Genomes and Genes
Gene Symbol: HSD17B10
Description: hydroxysteroid (17-beta) dehydrogenase 10
Alias: 17b-HSD10, ABAD, CAMR, DUPXp11.22, ERAB, HADH2, HCD2, MHBD, MRPP2, MRX17, MRX31, MRXS10, SCHAD, SDR5C1, 3-hydroxy-2-methylbutyryl-CoA dehydrogenase, 3-hydroxyacyl-CoA dehydrogenase type-2, AB-binding alcohol dehydrogenase, amyloid-beta peptide binding alcohol dehydrogenase, endoplasmic reticulum-associated amyloid beta-peptide-binding protein, mitochondrial RNase P subunit 2, mitochondrial ribonuclease P protein 2, short chain L-3-hydroxyacyl-CoA dehydrogenase type 2, short chain dehydrogenase/reductase family 5C, member 1, short chain type dehydrogenase/reductase XH98G2
Publications142 found, 100 shown here
- Human short-chain L-3-hydroxyacyl-CoA dehydrogenase: cloning and characterization of the coding sequenceP J Vredendaal
Department for Metabolic Diseases, Wilhelmina Childrens Hospital, Utrecht, The Netherlands
Biochem Biophys Res Commun 223:718-23. 1996The cDNA encompassing the complete coding sequence of human liver short-chain L-3-hydroxyacyl-CoA dehydrogenase (SCHAD) was isolated and characterized...
- ABAD directly links Abeta to mitochondrial toxicity in Alzheimer's diseaseJoyce W Lustbader
Center for Reproductive Sciences and Department of Obstetrics and Gynecology, College of Physicians and Surgeons, Columbia University, 630 West 168th Street, New York, NY 10032, USA
Science 304:448-52. 2004..Here, we demonstrate that Abeta-binding alcohol dehydrogenase (ABAD) is a direct molecular link from Abeta to mitochondrial toxicity...
- An intracellular protein that binds amyloid-beta peptide and mediates neurotoxicity in Alzheimer's diseaseS D Yan
Department of Pathology, Columbia University, College of Physicians and Surgeons, New York, New York 10032, USA
Nature 389:689-95. 1997..It binds an intracellular polypeptide known as ERAB, thought to be a hydroxysteroid dehydrogenase enzyme, which is expressed in normal tissues, but is overexpressed in ..
- Behavioral stress causes mitochondrial dysfunction via ABAD up-regulation and aggravates plaque pathology in the brain of a mouse model of Alzheimer diseaseJi Seon Seo
Department of Brain and Cognitive Sciences, Ewha Women s University, Seoul 120 750, Republic of Korea
Free Radic Biol Med 50:1526-35. 2011..corticosterone) and Aβ in SH-SY5Y cells increased the expression of 17β-hydroxysteroid dehydrogenase (ABAD), mitochondrial dysfunction, and levels of ROS, whereas blockade of ABAD expression by siRNA-ABAD in SH-SY5Y cells ..
- HSD17B10: a gene involved in cognitive function through metabolism of isoleucine and neuroactive steroidsSong Yu Yang
Department of Neurochemistry, New York State Institute for Basic Research in Developmental Disabilities, 1050 Forest Hill Road, Staten Island, NY 10314, USA
Mol Genet Metab 92:36-42. 2007The HSD17B10 gene maps on chromosome Xp11.2, a region highly associated with X-linked mental retardation...
- Enhanced levels of mitochondrial enzyme 17beta-hydroxysteroid dehydrogenase type 10 in patients with Alzheimer disease and multiple sclerosisZdena Kristofikova
Alzheimer Disease Center, Prague Psychiatric Centre, Ustavni 91, 181 03 Prague 8, Bohnice, Czech Republic
Mol Biosyst 5:1174-9. 2009..In patients with multiple sclerosis, our results are the first to demonstrate significant changes in enzyme expression and to suggest possible alterations in amyloid beta peptides...
- A human brain L-3-hydroxyacyl-coenzyme A dehydrogenase is identical to an amyloid beta-peptide-binding protein involved in Alzheimer's diseaseX Y He
Department of Pharmacology, New York State Institute for Basic Research in Developmental Disabilities, Staten Island, New York 10314, USA
J Biol Chem 273:10741-6. 1998..of this human brain enzyme is identical to that of an endoplasmic reticulum amyloid beta-peptide-binding protein (ERAB), which mediates neurotoxicity in Alzheimer's disease (Yan, S. D., Fu, J., Soto, C., Chen, X., Zhu, H...
- Binding of amyloid beta-peptide to mitochondrial hydroxyacyl-CoA dehydrogenase (ERAB): regulation of an SDR enzyme activity with implications for apoptosis in Alzheimer's diseaseU C Oppermann
Department of Medical Biochemistry and Biophysics, Karolinska Institutet, Stockholm, Sweden
FEBS Lett 451:238-42. 1999The intracellular amyloid beta-peptide (A beta) binding protein, ERAB, a member of the short-chain dehydrogenase/reductase (SDR) family, is known to mediate apoptosis in different cell lines and to be a class II hydroxyacyl-CoA ..
- Genetic polymorphisms of 17 β-hydroxysteroid dehydrogenase 3 and the risk of hypospadiasFumihiro Sata
Department of Public Health, Hokkaido University Graduate School of Medicine, Sapporo, Japan
J Sex Med 7:2729-38. 2010..In this regard, enzymes 17 β-hydroxysteroid dehydrogenase type 3 (17 β HSD3, encoded by HSD17B3) and steroid 5 α-reductase type 2 (encoded by SRD5A2) play crucial roles...
- Type 10 17β-hydroxysteroid dehydrogenase expression is regulated by C/EBPβ in HepG2 cellsMirja Rotinen
Department of Health Sciences, Universidad Publica de Navarra, Avda Barañain, 31008 Pamplona, Spain
J Steroid Biochem Mol Biol 122:164-71. 2010..C/EBPα or C/EBPβ in HepG2 cells showed that HSD17B11 expression was induced by both transcription factors while HSD17B10 expression was only induced by C/EBPβ...
