lysosomal acid lipase

Summary

Gene Symbol: lysosomal acid lipase
Description: lipase A, lysosomal acid, cholesterol esterase
Alias: CESD, LAL, acid cholesteryl ester hydrolase, cholesterol ester hydrolase, cholesteryl esterase, lysosomal acid lipase, lysosomal acid lipase/cholesteryl ester hydrolase, sterol esterase
Species: human

Top Publications

  1. ncbi Androgenic modulation of lipid metabolism at subcellular sites in cholestatic rats
    A Hossain
    Department of Physiology, Louisiana State University Medical Center, New Orleans
    Horm Metab Res 26:19-25. 1994
  2. ncbi Effects of antinutritional factors on protein digestibility and amino acid availability in foods
    G Sarwar Gilani
    Health Canada, Nutrition Research Division, Bureau of Nutritional Sciences, Banting Research Centre AL 2203 C, Tunney s Pasture, Ottawa, ON, K1A 0L2, Canada
    J AOAC Int 88:967-87. 2005
  3. ncbi Occupational stress, social support, and depression among black and white professional-managerial women
    M B Snapp
    Department of Sociology and Social Work, Memphis State University
    Women Health 18:41-79. 1992
  4. ncbi [Influence of alleles of apolipoprotein E on restenosis after coronary angioplasty in women]
    L Flork
    Service de cardiologie, CHU G-Montpied, Clermont-Ferrand
    Arch Mal Coeur Vaiss 91:1475-9. 1998
  5. ncbi [The effect of temperature on the growth and lipopolysaccharide production of gram-negative bacteria]
    C Weber Frick
    ,
    Zentralbl Bakteriol Mikrobiol Hyg B 187:56-69. 1988
  6. ncbi [The growth and lipopolysaccharide production of Escherichia coli and Pseudomonas aeruginosa]
    C Weber Frick
    ,
    Zentralbl Bakteriol Mikrobiol Hyg B 186:478-93. 1988
  7. ncbi The effects of the endothelin ETA receptor antagonist, FR 139317, on infarct size in a rabbit model of acute myocardial ischaemia and reperfusion
    L Mcmurdo
    William Harvey Research Institute, St Bartholomew s Hospital Medical College, London
    Br J Pharmacol 112:75-80. 1994
  8. ncbi Endotoxemia and acute-phase proteins in major abdominal surgery
    K Buttenschoen
    Department of General Surgery, University of Ulm, Steinhoevelstrasse 9, D 89075, Ulm, Germany
    Am J Surg 181:36-43. 2001
  9. ncbi Performance assessment of new multiplex probe assay for identification of mycobacteria
    E Tortoli
    Centro Regionale di Riferimento per la Diagnostica delle Micobatteriosi, Laboratorio di Microbiologia e Virologia, Ospedale di Careggi, Piastra del servizi, Viale Morgagni 85, 50134 Florence, Italy
    J Clin Microbiol 39:1079-84. 2001
  10. ncbi Lysosomal acid lipase mutations that determine phenotype in Wolman and cholesterol ester storage disease
    R A Anderson
    Department of Internal Medicine, Wake Forest University School of Medicine, Winston Salem, North Carolina, 27157, USA
    Mol Genet Metab 68:333-45. 1999

Research Grants

  1. Disordered Cellular Cholesterol Movement, Cell Death, and Multisystem Disease
    John M Dietschy; Fiscal Year: 2010
  2. Pathophysiology of PPARgamma in the lung
    Hong Du; Fiscal Year: 2010
  3. Pathophysiology of PPARgamma in the lung
    Hong Du; Fiscal Year: 2010
  4. Pathophysiology of PPARgamma in the lung
    Hong Du; Fiscal Year: 2009
  5. NEW METHODS FOR EVAL OF ORGANIC DUST AEROSOLS
    Stephen Reynolds; Fiscal Year: 2006
  6. PATHOGENESIS OF LYSOSOMAL ACID LIPASE DEFICIENCY
    Hong Du; Fiscal Year: 2001
  7. Cholesterol Ester Hydrolysis and Cholesterol Efflux
    Shobha Ghosh; Fiscal Year: 2006
  8. Macrophage-lipoprotein interactions
    Frederick R Maxfield; Fiscal Year: 2010
  9. BILE ACID SIGNALING & HEPATIC CHOLESTEROL METABOLISM
    Phillip Hylemon; Fiscal Year: 2001
  10. Benzo(a) pyrene Metabolism: Phenotyping and Genotyping
    Stephen Hecht; Fiscal Year: 2005

Scientific Experts

Detail Information

Publications133 found, 100 shown here

  1. ncbi Androgenic modulation of lipid metabolism at subcellular sites in cholestatic rats
    A Hossain
    Department of Physiology, Louisiana State University Medical Center, New Orleans
    Horm Metab Res 26:19-25. 1994
    ..testosterone enanthate (5 mg/kg body weight), increased triacylglycerol lipase (TG-lipase) and neutral cholesterol ester hydrolase (CE-hydrolase) activity in endosomes of normal rats...
  2. ncbi Effects of antinutritional factors on protein digestibility and amino acid availability in foods
    G Sarwar Gilani
    Health Canada, Nutrition Research Division, Bureau of Nutritional Sciences, Banting Research Centre AL 2203 C, Tunney s Pasture, Ottawa, ON, K1A 0L2, Canada
    J AOAC Int 88:967-87. 2005
    ..products, yielding Maillard compounds, oxidized forms of sulfur amino acids, D-amino acids, and lysinoalanine (LAL, an unnatural amino acid derivative)...
  3. ncbi Occupational stress, social support, and depression among black and white professional-managerial women
    M B Snapp
    Department of Sociology and Social Work, Memphis State University
    Women Health 18:41-79. 1992
    ..Depression was measured using the Center for Epidemiologic Studies Depression Scale (CESD)...
  4. ncbi [Influence of alleles of apolipoprotein E on restenosis after coronary angioplasty in women]
    L Flork
    Service de cardiologie, CHU G-Montpied, Clermont-Ferrand
    Arch Mal Coeur Vaiss 91:1475-9. 1998
    ..A greater number of patients is required and further studies are desirable to determine the inflammatory and/or immunological mechanisms through which APO E could influence restenosis...
  5. ncbi [The effect of temperature on the growth and lipopolysaccharide production of gram-negative bacteria]
    C Weber Frick
    ,
    Zentralbl Bakteriol Mikrobiol Hyg B 187:56-69. 1988
    ..the stationary growth phase the amount of lipopolysaccharides (LPS) formed was determined by means of the three LAL tests, i.e. the Capillary test, the "Mini" Endotoxin test and the Coatest endotoxin method...
  6. ncbi [The growth and lipopolysaccharide production of Escherichia coli and Pseudomonas aeruginosa]
    C Weber Frick
    ,
    Zentralbl Bakteriol Mikrobiol Hyg B 186:478-93. 1988
    ..respectively by counting the cfu and by simultaneous determination of LPS by means of the three miniaturized LAL-tests, i.e. the capillary test, the "Mini" endotoxin test and the Coatest endotoxin method...
