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GoPubMed Proteins lists recent and important papers and reviews for proteins. Page last changed on 19 Aug 2016.

N-acylsphingosine amidohydrolase

SOX3, acid ceramidase
This gene encodes a heterodimeric protein consisting of a nonglycosylated alpha subunit and a glycosylated beta subunit that is cleaved to the mature enzyme posttranslationally. The encoded protein catalyzes the synthesis and degradation of ceramide into sphingosine and fatty acid. Mutations in this gene have been associated with a lysosomal storage disorder known as Farber disease. Multiple transcript variants encoding several distinct isoforms have been identified for this gene. [provided by RefSeq, Jul 2008] (from NCBI)
Top mentioned proteins: ACID, CAN, Sox2, HAD, SRY
Papers on SOX3
Targeting acid ceramidase sensitises head and neck cancer to cisplatin.
Jang et al., Seoul, South Korea. In Eur J Cancer, Jan 2016
BACKGROUND: Acid ceramidase (AC), a key enzyme in ceramide metabolism, plays a role in cancer progression and resistance to therapy.
Atom based 3D-QSAR studies on 2,4-dioxopyrimidine-1-carboxamide analogs: Validation of experimental inhibitory potencies towards acid ceramidase.
Bhabak et al., Calcutta, India. In Eur J Pharm Sci, Jan 2016
Pharmacological inhibition of ceramidases and more particularly, acid ceramidase (aCDase) is suggestive of a chemotherapeutic approach as it increases the cellular concentration of ceramide inducing apoptosis.
Polyalanine expansions drive a shift into α-helical clusters without amyloid-fibril formation.
Hatters et al., Melbourne, Australia. In Nat Struct Mol Biol, Dec 2015
Such clustering was pervasive in cells irrespective of visible aggregate formation, and it disrupted the normal physiological oligomeric state of two human proteins natively containing polyAla: ARX and SOX3.
Recent advances in central congenital hypothyroidism.
Dattani et al., London, United Kingdom. In J Endocrinol, Dec 2015
Genetic ascertainment is possible in a minority of cases and reveals mutations in genes controlling the TSH biosynthetic pathway (TSHB, TRHR, IGSF1) in isolated TSH deficiency, or early (HESX1, LHX3, LHX4, SOX3, OTX2) or late (PROP1, POU1F1) pituitary transcription factors in combined hormone deficits.
Discovery of New Acid Ceramidase-Targeted Acyclic 5-Alkynyl and 5-Heteroaryl Uracil Nucleosides.
Raić-Malić et al., Zagreb, Croatia. In Acs Med Chem Lett, Dec 2015
Abrogation of acid ceramidase (ASAH1) expression of 9c and 9e indicated that these compounds could perturb ASAH1-mediated sphingolipid signaling.
Tamoxifen regulation of sphingolipid metabolism--Therapeutic implications.
Cabot et al., Greenville, United States. In Biochim Biophys Acta, Sep 2015
Additionally, tamoxifen and metabolites N-desmethyltamoxifen and 4-hydroxytamoxifen, have been shown to inhibit ceramide hydrolysis by the enzyme acid ceramidase.
The ART of Lowering Ceramides.
Summers, Melbourne, Australia. In Cell Metab, Sep 2015
Using a genetic mouse model to acutely degrade ceramides in adipose tissue or the liver (i.e., by conditionally expressing acid ceramidase), in this issue of Cell MetabolismXia et al. (2015) identify roles for these molecules in insulin resistance, steatohepatitis, and interorgan communication.
Targeted Induction of Ceramide Degradation Leads to Improved Systemic Metabolism and Reduced Hepatic Steatosis.
Scherer et al., Dallas, United States. In Cell Metab, Sep 2015
We have developed transgenic mice inducibly expressing acid ceramidase that display a reduction in ceramides in adult mouse tissues.
The molecular medicine of acid ceramidase.
Schuchman et al., In Biol Chem, Jun 2015
Acid ceramidase (N-acylsphingosine deacylase, EC;
Novel mechanisms of action of classical chemotherapeutic agents on sphingolipid pathways.