- X-inactivation of HSD17B10 revealed by cDNA analysis in two female patients with 17β-hydroxysteroid dehydrogenase 10 deficiencyJudit Garcia-Villoria
Sección de Errores Congénitos del Metabolismo IBC, Servicio de Bioquímica y Genética Molecular, Hospital Clinic, IDIBAPS, Barcelona, Spain
Eur J Hum Genet 18:1353-5. 2010..The gene encoding HSD10, HSD17B10, has been reported as one of the few genes that escapes X-inactivation...
- Expanded substrate screenings of human and Drosophila type 10 17beta-hydroxysteroid dehydrogenases (HSDs) reveal multiple specificities in bile acid and steroid hormone metabolism: characterization of multifunctional 3alpha/7alpha/7beta/17beta/20beta/21-HNaeem Shafqat
Department of Medical Biochemistry and Biophysics, Karolinska Institutet, SE 171 77 Stockholm, Sweden
Biochem J 376:49-60. 2003....
- A 5-methylcytosine hotspot responsible for the prevalent HSD17B10 mutationSong Yu Yang
Department of Developmental Biochemistry, NYS Institute for Basic Research in Developmental Disabilities, Staten Island, NY 10314, USA
Gene 515:380-4. 2013..hydroxysteroid (17β) dehydrogenase X (HSD10) deficiency are due to a missense C>T mutation in exon 4 of the HSD17B10 gene. The resulting HSD10 (p...
- Oxidative 3alpha-hydroxysteroid dehydrogenase activity of human type 10 17beta-hydroxysteroid dehydrogenaseXue Ying He
Department of Pharmacology, New York State Institute for Basic Research in Developmental Disabilities, Staten Island, NY 10314, USA
J Steroid Biochem Mol Biol 87:191-8. 2003..The experimental results lead to the conclusion that mitochondrial 17beta-HSD10 plays a significant part in a non-classical androgen synthesis pathway along with microsomal retinol dehydrogenases...
- 2-Methyl-3-hydroxybutyryl-CoA dehydrogenase deficiency is caused by mutations in the HADH2 geneRob Ofman
Department of Clinical Chemistry, Academic Medical Center, Emma Children s Hospital, University of Amsterdam, The Netherlands
Am J Hum Genet 72:1300-7. 2003..This led to the identification of the X-chromosomal gene involved, which previously had been denoted "HADH2." Sequence analysis of the HADH2 gene from patients with MHBD deficiency revealed the presence of two missense ..
- 2-Methyl-3-hydroxybutyryl-CoA dehydrogenase (MHBD) deficiency: an X-linked inborn error of isoleucine metabolism that may mimic a mitochondrial diseaseCelia Perez-Cerda
Institut de Bioquímica, Corporaciò Sanitària Cláinic, Edifici Helios III, 08028 Barcelona, Spain
Pediatr Res 58:488-91. 2005We describe three patients, from two Spanish families, with 2-methyl-3-hydroxybutyryl-CoA dehydrogenase (MHBD) deficiency, a recently described X-linked neurodegenerative inborn error of isoleucine metabolism...
- Abundant type 10 17 beta-hydroxysteroid dehydrogenase in the hippocampus of mouse Alzheimer's disease modelXue Ying He
Department of Pharmacology, New York State Institute for Basic Research in Developmental Disabilities, Staten Island, NY 10314, USA
Brain Res Mol Brain Res 99:46-53. 2002..reticulum as suggested by its trivial name 'endoplasmic reticulum-associated amyloid-beta biding protein (ERAB)'...
- Mental retardation linked to mutations in the HSD17B10 gene interfering with neurosteroid and isoleucine metabolismSong Yu Yang
Department of Neurochemistry, New York State Institute for Basic Research in Developmental Disabilities, Staten Island, NY 10314, USA
Proc Natl Acad Sci U S A 106:14820-4. 2009Mutations in the HSD17B10 gene were identified in two previously described mentally retarded males. A point mutation c.776G>C was found from a survivor (SV), whereas a potent mutation, c...
- Characterization and localization of human type10 17beta-hydroxysteroid dehydrogenaseX Y He
Department of Pharmacology, New York State Institute for Basic Research in Developmental Disabilities, New York 10314, USA
Eur J Biochem 268:4899-907. 2001..studies demonstrate that this protein, which has been referred to as ER-associated amyloid beta-binding protein (ERAB), is not detectable in the ER of normal tissues...
- A non-enzymatic function of 17beta-hydroxysteroid dehydrogenase type 10 is required for mitochondrial integrity and cell survivalKatharina Rauschenberger
Institute of Human Genetics, Heidelberg University, Heidelberg, Germany
EMBO Mol Med 2:51-62. 2010..The disease-causing gene is HSD17B10 and encodes 17beta-hydroxysteroid dehydrogenase type 10 (HSD10), a protein also implicated in the pathogenesis of ..
- Role of ERAB/L-3-hydroxyacyl-coenzyme A dehydrogenase type II activity in Abeta-induced cytotoxicityS D Yan
Departments of Pathology, Physiology and Surgery, College of Physicians and Surgeons of Columbia University, New York, New York 10032, USA
J Biol Chem 274:2145-56. 1999Endoplasmic reticulum-associated amyloid beta-peptide (Abeta)-binding protein (ERAB)/L-3-hydroxyacyl-CoA dehydrogenase type II (HADH II) is expressed at high levels in Alzheimer's disease (AD)-affected brain, binds Abeta, and contributes ..
- Intracellular oxidation of allopregnanolone by human brain type 10 17beta-hydroxysteroid dehydrogenaseXue Ying He
Department of Neurochemistry, New York State Institute for Basic Research in Developmental Disabilities, Staten Island, NY 10314, USA
Brain Res 1040:29-35. 2005..The elevated level of 17beta-HSD10 in activated astrocytes is a new feature found in brains of people with AD, and it may have important impact on AD pathogenesis...
- ABAD enhances Abeta-induced cell stress via mitochondrial dysfunctionKazuhiro Takuma
Departments of Surgery, Pathology, and Neurology, College of Physicians and Surgeons, Columbia University, New York, New York 10032, USA
FASEB J 19:597-8. 2005Amyloid-beta peptide (Abeta) binding alcohol dehydrogenase (ABAD), an enzyme present in neuronal mitochondria, is a cofactor facilitating Abeta-induced cell stress...