  7. ncbi The effects of the endothelin ETA receptor antagonist, FR 139317, on infarct size in a rabbit model of acute myocardial ischaemia and reperfusion
    L Mcmurdo
    William Harvey Research Institute, St Bartholomew s Hospital Medical College, London
    Br J Pharmacol 112:75-80. 1994
    ..infarct size (48 +/- 5%) after 45 min occlusion of the antero-lateral branch of the left coronary artery (LAL) and 2 h reperfusion.4...
  8. ncbi Endotoxemia and acute-phase proteins in major abdominal surgery
    K Buttenschoen
    Department of General Surgery, University of Ulm, Steinhoevelstrasse 9, D 89075, Ulm, Germany
    Am J Surg 181:36-43. 2001
    ..Translocation of endotoxin is a controversial issue. The ability of plasma to inactivate endotoxin is an indirect measure of endotoxemia. Endotoxin is a potent stimulator of the inflammatory response and affects the innate immune system...
  9. ncbi Performance assessment of new multiplex probe assay for identification of mycobacteria
    E Tortoli
    Centro Regionale di Riferimento per la Diagnostica delle Micobatteriosi, Laboratorio di Microbiologia e Virologia, Ospedale di Careggi, Piastra del servizi, Viale Morgagni 85, 50134 Florence, Italy
    J Clin Microbiol 39:1079-84. 2001
    ..The turnaround time of the method is approximately 6 h, including a preliminary PCR amplification...
  10. ncbi Lysosomal acid lipase mutations that determine phenotype in Wolman and cholesterol ester storage disease
    R A Anderson
    Department of Internal Medicine, Wake Forest University School of Medicine, Winston Salem, North Carolina, 27157, USA
    Mol Genet Metab 68:333-45. 1999
    ..ester storage disease (CESD), associated with the genetic deficiency of human lysosomal acid lipase/cholesterol ester hydrolase (hLAL) function were investigated with the determination of HLAL activity levels, mRNA and protein ..
  11. ncbi A new mutation in the gene for lysosomal acid lipase leads to Wolman disease in an African kindred
    S Ries
    Institute of Clinical Chemistry and Laboratory Medicine, University of Regensburg, Germany
    J Lipid Res 37:1761-5. 1996
    ..WD) are both autosomal recessive disorders associated with reduced activity and genetic defects of lysosomal acid lipase (LAL)...
  12. ncbi Extraction of cyanobacterial endotoxin
    John Papageorgiou
    CRC for Water Quality and Treatment, Australian Water Quality Centre, Private Mail Bag 3, Salisbury, South Australia, 5108, Australia
    Environ Toxicol 19:82-7. 2004
    ..The Limulus polyphemus amoebocyte lysate (LAL) assay was employed to compare the performance of a novel method utilizing a 1-butanol-water (HBW) solvent system ..
  13. ncbi Genetic and biochemical evidence that CESD and Wolman disease are distinguished by residual lysosomal acid lipase activity
    C Aslanidis
    Institute for Clinical Chemistry and Laboratory Medicine, University of Regensburg, Regensburg, 93042, Germany
    Genomics 33:85-93. 1996
    ..disease are both autosomal recessive disorders associated with reduced activity and genetic defects of lysosomal acid lipase (LAL)...
  14. ncbi Characterization of the extracellular lipase, LipA, of Acinetobacter calcoaceticus BD413 and sequence analysis of the cloned structural gene
    R G Kok
    Department of Microbiology, E C Slater Institute, BioCentrum Amsterdam, The Netherlands
    Mol Microbiol 15:803-18. 1995
    ..The active site serine of AcLipA was changed to an alanine, via site-directed mutagenesis, resulting in production of an inactive extracellular lipase...
  15. ncbi The influence of sanitizers on the lipopolysaccharide composition of Escherichia coli O111
    P Venter
    School for Agriculture and Environmental Sciences, P Bag X20539, Central University of Technology, Free State, Bloemfontein 9300, South Africa
    Int J Food Microbiol 111:221-7. 2006
    ..to cast light on the applicability of O-antigen-based serotyping and endotoxin based Limulus Amebocyte Lysate (LAL) assays applied in the food industry for the identification and quantification of Gram-negative bacteria. E...
  16. ncbi Human lysosomal acid lipase/cholesteryl ester hydrolase and human gastric lipase: site-directed mutagenesis of Cys227 and Cys236 results in substrate-dependent reduction of enzymatic activity
    P Lohse
    Department of Clinical Chemistry, Grosshadern Clinic, University of Munich, Germany
    J Lipid Res 38:1896-905. 1997
    Chemical modification studies and site-directed mutagenesis experiments have provided evidence that human lysosomal acid lipase/cholesteryl ester hydrolase (HLAL), human gastric lipase (HGL), and rat lingual lipase (RLL) are serine ..
  17. ncbi A novel missense LIPA gene mutation, N98S, in a patient with cholesteryl ester storage disease
    Amanda J Hooper
    Department of Core Clinical Pathology and Biochemistry, PathWest Laboratory Medicine WA, Royal Perth Hospital, Perth, Australia
    Clin Chim Acta 398:152-4. 2008
    b>Lysosomal acid lipase plays an important role in maintaining cellular cholesterol homeostasis...
  18. ncbi Intercellular transport of lysosomal acid lipase mediates lipoprotein cholesteryl ester metabolism in a human vascular endothelial cell-fibroblast coculture system
    G N Sando
    Department of Internal Medicine, University of Iowa, Iowa City 52242
    Cell Regul 1:661-74. 1990
    ..from studies of human cell culture models to support the premise that the extracellular transport of lysosomal acid lipase has a function in lipoprotein cholesteryl ester metabolism in vascular tissue...
  19. ncbi Targeted disruption of the mouse lysosomal acid lipase gene: long-term survival with massive cholesteryl ester and triglyceride storage
    H Du
    Division of Human Genetics, Children s Hospital Research Foundation, 3333 Burnet Avenue, Cincinnati, OH 45229 3039, USA
    Hum Mol Genet 7:1347-54. 1998
    b>Lysosomal acid lipase (LAL) is essential for the hydrolysis of the triglycerides and cholesteryl esters in lysosomes...
  20. ncbi A novel variant of lysosomal acid lipase (Leu336-->Pro) associated with acid lipase deficiency and cholesterol ester storage disease
    U Seedorf
    Institut für Arterioskleroseforschung, Westfalische Wilhelms Universitat, Germany
    Arterioscler Thromb Vasc Biol 15:773-8. 1995
    ..studies have shown a G-->A mutation at the 3' splice junction of exon 8 (E8SJM) of the gene encoding lysosomal acid lipase (LAL) in two kindreds with CESD...
  21. ncbi [Acid lipase deficiency: Wolman disease and cholesteryl ester storage disease]
    A Tanaka
    Department of Pediatrics, Osaka City University School of Medicine
    Nihon Rinsho 53:3004-8. 1995
    Wolman disease and cholesteryl ester storage disease (CESD) are caused by a deficiency of lysosomal acid lipase activity, resulting in massive accumulation of cholesteryl ester and triglycerides...
