inflammation Metabolism Disorder, Lipid Dietary Supplement: Rapeseed oil Dietary Supplement: Sunflower oil Not Applicable Detailed Description

2017 Clinical Trials

158. Diacylglycerol Enhances the Effects of Alpha-Linolenic Acid Against Visceral Fat: A Double-Blind Randomized Controlled Trial. (PubMed)

Diacylglycerol Enhances the Effects of Alpha-Linolenic Acid Against Visceral Fat: A Double-Blind Randomized Controlled Trial. To investigate the effect of alpha-linolenic acid-rich diacylglycerol (ALA-DAG) compared with alpha-linolenic acid-rich triacylglycerol (ALA-TAG) on visceral fat area (VFA) in people with overweight.Subjects with overweight were recruited to a randomized, double-blind, controlled, parallel-group designed trial and randomly allocated to two groups that consumed either 2.5

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2017 Obesity

159. Alpha-lipoic acid supplement in obesity treatment: A systematic review and meta-analysis of clinical trials. (PubMed)

Alpha-lipoic acid supplement in obesity treatment: A systematic review and meta-analysis of clinical trials. Previous studies have supported positive roles of antioxidant supplements on weight-loss. One antioxidant supplement is Alpha-lipoic acid. However, recommending ALA as an anti-obesity supplement remains controversial. Accordingly, the purpose of the present study was to perform a meta-analysis on the effects of ALA supplement on anthropometric indices among adult subjects.We searched

2017 Clinical nutrition (Edinburgh, Scotland)

160. Dietary fatty acid intake after myocardial infarction: a theoretical substitution analysis of the Alpha Omega Cohort. (PubMed)

Dietary fatty acid intake after myocardial infarction: a theoretical substitution analysis of the Alpha Omega Cohort. Replacement of saturated fatty acids (SFAs) with unsaturated fatty acids (UFAs), especially polyunsaturated fatty acids (PUFAs), has been associated with a lower risk of ischemic heart disease (IHD). Whether this replacement is beneficial for drug-treated patients with cardiac disease is not yet clear.In a prospective study of Dutch patients with cardiac disease (Alpha Omega (...) Cohort), we examined the risk of cardiovascular disease (CVD) and IHD mortality when the sum of SFAs and trans fatty acids (TFAs) was theoretically replaced by total UFAs, PUFAs, or cis monounsaturated fatty acids (MUFAs).We included 4146 state-of-the-art drug-treated patients aged 60-80 y with a history of myocardial infarction (79% male patients) and reliable dietary data at baseline (2002-2006). Cause-specific mortality was monitored until 1 January 2013. HRs for CVD mortality and IHD mortality

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2017 American Journal of Clinical Nutrition

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