- 3-Hydroxyacyl-CoA dehydrogenase and short chain 3-hydroxyacyl-CoA dehydrogenase in human health and diseaseSong Yu Yang
Department of Neurochemistry, New York State Institute for Basic Research in Developmental Disabilities, Staten Island, 10314, USA
FEBS J 272:4874-83. 2005..HAD has a preference for medium chain substrates, whereas short chain 3-hydroxyacyl-CoA dehydrogenase (SCHAD) acts on a wide spectrum of substrates, including steroids, cholic acids, and fatty acids, with a preference for ..
- Crystal structure of human ABAD/HSD10 with a bound inhibitor: implications for design of Alzheimer's disease therapeuticsCharles R Kissinger
Pfizer La Jolla, 10777 Science Center Dr, San Diego, CA 92121, USA
J Mol Biol 342:943-52. 2004..dehydrogenase type 10 (HSD10), also known as amyloid beta-peptide-binding alcohol dehydrogenase (ABAD), has been implicated in the development of Alzheimer's disease...
- Human brain short chain L-3-hydroxyacyl coenzyme A dehydrogenase is a single-domain multifunctional enzyme. Characterization of a novel 17beta-hydroxysteroid dehydrogenaseX Y He
Departments of Pharmacology, New York State Institute for Basic Research in Developmental Disabilities, Staten Island, New York 10314, USA
J Biol Chem 274:15014-9. 1999Human brain short chain L-3-hydroxyacyl-CoA dehydrogenase (SCHAD) was found to catalyze the oxidation of 17beta-estradiol and dihydroandrosterone as well as alcohols...
- Comparative evolutionary genomics of the HADH2 gene encoding Abeta-binding alcohol dehydrogenase/17beta-hydroxysteroid dehydrogenase type 10 (ABAD/HSD10)Alexandra T Marques
REQUIMTE, Departamento de Quimica, Faculdade de Ciencias, Universidade do Porto, Rua do Campo Alegre, 687, 4169 007 Porto, Portugal
BMC Genomics 7:202. 2006..Here we use comparative genomic analyses to study the evolution of the HADH2 gene encoding ABAD/HSD10 across several eukaryotic species.
- Estrogen receptor alpha interacts with 17beta-hydroxysteroid dehydrogenase type 10 in mitochondriaVirginija Jazbutyte
Laboratory of Molecular Cardiology, Department of Medicine, University Clinics Wuerzburg, Josef Schneider Strasse 2, D20, 97080 Wuerzburg, Germany
Biochem Biophys Res Commun 384:450-4. 2009..GST pull-down experiments confirmed the interaction of ERalpha and 17beta-HSD10. These findings suggest that the ERalpha estrogen receptor might be involved in regulating intracellular estrogen levels by modulating 17beta-HSD10 activity...
- Chromosomal basis of X chromosome inactivation: identification of a multigene domain in Xp11.21-p11.22 that escapes X inactivationA P Miller
Department of Genetics and Center for Human Genetics, Case Western Reserve University School of Medicine and University Hospitals of Cleveland, Cleveland, OH 44106, USA
Proc Natl Acad Sci U S A 95:8709-14. 1998..Genes located both distal and proximal to this cluster are subject to inactivation, thereby defining a unique multigene domain on the proximal short arm that is transcriptionally active on the inactive X chromosome...
- The reduced expression of the HADH2 protein causes X-linked mental retardation, choreoathetosis, and abnormal behaviorClaus Lenski
Department of Obstetrics and Gynecology, Technical University, Munich, Germany
Am J Hum Genet 80:372-7. 2007..with a unique clinical phenotype characterized by mild mental retardation, choreoathetosis, and abnormal behavior (MRXS10). Linkage analysis in this family revealed a candidate region of 13...
- Cloning and expression of cDNA for a newly identified isozyme of bovine liver 3-hydroxyacyl-CoA dehydrogenase and its import into mitochondriaS Furuta
Department of Biochemistry, Shinshu University School of Medicine, Nagano, Japan
Biochim Biophys Acta 1350:317-24. 1997....
- Recognition of structurally diverse substrates by type II 3-hydroxyacyl-CoA dehydrogenase (HADH II)/amyloid-beta binding alcohol dehydrogenase (ABAD)A J Powell
Department of Biochemistry, University of Bristol, Bristol, UK
J Mol Biol 303:311-27. 2000Human type II hydroxyacyl-CoA dehydrogenase/amyloid-beta binding alcohol dehydrogenase (HADH II/ABAD) is an oxidoreductase whose salient features include broad substrate specificity, encompassing 3-hydroxyacyl-CoA derivatives, ..
- Tobacco streak virus isolated from lettuceF S Abtahi
Department of Plant Protection, Collage of Agriculture, University of Tehran, Karaj, Islamic Republic of Iran
Pak J Biol Sci 12:707-11. 2009..Five isolates (T1, T2, T3, T4 and T5), which are collected, respectively from Mohammad Abad (Karaj), Malek Abad (Karaj), Hashtgerd (Karaj), Tarand Balla (Varamin) and Deh mah sin (Pishva) were inoculated on ..
- Framing access to medicines in developing countries: an analysis of media coverage of Canada's Access to Medicines RegimeLaura C Esmail
Leslie Dan Faculty of Pharmacy, University of Toronto, 144 College Street, Toronto, Ontario, Canada
BMC Int Health Hum Rights 10:1. 2010..Over the course of eight months, the legislation, now known as Canada's Access to Medicines Regime (CAMR), went through a controversial policy development process and the newspaper media was one of the major venues in ..
- Prevalence of human papillomavirus genotypes in cytologic abnormalities from unvaccinated women living in north-western SpainAna Pastora Otero-Motta
Department of Pathology, Hospital Universitario de Salamanca, Spain
APMIS 119:204-15. 2011..0001). The implementation of bivalent prophylactic vaccination could potentially lead to prevention in 32% of the population included in the study - in at least a quarter of patients with ACUS/LSIL (26.7%), and in half of HSIL (50.2%)...
- Effect of sowing dates on yield and yield components of spring safflower (Carthamus tinctorius L.) in Isfahan regionS Nikabadi
Department of Agronomy, Faculty of Agriculture, Islamic Azad University, Branch of Khorasgan, Isfahan, Iran
Pak J Biol Sci 11:1953-6. 2008..of two safflower varieties, an experiment was conducted at Agriculture Research Station of Isfahan Kabotar Abad in 2004...