  22. ncbi Correlation of HPV-16/18 infection of human papillomavirus with lung squamous cell carcinomas in Western China
    Yan Yu
    Department of Public Health, Xi an Jiaotong University School of Medicine, Shaanxi 710061, P R China
    Oncol Rep 21:1627-32. 2009
    ....
  23. ncbi Physiology and morphology of protocerebral olfactory neurons in the male moth Manduca sexta
    R Kanzaki
    Arizona Research Laboratories, Division of Neurobiology, University of Arizona, Tucson 85721
    J Comp Physiol A 168:281-98. 1991
    ..neurons (PCNs) innervate a particular neuropil region lateral to the central body, the lateral accessory lobe (LAL), which appears to be important for processing olfactory information. 3...
  24. ncbi Physiological, molecular, and cellular mechanisms of impaired seawater tolerance following exposure of Atlantic salmon, Salmo salar, smolts to acid and aluminum
    Michelle Y Monette
    Organismic and Evolutionary Biology Program, University of Massachusetts, Amherst, MA 01003
    Aquat Toxicol 99:17-32. 2010
    ..Smolts were exposed to: control (pH 6.5, 3.4 micrpg l(-1) Al), acid and low Al (LAl: pH 5.4, 11 microg l(-1) Al), acid and moderate Al (MAl: pH 5...
  25. ncbi A histidine to tyrosine replacement in lysosomal acid lipase causes cholesteryl ester storage disease
    F Pagani
    International Centre for Genetic Engineering and Biotechnology, Trieste, Italy
    Hum Mol Genet 3:1605-9. 1994
    ..b>Lysosomal acid lipase (LAL) activity in cultured skin fibroblasts and peripheral lymphocytes was reduced to approximately 3% and ..
  26. ncbi Subclinical course of cholesteryl ester storage disease in an adult with hypercholesterolemia, accelerated atherosclerosis, and liver cancer
    M Elleder
    Institute of Inherited Metabolic Disorders, Charles University Prague, 1st Faculty of Medicine and General Faculty Hospital, Praha, Czech Republic
    J Hepatol 32:528-34. 2000
    Few cases of asymptomatic cholesteryl ester storage disease (CESD) due to low enzymatic activity of human lysosomal acid lipase/cholesteryl ester hydrolase (hLAL) have been reported thus far in adults Here, we describe a 51-year-old man ..
  27. ncbi Two common mutations (D9N, N291S) in lipoprotein lipase: a cumulative analysis of their influence on plasma lipids and lipoproteins in men and women
    J J Kastelein
    Department of Vascular Medicine, Academic Medical Center, University of Amsterdam, The Netherlands
    Clin Genet 56:297-305. 1999
    ..52(0.29) mmol/l vs. 1.53(0.35) mmol/l: p = 0.83). Our data provide evidence that common variants of the LPL gene are significant modulators of lipid and lipoprotein levels in both men and women...
  28. ncbi Clinical, biochemical and histological analysis of seven patients with cholesteryl ester storage disease
    A Tylki-Szymanska
    Department of Metabolic Diseases, Children s Memorial Health Institute, Warsaw, Poland
    Acta Paediatr Jpn 39:643-6. 1997
    b>Lysosomal acid lipase (LAL) deficiency leads to two phenotypically different diseases: cholesteryl ester storage disease (CESD) and Wolman's disease. Lysosomal acid lipase hydrolyzes cholesteryl esters and triglycerides...
  29. ncbi Accumulated lipids, aberrant fatty acid composition and defective cholesterol ester hydrolase activity in cholesterol ester storage disease
    T Todoroki
    Department of Laboratory Medicine, School of Medicine, Keio University, Tokyo, Japan
    Ann Clin Biochem 37:187-93. 2000
    ..We also found that acid cholesterol ester hydrolase activity in the CESD liver was reduced to 5% of that in the control liver, while neutral cholesterol ..
  30. ncbi Molecular analysis of Mycobacterium isolates from extrapulmonary specimens obtained from patients in Mexico
    Cosme Alvarado Esquivel
    Department of Microbiology, Faculty of Medicine, , Durango, Mexico
    BMC Clin Pathol 9:1. 2009
    ..tuberculosis complex infections in lymph nodes was not found...
  31. ncbi A genotypic analysis of patients receiving Zidovudine with either Lamivudine, Didanosine or Zalcitabine dual therapy using the LiPA point mutation assay to detect genotypic variation at codons 41, 69, 70, 74, 184 and 215
    S Rusconi
    Istituto di Malattie Infettive e Tropicali, Universita di Milano, Ospedale Luigi Sacco, Via GB Grassi 74, 20157, Milan, Italy
    J Clin Virol 19:135-42. 2000
    ..We used LiPA HIV-1 RT assay to identify the presence of either consensus methionine 184 or the mutant valine 184 with three groups of patients who were treated with ZDV/3TC, ZDV/ddI or ZDV/ddC combination therapy...
  32. ncbi A novel variant of lysosomal acid lipase in cholesteryl ester storage disease associated with mild phenotype and improvement on lovastatin
    C Gasche
    Department of Gastroenterology and Hepatology, University of Vienna, Austria
    J Hepatol 27:744-50. 1997
    ..ester storage disease (CESD) is a rare congenital disorder of lipid metabolism, with mutation of the lysosomal acid lipase gene, causing chronic liver disease, usually before adolescence...
  33. ncbi Wolman disease/cholesteryl ester storage disease: efficacy of plant-produced human lysosomal acid lipase in mice
    Hong Du
    Division and Program in Human Genetics, Cincinnati Children s Hospital Research Foundation, Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, OH 45229, USA
    J Lipid Res 49:1646-57. 2008
    b>Lysosomal acid lipase (LAL) is an essential enzyme that hydrolyzes triglycerides (TGs) and cholesteryl esters (CEs) in lysosomes. Genetic LAL mutations lead to Wolman disease (WD) and cholesteryl ester storage disease (CESD)...
  34. ncbi New lysosomal acid lipase gene mutants explain the phenotype of Wolman disease and cholesteryl ester storage disease
    F Pagani
    International Centre for Genetic Engineering and Biotechnology, Trieste, Italy
    J Lipid Res 39:1382-8. 1998
    Deficiency of lysosomal acid lipase (LAL) leads to either Wolman disease (WD) or the more benign cholesteryl ester storage disease (CESD)...
  35. ncbi Long-term administration of the HMG-CoA reductase inhibitor lovastatin in two patients with cholesteryl ester storage disease
    F Rassoul
    Department of Clinical Chemistry and Pathobiochemistry, University Leipzig Working Group Health Promotion and Prevention of Atherosclerosis AGA, Germany
    Int J Clin Pharmacol Ther 39:199-204. 2001
    ..competitive inhibitor of 3-hydroxy-3-methylglutaryl-coenzyme A (HMG-CoA) reductase, was initiated in two patients with cholesteryl ester storage disease (CESD), and concentrations of plasma lipids were monitored over a period of 9 years.