Haimovitz-Friedman et al., In Biol Chem, Jun 2015
In particular, it has been shown that activation of the acid ceramidase can lead to the formation of sphingosine 1-phosphate, which then antagonizes ceramide signaling by initiating a pro-survival signaling pathway.
Genetic Defects in Thyroid Hormone Supply
Macchia et al., Madagascar. In Unknown Journal, 2015
This situation can be determined by alterations in genes involved in ontogeny of the thyroid, including PIT1 and PRPO1, HESX1, LHX3, LHX4 and SOX3 as well as by mutation in the TSH-beta or in the immunoglobulin superfamily member 1 (IGSF1) genes.
Molecular Cloning, Promoter Analysis and Expression Profiles of the sox3 Gene in Japanese Flounder, Paralichthys olivaceus.
Zhang et al., Qingdao, China. In Int J Mol Sci, 2014
Sox3, which belongs to the SoxB1 subgroup, plays major roles in neural and gonadal development.
Acid sphingomyelinase-ceramide system mediates effects of antidepressant drugs.
Kornhuber et al., Essen, Germany. In Nat Med, 2013
Mice overexpressing Asm, heterozygous for acid ceramidase, treated with blockers of ceramide metabolism or directly injected with C16 ceramide in the hippocampus had higher ceramide concentrations and lower rates of neuronal proliferation, maturation and survival compared with controls and showed depression-like behavior even in the absence of stress.
Acid ceramidase-mediated production of sphingosine 1-phosphate promotes prostate cancer invasion through upregulation of cathepsin B.
Liu et al., Charleston, United States. In Int J Cancer, 2012
Acid ceramidase, through sphingosine 1-phosphate, promotes an invasive phenotype in prostate cancer by causing overexpression and secretion of cathepsin B through activation and nuclear expression of Ets1.
Spinal muscular atrophy associated with progressive myoclonic epilepsy is caused by mutations in ASAH1.
Melki et al., Le Kremlin-Bicêtre, France. In Am J Hum Genet, 2012
Our results reveal a wide phenotypic spectrum associated with ASAH1 mutations in spinal muscular atrophy associated with progressive myoclonal epilepsy.
Acid ceramidase as a chemotherapeutic target to overcome resistance to the antitumoral effect of choline kinase α inhibition.
Lacal et al., Madrid, Spain. In Curr Cancer Drug Targets, 2012
ASAH1 inhibition synergistically sensitizes lung cancer cells resistant to the antiproliferative effect of choline kinase alpha inhibitors.
Distinct lineage specification roles for NANOG, OCT4, and SOX2 in human embryonic stem cells.
Ivanova et al., New Haven, United States. In Cell Stem Cell, 2012
NANOG represses embryonic ectoderm differentiation but has little effect on other lineages, whereas SOX2 and SOX3 are redundant and repress mesendoderm differentiation.
Disruption of SoxB1-dependent Sonic hedgehog expression in the hypothalamus causes septo-optic dysplasia.
Epstein et al., Philadelphia, United States. In Dev Cell, 2012
It was shown that Sox2 and Sox3 are dose-dependent regulators of sonic hedgehog transcription that directly bind and activate a long-range Shh forebrain enhancer.
Congenital hydrocephalus and abnormal subcommissural organ development in Sox3 transgenic mice.
Thomas et al., Adelaide, Australia. In Plos One, 2011
Subcommissural organ function is essential for the prevention of hydrocephalus and overexpression of Sox3 in the dorsal midline alters progenitor cell differentiation in a dose-dependent manner.
SOX antibodies in small-cell lung cancer and Lambert-Eaton myasthenic syndrome: frequency and relation with survival.
Verschuuren et al., Leiden, Netherlands. In J Clin Oncol, 2009
PATIENTS AND METHODS: We used recombinant SOX1, SOX2, SOX3, SOX21, HuC, HuD, or HelN1 proteins in an ELISA to titrate serum samples and validated the assay by western blot.
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