- The benefits and costs of reducing emissions from the electricity sectorKaren Palmer
Resources for the Future, 1616 P Street, NW, Washington, DC 20036, USA
J Environ Manage 83:115-30. 2007..Rule (CAIR) as characterized in the supplemental rule proposed in June 2004, and the Clean Air Mercury Rule (CAMR) as proposed in February 2004...
- Dominantly inherited hyperinsulinaemic hypoglycaemiaP de Lonlay
INSERM U393 and Department of Pediatrics, Hopital Necker Enfants Malades, Paris, France
J Inherit Metab Dis 28:267-76. 2005..dominantly inherited HI; (ii) metabolic disorders implicating the short-chain L-3-hydroxyacyl-CoA dehydrogenase (SCHAD) enzyme inrecessively inherited HI, the glucokinase gene (GK), the glutamate dehydrogenase gene (GLUD1) when ..
- Regional localisation of two non-specific X-linked mental retardation genes (MRX30 and MRX31)A J Donnelly
Department of Cytogenetics and Molecular Genetics, Women s and Children s Hospital, Adelaide, Australia
Am J Med Genet 64:113-20. 1996..3) and DXS424 (Xq24). A significant multipoint lod score of 2.78 was detected between the loci DXS1120 and DXS456. MRX31 maps to a 12 cM region that spans the centromere from DXS1126 (Xp11.23) to DXS1124 (Xq13.3)...
- Medicaid managed care: are academic medical centers penalized by attracting patients with high-cost conditions?Michele Heisler
The Robert Wood Johnson Clinical Scholars Program, University of Michigan School of Medicine, Ann Arbor, Mich, USA
Am J Manag Care 9:19-29. 2003....
- Indoor radon levels and lung cancer risk estimates in seven cities of the Bahawalpur Division, Pakistan- Matiullah
Pakistan Institute of Engineering and Applied Sciences, P O Nilore, Islamabad, Pakistan
Radiat Prot Dosimetry 107:269-76. 2003..These included Fort Abbas, Minchin Abad, Hasilpur, Bahawalpur, Liaqatpur, Rahimyar Khan and Sadiq Abad...
- Variants of smooth Salmonella enterica serovar Enteritidis that grow to higher cell density than the wild type are more virulentJ Guard-Petter
Agricultural Research Service, United States Department of Agriculture, Athens, Georgia 30605, USA
Appl Environ Microbiol 64:2166-72. 1998..Whereas SE-HCD1 killed 70% of 6-day-old chicks challenged subcutaneously, the same dose of SE-HCD2 did not kill any chicks...
- Transcription of the cam operon and camR genes in Pseudomonas putida PpG1M Fujita
Department of Biotechnology, Faculty of Engineering, Fukuyama University, Hiroshima, Japan
J Bacteriol 175:6953-8. 1993..operon (camDCAB) encoding enzymes involved in the degradation pathway of D-camphor is negatively regulated by the CamR protein, and camR is autorepressed...
- Reye-like syndrome resulting from novel missense mutations in mitochondrial medium- and short-chain l-3-hydroxy-acyl-CoA dehydrogenaseMichael J Bennett
Department of Pathology and Laboratory Medicine, University of Pennsylvania, Philadelphia, PA, USA
Mol Genet Metab 89:74-9. 2006Medium- and short-chain l-3-hydroxy-acyl-CoA dehydrogenase (M/SCHAD) deficiency is a recessively inherited disorder of fatty acid oxidation. Currently, only four patients from three families have been reported in the literature...
- Soil contamination with Toxocara spp. eggs in the public parks from three areas of Khorram Abad, IranM Zibaei
Department of Parasitology, School of Medicine, Lorestan University of Medical Sciences, Khorram Abad, Iran
Nepal Med Coll J 12:63-5. 2010..The present investigation clearly shows that public parks have been contaminated with Toxocara eggs in Khorram Abad, suggesting that care should be taken when using public parks.
- Crystal structure of the TetR/CamR family repressor Mycobacterium tuberculosis EthR implicated in ethionamide resistanceLynn G Dover
School of Biosciences, The University of Birmingham, Edgbaston, Birmingham B15 2TT, UK
J Mol Biol 340:1095-105. 2004..Activation of the pro-drug ethionamide is regulated by the Baeyer-Villiger monooxygenase EthA and the TetR/CamR family repressor EthR, whose open reading frames are separated by 75 bp on the Mycobacterium tuberculosis genome...
- Isfahan Healthy Heart Programme: a comprehensive integrated community-based programme for cardiovascular disease prevention and control. Design, methods and initial experienceNizal Sarraf-Zadegan
Isfahan Cardiovascular Center, Isfahan University of Medical Sciences, Isfahan, Iran
Acta Cardiol 58:309-20. 2003..A primary survey was done to collect baseline data from interventional (Isfahan and Najaf-Abad) and reference (Arak) communities...
- Deletion of new covalently linked cell wall glycoproteins alters the electrophoretic mobility of phosphorylated wall components of Saccharomyces cerevisiaeV Mrsa
Lehrstuhl für Zellbiologie und Pflanzenphysiologie, Universitat Regensburg, 93040 Regensburg, Germany
J Bacteriol 181:3076-86. 1999..Moukadiri, J. Armero, A. Abad, R. Sentandreu, and J. Zueco, J. Bacteriol. 179:2154-2162, 1997)...
- Metronidazole resistance in Helicobacter pylori is due to null mutations in a gene (rdxA) that encodes an oxygen-insensitive NADPH nitroreductaseA Goodwin
Department of Microbiology and Immunology, Faculty of Medicine, Dalhousie University, Halifax, Nova Scotia, Canada
Mol Microbiol 28:383-93. 1998..pylori rendered it MtzS; and (iii) replacement of rdxA in MtzS H. pylori with an rdxA::camR null insertion allele resulted in a MtzR phenotype. The 630 bp rdxA genes of five pairs of H...
- Evidence that 2-methylacetoacetate induces oxidative stress in rat brainGuilhian Leipnitz
Departamento de Bioquimica, Instituto de Ciencias Basicas da Saude, Universidade Federal de Rio Grande do Sul, Rua Ramiro Barcelos, Anexo, Porto Alegre, RS, Brazil
Metab Brain Dis 25:261-7. 2010..in mitochondrial 2-methylacetoacetyl-CoA thiolase (KT) and 2-methyl-3-hydroxybutyryl-CoA dehydrogenase (MHBD) deficiencies, on important parameters of oxidative stress in cerebral cortex from young rats...