  36. ncbi Serratia ATP-binding cassette protein exporter, Lip, recognizes a protein region upstream of the C terminus for specific secretion
    K Omori
    Discovery Research Laboratory, Tanabe Seiyaku Co, Ltd, 2 50, Kawagishi 2 chome, Toda, Saitama 335 8505, Japan
    J Biol Chem 276:27111-9. 2001
    ..Thus, a determinant within the secretory protein for Lip-mediated secretion was fully defined...
  37. ncbi Compound heterozygosity for a Wolman mutation is frequent among patients with cholesteryl ester storage disease
    P Lohse
    Department of Clinical Chemistry, Grosshadern Clinic, University of Munich, Munich, Germany
    J Lipid Res 41:23-31. 2000
    ..rare autosomal recessive lipoprotein-processing disorders caused by mutations in the gene encoding human lysosomal acid lipase. Thus far we have elucidated the genetic defects in 15 unrelated CESD patients...
  38. ncbi Escherichia coli LipA is a lipoyl synthase: in vitro biosynthesis of lipoylated pyruvate dehydrogenase complex from octanoyl-acyl carrier protein
    J R Miller
    Howard Hughes Medical Institute, Department of Biological Chemistry, University of Michigan, Ann Arbor, Michigan 48109 0606, USA
    Biochemistry 39:15166-78. 2000
    ..These results provide information about the mechanism of LipA catalysis and place LipA within the family of iron-sulfur proteins that utilize AdoMet for radical-based chemistry...
  39. ncbi Lipoyl synthase requires two equivalents of S-adenosyl-L-methionine to synthesize one equivalent of lipoic acid
    Robert M Cicchillo
    Department of Biochemistry and Molecular Biology, The Pennsylvania State University, University Park, Pennsylvania 16802, USA
    Biochemistry 43:6378-86. 2004
    ..Last, our data indicate that 2 equiv of AdoMet are cleaved irreversibly in forming 1 equiv of [lipoyl]H-protein and are consistent with a model in which two LipA proteins are required to synthesize one lipoyl group...
  40. ncbi Performance characteristics of the limulus amebocyte lysate assay and gas chromatography-mass spectrum analysis of lipopolysaccharides relative to nitric oxide production by peritoneal exudates of cells
    Hsi Hsun Lin
    Department of Infectious Disease, E Da Hospital I Shou University, Kaoshiung, Taiwan
    J Hazard Mater 145:431-6. 2007
    Limulus amebocyte lysate (LAL) assay and gas chromatography-mass spectrum (GC-MS) analysis are usually used for the quantification of lipopolysaccharides (LPS) from the environment...
  41. ncbi Enzyme therapy for lysosomal acid lipase deficiency in the mouse
    H Du
    The Children s Hospital Research Foundation, Division of Human Genetics, 3333 Burnet Avenue, Cincinnati, OH 45229 3039, USA
    Hum Mol Genet 10:1639-48. 2001
    b>Lysosomal acid lipase (LAL) is the critical enzyme for the hydrolysis of the triglycerides (TG) and cholesteryl esters (CE) delivered to lysosomes...
  42. ncbi Wolman disease and cholesteryl ester storage disease diagnosed by histological and ultrastructural examination of intestinal and liver biopsy
    Renata Boldrini
    Dipartimento di Anatomia Patologia, Ospedale Pediatrico, Bambino Gesu, Rome, Italy
    Pathol Res Pract 200:231-40. 2004
    Deficient activity of lysosomal acid lipase (LAL) results in massive accumulation of cholesteryl esters and triglycerides in most tissues of the body...
  43. ncbi Critical roles of lysosomal acid lipase in myelopoiesis
    Peng Qu
    Center for Immunobiology, Indiana University School of Medicine, Indianapolis, IN 46202, USA
    Am J Pathol 176:2394-404. 2010
    b>Lysosomal acid lipase (LAL) is a key enzyme that cleaves cholesteryl esters and triglycerides to generate free fatty acids and cholesterol in lysosomes...
  44. ncbi Altered mononuclear phagocyte differentiation associated with genetic defects of the lysosomal acid lipase
    G Rothe
    Institute for Clinical Chemistry and Laboratory Medicine, University of Regensburg, Germany
    Atherosclerosis 130:215-21. 1997
    ..may be related to atherogenesis, was analyzed using recently characterized autosomal recessive defects of lysosomal acid lipase (LAL) expression as model system...
  45. ncbi [Determination of hepatitis B virus genotype and detection of lamivudine-resistance mutations]
    M C Nogales
    Servicio de Microbiologia, Hospital Universitario de Valme, Sevilla, Spain
    Gastroenterol Hepatol 27:515-20. 2004
    ..To analyze the influence of several genotypes on the pattern of mutations developed and on values of viral load and alanine aminotransferase (ALT) after their development...
  46. ncbi Protein-bound D-amino acids, and to a lesser extent lysinoalanine, decrease true ileal protein digestibility in minipigs as determined with (15)N-labeling
    M De Vrese
    Federal Dairy Research Centre, Department of Physiology and Biochemistry of Nutrition, D 24103 Kiel, Germany
    J Nutr 130:2026-31. 2000
    ..of food may increase the concentrations of protein-bound D-amino acids and cross-links such as lysinoalanine (LAL)...
  47. ncbi Structural classification by the Lipase Engineering Database: a case study of Candida antarctica lipase A
    Michael Widmann
    Institute of Technical Biochemistry, University of Stuttgart, Allmandring 31, 70569 Stuttgart, Germany
    BMC Genomics 11:123. 2010
    ..This involved the comparison of CALA to similar structures as well as sequence-based comparisons against the content of the LED...
  48. ncbi A novel lipase/chaperone pair from Ralstonia sp. M1: analysis of the folding interaction and evidence for gene loss in R. solanacearum
    D T Quyen
    Institute of Biotechnology, Vietnamese Academy of Science and Technology, 18 Hoang Quoc Viet Road, Caugiay District, 10600 Hanoi, Vietnam
    Mol Genet Genomics 272:538-49. 2004
    ..A lipase:chaperone ratio of 1:10 was found to be optimal, yielding an enzyme preparation with a specific activity of 650 U/mg...
  49. ncbi Expression of lysosomal acid lipase mutants detected in three patients with cholesteryl ester storage disease
    F Pagani
    International Centre for Genetic Engineering and Biotechnology, Trieste, Italy
    Hum Mol Genet 5:1611-7. 1996
    b>Lysosomal acid lipase (LAL) gene mutations were identified in three patients with cholesteryl ester storage disease (CESD)...
  50. ncbi Immunoreactivity against choline acetyltransferase, gamma-aminobutyric acid, histamine, octopamine, and serotonin in the larval chemosensory system of Dosophila melanogaster
    François Python
    Department of Biology and Program in Neuroscience, University of Fribourg, CH 1700 Fribourg, Switzerland
    J Comp Neurol 453:157-67. 2002
    ..As in the adult fly, larval olfactory afferents project into the (larval) antennal lobe (LAL), where they synapse onto local interneurons and projection neurons, whereas gustatory afferents terminate ..
