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Lipase A, lysosomal acid, cholesterol esterase

LAL, lysosomal acid lipase, LIPA, CESD, cholesteryl esterase, cholesterol ester hydrolase
This gene encodes lipase A, the lysosomal acid lipase (also known as cholesterol ester hydrolase). This enzyme functions in the lysosome to catalyze the hydrolysis of cholesteryl esters and triglycerides. Mutations in this gene can result in Wolman disease and cholesteryl ester storage disease. Alternatively spliced transcript variants encoding the same protein have been found for this gene. [provided by RefSeq, Jul 2008] (from NCBI)
Top mentioned proteins: ACID, CAN, HAD, ESI, V1a
Papers using LAL antibodies
TRAM is specifically involved in the Toll-like receptor 4-mediated MyD88-independent signaling pathway.
Fritz Jörg Hermann, In PLoS ONE, 2002
... The Limulus Amebocyte Lysate (LAL) assay was performed with a GenScript Chromogenic LAL endotoxin assay ...
Analysis of relative gene expression data using real-time quantitative PCR and the 2(-Delta Delta C(T)) Method.
Sambhara Suryaprakash, In PLoS ONE, 2000
... Endotoxin levels were measured using the ToxinSensor™ chromogenic LAL endotoxin assay kit from GenScript (Piscataway, NJ) ...
Papers on LAL
Macrophage-mediated cholesterol handling in atherosclerosis.
Orekhov et al., Moscow, Russia. In J Cell Mol Med, Jan 2016
Lysosomal acid lipase (LAL) hydrolyses cholesterol esters that are enriched in LDL to free cholesterol and free fatty acids.
EliA is required for inducing the stearyl alcohol-mediated expression of secretory proteins and production of polyester in Ralstonia sp. NT80.
Ishizuka et al., In Microbiology, Jan 2016
Comparative proteomic analysis of extracellular proteins from NT80 cells, grown in the presence or absence of stearyl alcohol, revealed that stearyl alcohol induced expressions of several secretory proteins including lipase (LipA), hemolysin-coregulated protein (Hcp), and nucleoside diphosphate kinase (Ndk).
The Type II Secreted Lipase/Esterase LesA is a Key Virulence Factor Required for Xylella fastidiosa Pathogenesis in Grapevines.
Dandekar et al., Davis, United States. In Sci Rep, Dec 2015
Analysis of the Xf secretome revealed a putative lipase/esterase (LesA) that was abundantly secreted in bacterial culture supernatant and was characterized as a protein ortholog of the cell wall-degrading enzyme LipA of Xanthomonas strains.
Interspecies Complementation of the LuxR Family Pathway-Specific Regulator Involved in Macrolide Biosynthesis.
Yoon et al., South Korea. In J Microbiol Biotechnol, Dec 2015
UNASSIGNED: PikD is a widely known pathway-specific regulator for controlling pikromycin production in Streptomyces venezuelae ATCC 15439, which is a representative of the large ATP-binding regulator of the LuxR family (LAL) in Streptomyces sp.. RapH and FkbN also belong to the LAL family of transcriptional regulators, which show greatest homology with the ATP-binding motif and helix-turn-helix DNA-binding motif of PikD.
Constitutive overexpression of asm18 increases the production and diversity of maytansinoids in Actinosynnema pretiosum.
Shen et al., Jinan, China. In Appl Microbiol Biotechnol, Dec 2015
Real-time PCR analysis showed that the overexpression of the asm18 gene selectively increased the transcription levels of the genes involved in the biosynthesis of the starter unit (asm43), polyketide assembly (asmA), post-PKS modification (asm21), as well as the transcription levels of the regulatory gene (asm8), which is a specific LAL-type activator in ansamitocin biosynthesis.
A Phase 3 Trial of Sebelipase Alfa in Lysosomal Acid Lipase Deficiency.
Quinn et al., Chicago, United States. In N Engl J Med, Oct 2015
BACKGROUND: Lysosomal acid lipase is an essential lipid-metabolizing enzyme that breaks down endocytosed lipid particles and regulates lipid metabolism.
Lysosomal Acid Lipase Deficiency
Murray et al., Seattle, United States. In Unknown Journal, Aug 2015
CLINICAL CHARACTERISTICS: The phenotypic spectrum of lysosomal acid lipase (LAL) deficiency ranges from the infantile-onset form (Wolman disease) to later-onset forms collectively known as cholesterol ester storage disease (CESD).