- Fluorodeoxyglucose positron emission tomography for the diagnosis of sarcoidosis in patients with unexplained chronic uveitisPascal Seve
Department of Internal Medicine, Hotel Dieu, Hospices Civils de Lyon, Lyon, France
Ocul Immunol Inflamm 17:179-84. 2009..To assess the value of fluorine-18 fluorodeoxyglucose positron emission tomography ((18)F-FDG PET) in patients with unexplained chronic uveitis...
- Lindane residues in cultivated cucumber and in the most consumed fish in Caspian Sea (Iran)M Shokrzadeh
Department of Toxicology, Faculty of Pharmacy, Mazandaran University of Medical Sciences, Sari, Iran
Toxicol Ind Health 25:517-23. 2009..of fish were caught using electric fishing from four major fishing centers (Chalous and Babolsar cities, Khazar Abad and Miankaleh regions) in Mazandaran province of Iran...
- Panel: Canada's law on global access to affordable medicinesTenu Avafia
United Nations Development Programme
HIV AIDS Policy Law Rev 14:76-83. 2009..agreements concerning intellectual property rights, which formed the basis of Canada's Access to Medicines Regime (CAMR)...
- New assessment of relationship between Apgar score and early neonatal mortalityS Vahabi
Department Anesthesiology, Lorestan University of Medical Sciences, Khoramabad, Iran
Minerva Pediatr 62:249-52. 2010..We designed this study to examine the original intent of Apgar system to predict neonatal survival during almost 50 years of its application...
- Do lifestyle interventions work in developing countries? Findings from the Isfahan Healthy Heart Program in the Islamic Republic of IranNizal Sarrafzadegan
Cardiovascular Research Center, Isfahan University of Medical Sciences, Isfahan, Islamic Republic of Iran
Bull World Health Organ 87:39-50. 2009..To assess the effects of a comprehensive, integrated community-based lifestyle intervention on diet, physical activity and smoking in two Iranian communities...
- An overview of beta-oxidation disordersDariusz Moczulski
Katedra Medycyny Wewnetrznej, Uniwersytet Medyczny w Łodzi, Łódź
Postepy Hig Med Dosw (Online) 63:266-77. 2009..on disorders associated with fatty-acid oxidation: deficiencies of beta-oxidation enzymes, namely VLCAD, TFP and LCHAD, MCAD, MCKAT, M/SCHAD, and SCAD, and deficiencies of the enzymes TCP I, CT, and CPT II of the carnitine cycle.
- Significant association between chronic antibody-mediated rejection and donor-specific antibodies against HLA-DRB rather than DQB in renal transplantationTakaaki Kobayashi
Department of Applied Immunology, Nagoya University School of Medicine, Nagoya, Japan
Hum Immunol 72:11-7. 2011..production of antidonor HLA antibody has been reported to be associated with chronic antibody-mediated rejection (CAMR). However, some donor-specific antibodies (DSA) do not seem to cause graft injury...
- Characteristics of pediatric and adolescent patients attending a naturopathic college clinic in CanadaKumanan Wilson
Department of Medicine, University of Toronto, Toronto, Ontario, Canada
Pediatrics 115:e338-43. 2005..We also determined factors associated with the use of CAM products and vaccination status...
- Mitochondrial fatty acid beta-oxidation in the human eye and brain: implications for the retinopathy of long-chain 3-hydroxyacyl-CoA dehydrogenase deficiencyTiina Tyni
Department of Pediatric Neurology, Hospital for Children and Adolescents, Helsinki University Central Hospital, 00029 HUS, Helsinki, Finland
Pediatr Res 56:744-50. 2004..MCAD) and very long-chain acyl-CoA dehydrogenase (VLCAD), short-chain 3-hydroxyacyl-CoA dehydrogenase (SCHAD), and mitochondrial trifunctional protein (MTP) harboring LCHAD...
- [The state of mental health primary care in the social services of El Alto, La Paz, Bolivia]Carmen Camacho-Arce
Instituto de Investigación de Psicología, Universidad Mayor de San Andres, La Paz, Bolivia
Rev Panam Salud Publica 25:511-7. 2009To determine the mental health and behavioral disorders (MHBD) treated by a Bolivian health network; and to gauge the perspective of the health professionals, community members, and care-providers in order to identify ways to better ..
- Familial hyperinsulinemic hypoglycemia caused by a defect in the SCHAD enzyme of mitochondrial fatty acid oxidationAnders Molven
Center for Medical Genetics and Molecular Medicine, Haukeland University Hospital, Bergen, Norway
Diabetes 53:221-7. 2004..infant was recently found to have a mutation in the gene encoding short-chain 3-hydroxyacyl-CoA dehydrogenase (SCHAD), an enzyme participating in mitochondrial fatty acid oxidation...
- Morphological characterisation of Paranoplocephala bairdi (Schad, 1954) (Cestoda: Anoplocephalidae) in heather voles Phenacomys spp. and tree voles Arborimus spp., and related species in voles and lemmings (Muridae: Arvicolinae)Voitto Haukisalmi
Finnish Forest Research Institute, Vantaa Research Centre, P O Box 18, FIN 01301 Vantaa, Finland
Folia Parasitol (Praha) 52:311-21. 2005The taxonomical status of Paranoplocephala bairdi (Schad, 1954)-like cestodes (Anoplocephalidae) in heather voles Phenacomys spp. and tree voles Arborimus spp...
- Replication allows inactivation of a knocked-in locus control region in inappropriate cell lineagesUrsula Menzel
Division of Molecular Immunology, National Institute for Medical Research, Medical Research Council, London NW7 1AA, UK
Proc Natl Acad Sci U S A 107:16928-33. 2010..region (LCR) on the expression of a highly characterized, developmentally regulated locus, we have targeted the hCD2-LCR as a single copy into the endogenous mouse CD8 gene complex...
- Prevalence of Cryptosporidium infection in camels (Camelus dromedarius) in a slaughterhouse in IranS M Razawi
Department of Parasitology, Veterinary School, Shiraz University, Shiraz, Iran
Trop Biomed 26:267-73. 2009..dromedarius) from Najaf-Abad slaughterhouse, Isfahan Province, central part of Iran...