  51. ncbi Characterization of lysosomal acid lipase mutations in the signal peptide and mature polypeptide region causing Wolman disease
    O Zschenker
    Department of Medicine, University Hospital Eppendorf, 20246 Hamburg, Germany
    J Lipid Res 42:1033-40. 2001
    Wolman disease results from an inherited deficiency of lysosomal acid lipase (LAL; EC 3.1.1.13). This enzyme is essential for the hydrolysis of cholesteryl esters and triacylglycerols derived from endocytosed lipoproteins...
  52. ncbi Substrate-induced lipase gene expression and aflatoxin production in Aspergillus parasiticus and Aspergillus flavus
    J Yu
    USDA ARS, Southern Regional Research Center, New Orleans, LA, USA
    J Appl Microbiol 95:1334-42. 2003
    ..To establish a relationship between lipase gene expression and aflatoxin production by cloning the lipA gene and studying its expression pattern in several aflatoxigenic and nontoxigenic isolates of Aspergillus flavus and A. parasiticus...
  53. ncbi bldA-dependent expression of the Streptomyces exfoliatus M11 lipase gene (lipA) is mediated by the product of a contiguous gene, lipR, encoding a putative transcriptional activator
    L Servin-Gonzalez
    Departamento de Biologia Molecular, Instituto de Investigaciones Biomedicas, Universidad Nacional Autonoma de Mexico, Ciudad Universitaria, D F, Mexico
    J Bacteriol 179:7816-26. 1997
    ....
  54. ncbi [Populations of afferent neurons in the sensory ganglia detected using lectin from Laburnum anagyroides]
    A D Nozdrachev
    Department of General Physiology, St Petersburg State University
    Morfologiia 124:38-40. 2003
    ..and trigeminal ganglion of adult albino rats using a conjugate of fucose-specific Laburnum anagyroides lectin (LAL) with peroxidase...
  55. ncbi Fiber types in the mouse levator auris longus muscle: a convenient preparation to study muscle and nerve plasticity
    I Erzen
    Institute of Anatomy, Medical Faculty, Ljubljana, Slovenia
    J Neurosci Res 59:692-7. 2000
    The histochemical composition of the levator auris longus (LAL) muscle has been investigated in adult NMRi mice...
  56. ncbi Kala-azar epidemic in Varanasi district, India
    R Kumar
    Department of Biochemistry, Faculty of Science, Banaras Hindu University, Varanasi, India
    Bull World Health Organ 77:371-4. 1999
    Reports at the Sir Sunder Lal Hospital, Banaras Hindu University, of a large number of kala-azar cases from one particular village in Varanasi district, Uttar Pradesh, led us to carry out an epidemiological study of the situation using ..
  57. ncbi Origin, microbiology, nutrition, and pharmacology of D-amino acids
    Mendel Friedman
    Western Regional Research Center, Agricultural Research Service, USDA, Albany, CA 94710, USA
    Chem Biodivers 7:1491-530. 2010
    ..acids (LAAs) to D-amino acids (DAAs) and concurrent formation of cross-linked amino acids such as lysinoalanine (LAL). The diet contains both processing-induced and naturally-formed DAA...
  58. ncbi Systematic mutagenesis of potential glycosylation sites of lysosomal acid lipase
    Oliver Zschenker
    Department of Medicine, University Hospital Eppendorf, Martinistrasse 52, 20246 Hamburg, Germany
    J Biochem 137:387-94. 2005
    b>Lysosomal acid lipase (LAL; EC 3.1.1.13) is a key enzyme in the intracellular lipid metabolism. It hydrolyzes exogenous triglycerides and cholesterol esters taken up by various cell types...
  59. ncbi Evaluation of luciferin-isopropyl acetal as a CYP3A4 substrate for human hepatocytes: effects of organic solvents, cytochrome P450 (P450) inhibitors, and P450 inducers
    Albert P Li
    Advanced Pharmaceutical Sciences Inc and In Vitro ADMET Laboratories LLC, 9221 Rumsey Road, Columbia, MD 21045, USA
    Drug Metab Dispos 37:1598-603. 2009
    ....
  60. ncbi [Comparison of three miniaturized Limulus-amoebocyte-lysate methods]
    C Weber Frick
    , ,
    Zentralbl Bakteriol Mikrobiol Hyg B 185:430-51. 1988
    Comparative examinations with three different micro-models for the LAL-test methods,--namely the capillary test, Endotoxin-test "Mini" and Coatest-Endotoxin-method-, tested with 11 Enterobacteriaceae- and 6 Pseudomonas-type-strains and ..
  61. ncbi Comparison of endotoxin (LPS) measurements and plate counts for bacteriological assessment of drinking water
    E Korsholm
    Department of Hygiene and Microbiology, Royal Veterinary and Agricultural University, Frederiksberg C, Denmark
    Zentralbl Bakteriol Mikrobiol Hyg B 185:121-30. 1987
    ..Kings agar B and R2A), and different fractions of LPS (total, free, and bound) were determined using a chromogenic LAL test kit. The closest correlation was found when total LPS was compared with R2A plate counts (r = 0...
  62. ncbi Levofloxacin-based and clarithromycin-based triple therapies as first-line and second-line treatments for Helicobacter pylori infection: a randomised comparative trial with crossover design
    Jyh Ming Liou
    Department of Internal Medicine and Primary Care Medicine, National Taiwan University Hospital, National Taiwan University, College of Medicine, No 7 Chung Shan S Road, Taipei, Taiwan
    Gut 59:572-8. 2010
    ..The aim of this study was to assess the eradication rates of these two regimens using different administration sequences...
  63. ncbi Prevalence and correlates of sleep disturbance in systemic sclerosis--results from the UCLA scleroderma quality of life study
    Tracy Frech
    Department of Internal Medicine, Division of Rheumatology, University of Utah, Salt Lake City, UT, USA
    Rheumatology (Oxford) 50:1280-7. 2011
    ..Rheumatologic disorders are associated with sleep disturbances. This study examines sleep disturbance correlates in patients with SSc...
  64. ncbi Endotoxin detection--from limulus amebocyte lysate to recombinant factor C
    Jeak Ling Ding
    Department of Biological Sciences, Faculty of Science, National University of Singapore, 14 Science Drive 4, Singapore, 117543, Singapore
    Subcell Biochem 53:187-208. 2010
    ..Thus early and sensitive detection of endotoxin is crucial to prevent endotoxaemia. The limulus amebocyte lysate (LAL) has been widely used for ~30 years for the detection of endotoxin in the quality assurance of injectable drugs and ..
  65. ncbi A novel cold-adapted lipase from Acinetobacter sp. XMZ-26: gene cloning and characterisation
    Xiaomei Zheng
    Biotechnology Research Institute, Chinese Academy of Agricultural Sciences, No 12 Zhongguancun South Street, Beijing 100081, People s Republic of China
    Appl Microbiol Biotechnol 90:971-80. 2011
    ..This cold-adapted lipase may find applications in the detergent industry and organic synthesis...
  66. ncbi Protein-protein interactions in assembly of lipoic acid on the 2-oxoacid dehydrogenases of aerobic metabolism
    Bachar H Hassan
    Department of Biochemistry, University of Illinois, Urbana, Illinois 61801, USA
    J Biol Chem 286:8263-76. 2011
    ..These results indicate that lipoic acid is not only assembled on the dehydrogenase lipoyl domains but that the enzymes that catalyze the assembly are also present "on site."..