Rare Genetic Disorders Altering Lipoproteins
Shapiro, Madagascar. In Unknown Journal, Jul 2015
The aim of this chapter is to examine other, less common genetic disorders including elevations of lipoprotein(a); disorders of high density lipoprotein (familial hypoalphalipoproteinemia, Tangier disease, and LCAT deficiency); familial hypocholesterolemias (familial hypobetalipoproteinemia, abetalipoproteinemia, PCSK9 loss of function mutations, familial combined hypolipidemia, and chylomicron retention disease); ß-sitosterolemia; cerebrotendinous xanthomatosis, and lysosomal acid lipase deficiency.
[Screening for mutants with thermostabe lipase A from Burkholderia sp. ZYB002].
Shu et al., In Wei Sheng Wu Xue Bao, Jul 2015
OBJECTIVE: We improved the thermostability of LipA from Burkholderia cecapia ZYB002 by protein engineering technology to expand the application of lipase LipA.
Aging. Lysosomal signaling molecules regulate longevity in Caenorhabditis elegans.
Wang et al., Houston, United States. In Science, Feb 2015
In the worm Caenorhabditis elegans, the lysosomal acid lipase LIPL-4 triggered nuclear translocalization of a lysosomal lipid chaperone LBP-8, which promoted longevity by activating the nuclear hormone receptors NHR-49 and NHR-80.
Effects of Light Intensity Activity on CVD Risk Factors: A Systematic Review of Intervention Studies.
Fenning et al., Rockhampton, Australia. In Biomed Res Int, 2014
The effects of light intensity physical activity (LIPA) on cardiovascular disease (CVD) risk factors remain to be established.
[Hormone-sensitive lipase/cholesteryl esterase from the adrenal cortex - structure, regulation and role in steroid hormone synthesis].
Trzeciak et al., In Postepy Biochem, 2014
Hormone-sensitive lipase/cholesteryl esterase (HSL), encoded by LIPE gene, plays a very important role in the metabolism of acylglycerols in the adipose tissue and cholesteryl esters in the adrenal cortex, gonads and placenta.
Cell-intrinsic lysosomal lipolysis is essential for alternative activation of macrophages.
Pearce et al., Saint Louis, United States. In Nat Immunol, 2014
We found that the uptake of triacylglycerol substrates via the scavenger receptor CD36 and their subsequent lipolysis by lysosomal acid lipase (LAL) was important for the engagement of elevated oxidative phosphorylation, enhanced spare respiratory capacity (SRC), prolonged survival and expression of genes that together define M2 activation.
Memory CD8(+) T cells use cell-intrinsic lipolysis to support the metabolic programming necessary for development.
Pearce et al., Saint Louis, United States. In Immunity, 2014
We have demonstrated that memory T cells rely on cell intrinsic expression of the lysosomal hydrolase LAL (lysosomal acid lipase) to mobilize FA for FAO and memory T cell development.
Macrophage-specific transgenic expression of cholesteryl ester hydrolase attenuates hepatic lipid accumulation and also improves glucose tolerance in ob/ob mice.
Ghosh et al., Richmond, United States. In Am J Physiol Endocrinol Metab, 2012
Transgenic overexpression of CEH in macrophages polarizes hepatic macrophages (Kupffer cells) to an anti-inflammatory M2 phenotype that attenuates hepatic lipid synthesis and accumulation.
Lysosomal lipase deficiency: molecular characterization of eleven patients with Wolman or cholesteryl ester storage disease.
Calandra et al., Reggio nell'Emilia, Italy. In Mol Genet Metab, 2012
study identified two novel mutations of LIPA gene in Wolman Disease patients which abolished the expression of LAL enzyme; also found that all cholesteryl ester storage disease patients carried the common mutant allele c.894G>A
Coordination of triacylglycerol and cholesterol homeostasis by DHR96 and the Drosophila LipA homolog magro.
Thummel et al., Salt Lake City, United States. In Cell Metab, 2012
magro encodes a LipA homolog that is secreted from the anterior gut into the intestinal lumen to digest dietary triacylglycerol.
Intragenic deletion as a novel type of mutation in Wolman disease.
Chung et al., New York City, United States. In Mol Genet Metab, 2011
a novel paternally inherited c.482delA mutation in exon 5 of Lipase A that results in a frameshift mutation at amino acid 161 is reported in a fatal case of Wolman disease
Gene profile of myeloid-derived suppressive cells from the bone marrow of lysosomal acid lipase knock-out mice.
Du et al., Indianapolis, United States. In Plos One, 2011
analysis of important gene pathways involved in the myeloid lineage cells towards MDSCs using lal-/- mouse model
Myeloid-specific expression of human lysosomal acid lipase corrects malformation and malfunction of myeloid-derived suppressor cells in lal-/- mice.
Du et al., Indianapolis, United States. In J Immunol, 2011
studies demonstrate that LAL in myeloid cells plays a critical role in maintaining normal hematopoietic cell development and balancing immunosuppression and inflammation
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