- The closed/open model for lipase activation. Addressing intermediate active forms of fungal enzymes by trapping of conformers in water-restricted environmentsH Gonzalez-Navarro
Departament de Bioquimica i Biologia Molecular, Facultat de Biologia, Universitat de Valencia, E 46100 Burjassot, Spain
Biochemistry 40:3174-83. 2001..b>Abad, C., and Braco, L. (1995) Proc. Natl. Acad. Sci. U.S.A. 92, 3308-3312]...
- Study of patients and carriers with 2-methyl-3-hydroxybutyryl-CoA dehydrogenase (MHBD) deficiency: difficulties in the diagnosisJudit Garcia-Villoria
Sección de Errores Congénitos del Metabolismo IBC, Servicio de Bioquímica y Genética Molecular, and CIBER de Enfermedades Raras, Hospital Clinic, Barcelona, Spain
Clin Biochem 42:27-33. 2009To search for biochemical and molecular markers for the diagnosis of patients and carriers with 2-Methyl-3-hydroxybutyryl-CoA dehydrogenase (MHBD) deficiency.
- Regulatory, effector, and cytotoxic T cell profiles in long-term kidney transplant patientsJoanna Ashton-Chess
INSERM, U643, Institut de Transplantation et de Recherche en Transplantation, Centre Hospitalier Universitaire de Nantes, Nantes, France
J Am Soc Nephrol 20:1113-22. 2009..the graft and significantly decreased in the peripheral blood of patients with chronic antibody-mediated rejection (CAMR). Quantifying peripheral blood GrzB mRNA demonstrated potential to aid in the noninvasive diagnosis of CAMR...
- Maternal diabetes adversely affects AMP-activated protein kinase activity and cellular metabolism in murine oocytesAnn M Ratchford
Department of Obstetrics and Gynecology, Washington University in St Louis, St Louis, MO 63110, USA
Am J Physiol Endocrinol Metab 293:E1198-206. 2007..enzyme activities of glucose-6-phosphate dehydrogenase (G6PDH), adenylate kinase, hydroxyacyl-CoA dehydrogenase (Hadh2), and glutamic pyruvate transaminase (Gpt2) were measured...
- Residues important for the function of a multihelical DNA binding domain in the new transcription factor family of Cam and Tet repressorsH Aramaki
Daiichi College of Pharmaceutical Sciences, Fukuoka, Japan
Protein Eng 8:1259-66. 1995We report that some prokaryotic repressors including CamR and TetR belong to the same family...
- Canada's Access to Medicines Regime: Promise or Failure of Humanitarian Effort?Jillian Clare Kohler
Assistant Professor, Leslie Dan Faculty of Pharmacy, University of Toronto, Toronto, ON
Healthc Policy 5:40-8. 2010..One example is Canada's Access to Medicines Regime (CAMR)...
- Muscle-specific adaptations, impaired oxidative capacity and maintenance of contractile function characterize diet-induced obese mouse skeletal muscleKarin E Shortreed
Department of Pathology and Molecular Medicine, McMaster University, Hamilton Ontario, Canada
PLoS ONE 4:e7293. 2009..The effects of diet-induced obesity on skeletal muscle function are largely unknown, particularly as it relates to changes in oxidative metabolism and morphology...
- A one-time-only combination: Emergency medicine exports and the TRIPS agreement under Canada's access to medicines regimeAshley Weber
Stikeman Elliott LLP inToronto
Health Hum Rights 12:109-22. 2010..The shipment was authorized under Canada's Access to Medicines Regime (CAMR), which implements the World Trade Organization (WTO) General Council Decision (the Decision), made in 2003, to ..
- Surface plasmon resonance and nuclear magnetic resonance studies of ABAD-Abeta interactionYiLin Yan
Department of Biology, Center for Biotechnology and Interdisciplinary Studies, Rensselaer Polytechnic Institute, Troy, New York 12180, USA
Biochemistry 46:1724-31. 2007Abeta binding alcohol dehydrogenase (ABAD) is an NAD-dependent mitochondrial dehydrogenase. The binding between ABAD and Abeta is likely a direct link between Abeta and mitochondrial toxicity in Alzheimer's disease...
- Mechanism of hyperinsulinism in short-chain 3-hydroxyacyl-CoA dehydrogenase deficiency involves activation of glutamate dehydrogenaseChanghong Li
Division of Endocrinology, The Children s Hospital of Philadelphia, Philadelphia, Pennsylvania 19104, USA
J Biol Chem 285:31806-18. 2010..with hyperinsulinism associated with inactivating mutations of short-chain 3-hydroxyacyl-CoA dehydrogenase (SCHAD) was examined in mice with a knock-out of the hadh gene (hadh(-/-))...
- Re: Hadh2 and 3-hydroxyacyl-CoA dehydrogenaseSong Yu Yang
Am J Physiol Endocrinol Metab 295:E987. 2008
- The hyperinsulinism/hyperammonemia syndromeAndrew A Palladino
The Children s Hospital of Philadelphia, Division of Endocrinology and Diabetes, University of Pennsylvania, School of Medicine, Philadelphia, PA 19104, USA
Rev Endocr Metab Disord 11:171-8. 2010..GDH has also been implicated in another form of HI, short-chain 3-hydroxyacyl-CoA dehydrogenase (SCHAD) deficiency associated HI...
- Advances in imaging of new targets for pharmacological intervention in stroke: real-time tracking of T-cells in the ischaemic brainF Ortolano
Centre for Biophotonics, University of Strathclyde, Glasgow, UK
Br J Pharmacol 159:808-11. 2010..We aim to extend the technique of real-time in situ brain imaging of T-cells, previously shown in models of immunological diseases, to models of experimental stroke...
- Pericentromeric genes for non-specific X-linked mental retardation (MRX)A Gedeon
Department of Cytogenetics and Molecular Genetics, Women s and Children s Hospital, Australia
Am J Med Genet 51:553-64. 1994..The MRX17 gene is localized with a peak lod of 2.41 (theta = 0...
- Number of entry tears is associated with aortic growth in type B dissectionsJip L Tolenaar
Thoracic Aortic Research Center, Policlinico San Donato Istituto di Ricovero e Cura a Carattere Scientifico, Milan, Italy
Ann Thorac Surg 96:39-42. 2013Aortic growth rate in acute type B aortic dissection (ABAD) is a significant predictor for aortic complications and death...