  67. ncbi Variations in eye volume, surface area, and shape with refractive error in young children by magnetic resonance imaging analysis
    Laurence S Lim
    Singapore Eye Research Institute, Singapore
    Invest Ophthalmol Vis Sci 52:8878-83. 2011
    ..qualitatively from the three-dimensional models and quantitatively by measurement of the longitudinal axial length (LAL), horizontal width, and vertical height along the cardinal axes. Results...
  68. ncbi Evaluation of quantification methods of occupational endotoxin exposure
    V Liebers
    Research Institute of Occupational Medicine BGFA of German Social Accident Insurance, Ruhr University Bochum, Bochum, Germany
    J Toxicol Environ Health A 70:1798-805. 2007
    ..Although the determination of endotoxin levels by using the Limulus amoebocyte lysate (LAL) assay is internationally accepted, reliability and variation of values measured with this test remain a point of ..
  69. ncbi Cognitive brain potentials in kindergarten children with subtyped risks of reading retardation
    Dirk J Bakker
    Vrije Universiteit, Amsterdam, The Netherlands
    Ann Dyslexia 57:99-111. 2007
    ..kindergarten children who either had been subtyped as being at-risk of developing an L- or P-type dyslexia (LAL versus LAP) or who were not at-risk...
  70. ncbi A calcium-gated lid and a large beta-roll sandwich are revealed by the crystal structure of extracellular lipase from Serratia marcescens
    Reto Meier
    Department of Chemistry and Biochemistry, University Bern Freiestrasse 3, CH 3012 Bern, Switzerland
    J Biol Chem 282:31477-83. 2007
    ..The analysis of the properties of the beta-roll domains suggests an intramolecular chaperone function...
  71. ncbi Crystallization and preliminary crystallographic studies of LipA, a secretory lipase/esterase from Xanthomonas oryzae pv. oryzae
    Gudlur Aparna
    Centre for Cellular and Molecular Biology, Uppal Road, Hyderabad 500 007, India
    Acta Crystallogr Sect F Struct Biol Cryst Commun 63:708-10. 2007
    ..8 degrees . Type II crystals have unit-cell parameters a = 103.6, b = 54.6, c = 66.3 A, beta = 92.6 degrees and diffract to 1.86 A. Solvent-content analysis shows one monomer in the asymmetric unit in both the crystal forms...
  72. ncbi Clinical depression versus distress among patients with type 2 diabetes: not just a question of semantics
    Lawrence Fisher
    Department of Family and Community Medicine, Box 0900, University of California San Francisco, San Francisco, CA 94143, USA
    Diabetes Care 30:542-8. 2007
    ..We sought to determine differences between structured interviews, symptom questionnaires, and distress measures for assessment of depression in patients with diabetes...
  73. ncbi The autophagy-lysosomal pathway is involved in TAG degradation in the liver: the effect of high-sucrose and high-fat diet
    M Cahova
    Department of Metabolism and Diabetes, Centre of Experimental Medicine, Institute for Clinical and Experimental Medicine, Prague, Czech Republic
    Folia Biol (Praha) 56:173-82. 2010
    ..Male Wistar rats were administered high-fat or high-sucrose diet for two weeks. Individual lipases (HL; TGH; LAL) were identified according to their different pH optimum...
  74. ncbi Overexpression of lysosomal acid lipase and other proteins in atherosclerosis
    Oliver Zschenker
    Medical Center, University Hospital Hamburg Eppendorf, Martinistrasse 52, 20246 Hamburg, Germany
    J Biochem 140:23-38. 2006
    ..Special focus is placed on the lysosomal acid lipase (LAL), because LAL connects extra-cellular with intra-cellular lipid metabolism and is the only hydrolase ..
  75. ncbi Effect of extraction and assay media on analysis of airborne endotoxin
    Suzanne Spaan
    Institute for Risk Assessment Sciences, Environmental Epidemiology Division, Utrecht University, P O Box 80178, 3508 TD Utrecht, The Netherlands
    Appl Environ Microbiol 74:3804-11. 2008
    ..yields when Tween 20 was added to the media used for filter extraction and in the Limulus amebocyte lysate (LAL) assay...
  76. ncbi From nanocrystal synthesis to functional nanostructure fabrication: laser ablation in liquid
    P Liu
    State Key Laboratory of Optoelectronic Materials and Technologies, Institute of Optoelectronic and Functional Composite Materials, Nanotechnology Research Center, School of Physics and Engineering, Zhongshan Sun Yat Sen University, Guangzhou 510275, Guangdong, P R China
    Phys Chem Chem Phys 12:3942-52. 2010
    ..For this issue, we, in this review, introduce recent developments in laser ablation in liquid (LAL) for the synthesis and fabrication of novel nanostructures with metastable phases and shapes...
  77. ncbi Integrating In Silico and In vitro approaches to dissect the stereoselectivity of Bacillus subtilis lipase A toward ketoprofen vinyl ester
    Zhong Ni
    Department of Chemistry, Zhejiang University, Hangzhou 310027, China
    Chem Biol Drug Des 78:301-8. 2011
    ....
  78. ncbi The expression of endotoxic activity in the Limulus test as compared to cytokine production in immune cells
    Klaus Brandenburg
    Forschungszentrum Borstel, Leibniz Zentrum für Medizin und Biowissenschaften, Borstel, Germany
    Curr Med Chem 16:2653-60. 2009
    ..of the clotting cascade from the horseshoe crab (Limulus polyphemus), the Limulus amoebocyte lysate test (LAL), has been the standard and most sensitive assay to detect bacterial endotoxins...
  79. ncbi Depressed mood is not a risk factor for incident dementia in a community-based cohort
    James T Becker
    Department of Psychiatry, School of Medicine, Graduate School of Public Health, University of Pittsburgh, 3501 Forbes Avenue, Pittsburgh, PA 15213, USA
    Am J Geriatr Psychiatry 17:653-63. 2009
    ..To determine the relationship between depressed mood and the development of Alzheimer disease in cognitively normal individuals...
  80. ncbi A lipA (yutB) mutant, encoding lipoic acid synthase, provides insight into the interplay between branched-chain and unsaturated fatty acid biosynthesis in Bacillus subtilis
    Natalia Martin
    Departamento de Microbiologia, Facultad de Ciencias Bioquimicas y Farmaceuticas, Instituto de Biologia Molecular y Celular de Rosario, Universidad Nacional de Rosario, Suipacha 531, 2000 Rosario, Argentina
    J Bacteriol 191:7447-55. 2009
    ..Furthermore, we show that the cold-sensitive phenotype of a B. subtilis strain deficient in Delta5 desaturase is suppressed by isoleucine only if LipA is present...
  81. ncbi Snake venomics and venom gland transcriptomic analysis of Brazilian coral snakes, Micrurus altirostris and M. corallinus
    Carlos Corrêa-Netto
    Instituto de Bioquimica Medica, Programa de Biologia Estrutural and Instituto Nacional de Biologia Estrutural e Bioimagem, Universidade Federal do Rio de Janeiro, Brazil
    J Proteomics 74:1795-809. 2011
    ..C-type lectin-like, L-amino acid oxidase) and 4 species-specific (CRISP, Kunitz-type inhibitor, lysosomal acid lipase in M. altirostris; serine proteinase in M. corallinus) toxin classes...
  82. ncbi Critical roles of lysosomal acid lipase in T cell development and function
    Peng Qu
    The Center for Immunobiology, Department of Pathology and Laboratory Medicine, Indiana University School of Medicine, 635 Barnhill Dr, Indianapolis, IN 46202, USA
    Am J Pathol 174:944-56. 2009
    b>Lysosomal acid lipase (LAL) cleaves cholesteryl esters and triglycerides to generate free fatty acids and cholesterol in lysosomes...
  83. ncbi Validation of the line-probe assay for rapid detection of rifampicin-resistant Mycobacterium tuberculosis in Vietnam
    N S Shah
    International Research and Programs Branch, Division of TB Elimination, Centers for Disease Control and Prevention, Atlanta, Georgia, USA
    Int J Tuberc Lung Dis 13:247-52. 2009
    ..We validated and compared this assay with conventional drug susceptibility testing (DST)...
  84. ncbi Evaluation of the INNO-LiPA mycobacteria v2 assay for identification of aquatic mycobacteria
    F Pourahmad
    Institute of Aquaculture, University of Stirling, Stirling, UK
    J Fish Dis 31:931-40. 2008
    ..Nevertheless, the kit proved to be a rapid and reliable method for identifying mycobacteria in the aquatic environment and would be particularly useful in laboratories without sequencing facilities...
  85. ncbi Role of lysosomal acid lipase in the intracellular metabolism of LDL-transported dehydroepiandrosterone-fatty acyl esters
    Feng Wang
    Dept of Medicine, Helsinki Univ Central Hospital, Haartmaninkatu 4, 00290 Helsinki, Finland
    Am J Physiol Endocrinol Metab 295:E1455-61. 2008
    ..fate of low-density lipoprotein-associated [(3)H]DHEA-FAE ([(3)H]DHEA-FAE-LDL) and the possible role of lysosomal acid lipase (LAL) in the hydrolysis of DHEA-FAE in cultured human cells...
  86. ncbi Evaluation of lot-to-lot repeatability and effect of assay media choice in the recombinant Factor C assay
    Jennifer Helen McKenzie
    Biomedical Engineering and Biotechnology Program, University of Massachusetts, Lowell, MA, USA
    J Environ Monit 13:1739-45. 2011
    ..using current biological assay methods arising in part from lot-to-lot variability of the Limulus-amebocyte lysate (LAL) reagents...
  87. ncbi Lysosomal acid lipase deficiency impairs regulation of ABCA1 gene and formation of high density lipoproteins in cholesteryl ester storage disease
    Kristin L Bowden
    Department of Medicine, Institute for Heart and Lung Health, St Paul s Hospital, Vancouver, British Columbia V6Z 1Y6, Canada
    J Biol Chem 286:30624-35. 2011
    ..ester storage disease (CESD), caused by mutations in the LIPA gene that result in less than 5% of normal lysosomal acid lipase (LAL) activity...
  88. ncbi Detection and typing of human papillomaviruses combining different methods: polymerase chain reaction, restriction fragment length polymorphism, line probe assay and sequencing
    Nina Milutin Gasperov
    Department of Molecular Medicine, Rudjer Boskovic Institute, Zagreb, Croatia
    Pathol Oncol Res 14:355-63. 2008
    ..Careful consideration has to be made before the implementation of either HPV typing methods in clinical laboratories...
  89. ncbi Efficient secretion of Bacillus subtilis lipase A in Saccharomyces cerevisiae by translational fusion to the Pir4 cell wall protein
    Maria Mormeneo
    Unidad de Microbiología Facultad de Farmacia, Burjassot, Valencia, Spain
    Appl Microbiol Biotechnol 80:437-45. 2008
    ..This is, to our knowledge, the first report of the efficient production, as a secreted protein, of lipase A of B. subtilis in baker's yeast...
  90. ncbi A novel genetic selection system for improved enantioselectivity of Bacillus subtilis lipase A
    Ykelien L Boersma
    Department of Pharmaceutical Biology, University of Groningen, Groningen, The Netherlands
    Chembiochem 9:1110-5. 2008
    ....
  91. ncbi Limulus amebocyte lysate assay for endotoxins by an adsorption method with polycation-immobilized cellulose beads
    Masayo Sakata
    Department of Applied Chemistry and Biochemistry, Graduate School of Science and Technology, Kumamoto University, 2 39 1 Kurokami, Kumamoto 860 8555, Japan
    Anal Sci 26:291-6. 2010
    To assay lipopolysaccharides (LPSs) in solutions containing Limulus amebocyte lysate (LAL)-inhibiting or LAL-enhancing compounds, we developed a selective endotoxin (LPS) assay using poly(epsilon-lysine)-immobilized cellulose beads (PL-..
  92. ncbi Comparison of the effectiveness of amitriptyline and gabapentin on chronic neuropathic pain in persons with spinal cord injury
    Diana H Rintala
    Michael E DeBakey Veterans Affairs Medical Center, Houston, TX 77030, USA
    Arch Phys Med Rehabil 88:1547-60. 2007
    ..To test the hypotheses that both amitriptyline and gabapentin are more effective in relieving neuropathic pain than an active placebo, diphenhydramine...
  93. ncbi [Colony count and LPS content of gram-negative bacteria in cold storage foods and water]
    C Weber Frick
    , ,
    Zentralbl Bakteriol Mikrobiol Hyg B 187:181-208. 1989
    ..of gram negative bacteria was monitored by means of both colony counts and LPS-formation as determined by the three LAL tests methods (the Capillary test, the "Mini" Endotoxin test and the Coatest endotoxin method)...
  94. ncbi Difference in substrate specificity between human and mouse lysosomal acid lipase: low affinity for cholesteryl ester in mouse lysosomal acid lipase
    J E Groener
    Department of Pediatrics, Leiden University Medical Center, Leiden, The Netherlands
    Biochim Biophys Acta 1487:155-62. 2000
    b>Lysosomal acid lipase (LAL) is essential for the intracellular degradation of cholesteryl esters (CE) and triacylglycerols (TG) that are delivered to lysosomes by low density lipoprotein (LDL) receptor mediated endocytosis...
  95. ncbi Comparative evaluation of three human immunodeficiency virus genotyping systems: the HIV-GenotypR method, the HIV PRT GeneChip assay, and the HIV-1 RT line probe assay
    J W Wilson
    Division of Infectious Diseases, Department of Internal Medicine, Mayo Clinic, Rochester, Minnesota 55905, USA
    J Clin Microbiol 38:3022-8. 2000
    ..We conclude that although discrepancies in results exist between assays, each assay showed a similar capacity to identify potentially clinically relevant mutations related to patient treatment regimens...