- Monogenic hyperinsulinemic hypoglycemia: current insights into the pathogenesis and managementKatherine Lord
Division of Endocrinology and Diabetes, The Children s Hospital of Philadelphia, 3615 Civic Center Boulevard, Abramson Research Center Room 802A, Philadelphia, PA, 19104, USA
Int J Pediatr Endocrinol 2013:3. 2013..More recently identified genetic causes include mutations in the genes encoding short-chain 3-hydroxyacyl-CoA (SCHAD), uncoupling protein 2 (UCP2), hepatocyte nuclear factor 4-alpha (HNF-4α), hepatocyte nuclear factor 1-alpha (HNF-..
- Is amyloid binding alcohol dehydrogenase a drug target for treating Alzheimer's disease?Eva Borger
School of Biology, Medical and Biological Sciences Building, North Haugh, University of St Andrews, Scotland UK KY16 9TF
Curr Alzheimer Res 10:21-9. 2013..the role of protecting the function of a specific mitochondrial protein, amyloid binding alcohol dehydrogenase (ABAD), will be the focus of this review...
- Short-chain 3-hydroxyacyl-coenzyme A dehydrogenase associates with a protein super-complex integrating multiple metabolic pathwaysSrinivas B Narayan
Department of Pathology and Laboratory Medicine, Children s Hospital of Philadelphia, Philadelphia, Pennsylvania, United States of America
PLoS ONE 7:e35048. 2012..We previously described an interaction between short-chain 3-hydroxyacyl-coenzyme A dehydrogenase (SCHAD) and glutamate dehydrogenase (GDH) explaining the clinical phenotype of hyperinsulinism in SCHAD-deficient ..
- Acute type B aortic dissection in the absence of aortic dilatationSanti Trimarchi
Thoracic Aortic Research Center, Policlinico San Donato IRCCS, Milan, Italy
J Vasc Surg 56:311-6. 2012Increasing aortic diameter is thought to be an important risk factor for acute type B aortic dissection (ABAD). However, some patients develop ABAD in the absence of aortic dilatation...
- Improved stable isotope dilution-gas chromatography-mass spectrometry method for serum or plasma free 3-hydroxy-fatty acids and its utility for the study of disorders of mitochondrial fatty acid beta-oxidationP M Jones
University of Texas Southwestern Medical Center, Department of Pathology, and Children s Medical Center of Dallas, TX 75235, USA
Clin Chem 46:149-55. 2000..in the blood are indicative of FAO disorders of the long- and short-chain 3-hydroxy-acyl-CoA dehydrogenases, LCHAD and SCHAD. We describe a serum/plasma assay for the measurement of 3-OH-FAs with carbon chain lengths from C(6) to C(16).
- First microscopical and molecular-based characterization of Leishmania major within naturally infected Phlebotomus salehi (Diptera; Psychodidae) in Fars province, southern IranM H Davami
Department of Medical Microbiology, School of Medicine, Jahrom University of Medical Sciences, Jahrom, Iran
Ann Trop Med Parasitol 105:485-91. 2011..They were caught from outdoors in five regions of Jahrom district including villages of Mousavieh, Ghotb-Abad, Heydar-Abad, Fath-Abad and Jahrom County. Eleven species of Phlebotomine (three Phlebotomus spp...
- Human HMG box transcription factor HBP1: a role in hCD2 LCR functionT Zhuma
Division of Molecular Immunology, National Institute for Medical Research, The Ridgeway, Mill Hill, London NW7 1AA, UK
EMBO J 18:6396-406. 1999The locus control region (LCR) of the human CD2 gene (hCD2) confers T cell-specific, copy-dependent and position-independent gene expression in transgenic mice...
- Putative GTPase GIMAP1 is critical for the development of mature B and T lymphocytesAmy Saunders
Immunology Programme, The Babraham Institute, Cambridge, UK
Blood 115:3249-57. 2010..Homozygous loss of this allele under the influence of the lymphoid-expressed hCD2-iCre recombinase transgene led to severe (> 85%) deficiency of mature T lymphocytes and, unexpectedly, of mature ..
- Inhibition of the mitochondrial enzyme ABAD restores the amyloid-β-mediated deregulation of estradiolYun An Lim
Alzheimer s and Parkinson s Disease Laboratory, Brain and Mind Research Institute, University of Sydney, Camperdown, New South Wales, Australia
PLoS ONE 6:e28887. 2011..reports indicate that Aβ may also interact directly with intracellular proteins such as the mitochondrial enzyme ABAD (Aβ binding alcohol dehydrogenase) in executing its toxic effects...
- Two genetic forms of hyperinsulinemic hypoglycemia caused by dysregulation of glutamate dehydrogenaseCharles A Stanley
University of Pennsylvania School of Medicine, Endocrinology Division, The Children s Hospital of Philadelphia, Philadelphia, PA 19026, United States
Neurochem Int 59:465-72. 2011..regulation by GTP and hyperinsulinism due to recessive deficiency of short-chain 3-hydroxy-acyl-CoA dehydrogenase (SCHAD, encoded by HADH1)...
- Inhibition of amyloid-beta (Abeta) peptide-binding alcohol dehydrogenase-Abeta interaction reduces Abeta accumulation and improves mitochondrial function in a mouse model of Alzheimer's diseaseJun Yao
Department of Pathology, Taub Institute for Research on Alzheimer Disease and the Aging Brain, Columbia University, New York, New York 10032, USA
J Neurosci 31:2313-20. 2011Amyloid-β (Aβ) peptide-binding alcohol dehydrogenase (ABAD), an enzyme present in neuronal mitochondria, exacerbates Aβ-induced cell stress...
- The Chehr Abad "Salt men" and the isotopic ecology of humans in ancient IranV Ramaroli
Department of Environmental Sciences, Università Cà Foscari, Calle Larga Santa Marta 2137, 30123 Venezia, Italy
Am J Phys Anthropol 143:343-54. 2010..We have carried out isotopic analysis (δ(13)C and δ(15)N) on five salt-preserved bodies from the salt mine at Chehr Abad, Iran, dating from the 4th C. BC through to the 4th C. AD...