  96. ncbi Evaluation of the LiPA MYCOBACTERIA assay for identification of mycobacterial species from BACTEC 12B bottles
    N Miller
    Department of Pathology, Jackson Memorial Medical Center, University of Miami, Miami, Florida, USA
    J Clin Microbiol 38:1915-9. 2000
    ..szulgai). The LiPA MYCOBACTERIA assay was easy to perform, and the interpretation of the positive bands was clear-cut. Following PCR amplification and gel electrophoresis, the LiPA assay was completed within 3 h...
  97. ncbi [Lysosomal acid lipase deficiency. Overview of Czech patients]
    M Elleder
    Ustav dĕdicných poruch metabolismu 1 LF UK, Praha
    Cas Lek Cesk 138:719-24. 1999
    ..It is characterized by the storage of CE (which gave this entity the name of cholesteryl storage disease--CESD)...
  98. ncbi Chemistry, biochemistry, nutrition, and microbiology of lysinoalanine, lanthionine, and histidinoalanine in food and other proteins
    M Friedman
    Western Regional Research Center, Agricultural Research Service, U S Department of Agriculture, Albany, CA 94710, USA
    J Agric Food Chem 47:1295-319. 1999
    ..and cross-linked amino acids such as dehydroalanine, methyldehydroalanine, beta-aminoalanine, lysinoalanine (LAL), ornithinoalanine, histidinoalanine (HAL), phenylethylaminoalanine, lanthionine (LAN), and methyl-lanthionine ..
  99. ncbi Hepatosplenomegalic lipidosis: what unless Gaucher? Adult cholesteryl ester storage disease (CESD) with anemia, mesenteric lipodystrophy, increased plasma chitotriosidase activity and a homozygous lysosomal acid lipase -1 exon 8 splice junction mutation
    S Vom Dahl
    Division of Gastroenterology, Hepatology and Infectious Diseases, Heinrich Heine University, Dusseldorf, Germany
    J Hepatol 31:741-6. 1999
    ..The activity of lysosomal acid lipase (LAL) in fibroblast homogenates was decreased to 12% of control subjects...
  100. ncbi The Streptomyces coelicolor A3(2) lipAR operon encodes an extracellular lipase and a new type of transcriptional regulator
    F Valdez
    Departamento de Biologia Molecular, Instituto de Investigaciones Biomedicas, Universidad Nacional Autonoma de Mexico, DF, Mexico
    Microbiology 145:2365-74. 1999
    ..A lipase-deficient strain of S. coelicolor was constructed and found to be slightly affected in production of the polyketide antibiotic actinorhodin...
  101. ncbi Molecular defects underlying Wolman disease appear to be more heterogeneous than those resulting in cholesteryl ester storage disease
    P Lohse
    Department of Clinical Chemistry, Grosshadern Clinic, University of Munich, Munich, Germany
    J Lipid Res 40:221-8. 1999
    Human lysosomal acid lipase/cholesteryl ester hydrolase (hLAL) is essential for the intralysosomal metabolism of cholesteryl esters and triglycerides taken up by receptor-mediated endocytosis of lipoprotein particles...

Research Grants76

  1. Disordered Cellular Cholesterol Movement, Cell Death, and Multisystem Disease
    John M Dietschy; Fiscal Year: 2010
    ..is processed through the late endosomal/lysosomal compartment of every cell where at least three proteins (lysosomal acid lipase, LAL;Niemann-Pick type C1, NPC1;and Niemann-Pick type C2, NPC2,) act in tandem to metabolize and move this ..
  2. Pathophysiology of PPARgamma in the lung
    Hong Du; Fiscal Year: 2010
    ..MMP-12) and that this control derives from fatty acid ligands, generated in the lysosomes by lysosomal acid lipase (LAL)...
  3. Pathophysiology of PPARgamma in the lung
    Hong Du; Fiscal Year: 2010
    ..MMP-12) and that this control derives from fatty acid ligands, generated in the lysosomes by lysosomal acid lipase (LAL)...
  4. Pathophysiology of PPARgamma in the lung
    Hong Du; Fiscal Year: 2009
    ..MMP-12) and that this control derives from fatty acid ligands, generated in the lysosomes by lysosomal acid lipase (LAL)...
  5. NEW METHODS FOR EVAL OF ORGANIC DUST AEROSOLS
    Stephen Reynolds; Fiscal Year: 2006
    ..Analysis using both assay (LAL and monoclonal antibody) and chemical (mass spectrometry) methods will help elucidate relationships between ..
  6. PATHOGENESIS OF LYSOSOMAL ACID LIPASE DEFICIENCY
    Hong Du; Fiscal Year: 2001
    ..These diseases are due to the mutations at the lal locus that leads to a deficiency of lysosomal acid lipase (LAL)...
  7. Cholesterol Ester Hydrolysis and Cholesterol Efflux
    Shobha Ghosh; Fiscal Year: 2006
    ..UC then moves to the plasma membrane for transfer to the acceptor molecule. Neutral Cholesterol ester hydrolase (CEH) catalyzes the hydrolysis of stored cholesterol esters...
  8. Macrophage-lipoprotein interactions
    Frederick R Maxfield; Fiscal Year: 2010
    ..The presence of lysosomal acid lipase in this compartment would lead to hydrolysis of cholesterol esters and release of free sterol, which can ..
  9. BILE ACID SIGNALING & HEPATIC CHOLESTEROL METABOLISM
    Phillip Hylemon; Fiscal Year: 2001
    ..and phospholipid homeostasis in the hepatocyte, including: CYP7a1, CYP8b1, LDL receptor, neutral cholesterol ester hydrolase (CEH) and mdr2 phospholipid transporter; (4) To determine if the regulation of CYP7a1 by bile acids and ..
  10. Benzo(a) pyrene Metabolism: Phenotyping and Genotyping
    Stephen Hecht; Fiscal Year: 2005
    ..the relationship between these metabolite levels and gene polymorphisms in smokers, examining cytochrome P450 lAl [CYP/Al], CYPIB], microsomal epoxide hydrolase [EPHXI], glutathione transferase M1 [CSTM1], and GSTPI; 3) Determine ..
  11. Adjustable Injectable Intraocular Lens
    Shiao Chang; Fiscal Year: 2007
    ..of an injectable, gel-filled capsular lens with an adjustable, anterior capsule supported intraocular lens (AC-LAL)...
  12. Regulation of Capsule Biosynthesis in N. meningitidis
    David Stephens; Fiscal Year: 2006
    ....
  13. Adjustable Phakic Intraocular Lens
    Shiao Chang; Fiscal Year: 2007
    ..0 D. In Phase II, we propose to further study the selected material to optimize the soft acrylic LAL formulation, develop the irradiation conditions to produce predictable myopic, hyperopic and astigmatic changes in ..
  14. MINORITY PREDOCTORAL FELLOWSHIP PROGRAM
    NATASHA NESBITT; Fiscal Year: 2005
    ..Lastly, analogues of AdoMet with the potential to stabilize a radical at the 5'-carbon of 5'-deoxyadenosine will be used to establish the involvement of radical intermediates in the LAS reaction. ..