- Short-chain 3-hydroxyacyl-CoA dehydrogenase deficiency: the clinical relevance of an early diagnosis and report of four new casesEsmeralda Martins
Unidade de Doenças Metabólicas, Hospital de Criancas Maria Pia, Rua da Boavista, 827, 4050 111 Porto, Portugal
J Inherit Metab Dis 34:835-42. 2011Short-chain 3-hydroxyacyl-CoA dehydrogenase (HADH, SCHAD) deficiency (OMIM #231530) represents a recently described disorder of mitochondrial fatty acid beta-oxidation, with less than ten cases described worldwide...
- Human high mobility group box transcription factor 1 affects thymocyte development and transgene variegationBelaid Sekkali
National Institute for Medical Research, Division of Molecular Immunology, London, United Kingdom
J Immunol 175:5203-12. 2005It has been shown previously that a human CD2 (hCD2) disabled locus control region (LCR) transgene is unable to establish an open chromatin configuration in all the T cells, and this leads to position effect variegation of the transgene...
- Application of sensitivity analysis for assessment of de-desertification alternatives in the central Iran by using Triantaphyllou methodMohammad Hassan Sadeghi Ravesh
Department of Environment, School of the Agriculture, Takestan Branch, Islamic Azad University, Takestan, Iran
Environ Monit Assess 179:31-46. 2011..select the optimal de-desertification alternatives based on the results of interviews with experts in Khezr Abad region, central Iran as the case study...
- Anopheline species composition in borderline of Iran-AzerbaijanM A Oshaghi
Department of Medical Entomology, School of Public Health, Tehran University of Medical Sciences, Tehran, Iran
Acta Trop 119:44-9. 2011..species composition, the malaria parasite species, and natural infectivity of mosquitoes distributed in Pars-Abad district, on the borderline of Azerbaijan in northwestern Iran...
- Summary of FDA antibody-mediated rejection workshopP Archdeacon
Division of Special Pathogen and Transplant Products, Office of Antimicrobial Products, Center for Drug Evaluation and Research, FDA, Silver Spring, MD, USA
Am J Transplant 11:896-906. 2011..Desensitization, acute AMR and chronic AMR (CAMR) were considered in the context of clinical trial design...
- Women have higher protein content of beta-oxidation enzymes in skeletal muscle than menAmy C Maher
Department of Medical Science, McMaster University, Hamilton, Ontario, Canada
PLoS ONE 5:e12025. 2010..05). There was no significant sex difference in the expression of short-chain hydroxyacyl-CoA dehydrogenase (SCHAD), or peroxisome proliferator activated receptor alpha (PPARalpha), or PPARgamma, genes potentially involved in the ..
- Combination of general and specific warm-ups improves leg-press one repetition maximum compared with specific warm-up in trained individualsCesar C C Abad
Bandeirantes University of São Paulo UNIBAN, Sao Paulo, Brazil
J Strength Cond Res 25:2242-5. 2011..Therefore, these results support the recommendations of the testing guidelines to perform a moderate intensity general warm-up in addition to the specific warm-up before maximum strength assessments...
- Modulation of the murine CD8 gene complex following the targeted integration of human CD2-locus control region sequencesUrsula Menzel
Division of Molecular Immunology, National Institute for Medical Research, Medical Research Council, London NW7 1AA, United Kingdom
J Immunol 187:3712-20. 2011The human CD2 (hCD2) locus control region (LCR) inserted in the mouse CD8 gene complex activates expression of the CD8 genes in T cell subsets in which the CD8 locus is normally silenced (e.g., CD4(+) single-positive T cells)...
- Woman abuse and pregnancy outcome among women in Khoram Abad, Islamic Republic of IranN Khodakarami
Shahid Beheshti Medical University, Reproductive Health Research Centre and Nurisng Midwifery School, Tehran, Islamic Republic of Iran
East Mediterr Health J 15:622-8. 2009..Given the high likelihood that a woman will access health care services during her pregnancy, physicians providing prenatal care are in a strategic position to screen for partner abuse...
- Acute type B aortic dissection in elderly patients: clinical features, outcomes, and simple risk stratification ruleRajendra H Mehta
Department of Cardiology, University of Michigan, Ann Arbor, Michigan 48105, USA
Ann Thorac Surg 77:1622-8; discussion 1629. 2004The clinical features and outcomes of elderly patients with acute type B aortic dissection (ABAD) are less well known...
- NMR STUDIES OF PROTEIN TARGET INTERACTIONSGerhard Wagner; Fiscal Year: 2002..T antigen that interact with retinoblastoma protein and Hsc70; and the interaction of the T-cell surface proteins hCD2 and HCD58...
- MOLECULAR BASIS OF A NEW FORM OF HYPERINSULINISMCHARLES ALFRED STANLEY; Fiscal Year: 2010..mutations of the mitochondrial fatty acid -oxidation enzyme, short-chain 3-hydroxy acyl-CoA dehydrogenase (SCHAD)...
- MITOCHONDRIAL SHORT CHAIN 3-OH-ACYL-COA DEHYDROGENASESArnold Strauss; Fiscal Year: 2003..This proposal focuses on two enzymes catalyzing the third step, the short chain 3-hydroxy-acyl-CoA dehydrogenases (SCHAD)...
- Cellular Cofactors, Neuronal Stress & Rescue, Aging & Alzheimer DiseaseShi Du Yan; Fiscal Year: 2010..The central theme of our Program Project is that specific, cell associated cofactors, RAGE (Receptor for AGEs) and ABAD (A ?binding alcohol dehydrogenase) are critical for concentrating the effects of low levels of A, relevant to early ..
- STRUCTURAL STUDIES OF LIPID/PROTEIN SYSTEMSLeonard Banaszak; Fiscal Year: 2002..beta-ketoacyl CoA reaction, humans have two different enzymes: one for short chain L-3-hydroxyacyl CoA substrates (SCHAD) and one for long chain metabolites (LCHAD). Preliminary x-ray data have identified the coenzyme site of SCHAD...
- ERAB, A BETA, NEUROTOXICITY AND ALZHEIMERS DISEASEShi Du Yan; Fiscal Year: 2003..We have identified a novel endoplasmic reticulum-associated Abeta binding protein termed ERAB which has properties of a b-hydroxyacyl-Coenzyme A dehydrogenase/short-chain alcohol dehydrogenase...