A ceramidase subtype that is active at acid pH. It plays an important role in sphingolipid degradation by catalyzing the lysosomal hydrolysis of ceramide to sphingosine and free fatty acid. Inherited deficiency of acid ceramidase activity results in FARBER LIPOGRANULOMATOSIS.
An enzyme that catalyzes the hydrolysis of a ceramidetrihexoside to a ceramidedihexoside plus galactose.
A ceramidase subtype that is active at neutral pH. It is found at high levels within the SMALL INTESTINE and in the BRAIN.
Amidohydrolases that are specific for the cleavage of the N-acyl linkage of CERAMIDES. Ceramidases are classified as acidic, neutral or basic according to the optimal pH with which they function.
A ceramidase subtype that is active at alkaline pH. It is found at high levels within the SMALL INTESTINE.
A sphingolipidosis subtype that is characterized by the histological appearance of granulomatous deposits in tissues. It results from the accumulation of CERAMIDES in various tissues due to an inherited deficiency of ACID CERAMIDASE.
Members of the class of neutral glycosphingolipids. They are the basic units of SPHINGOLIPIDS. They are sphingoids attached via their amino groups to a long chain fatty acyl group. They abnormally accumulate in FABRY DISEASE.
An amino alcohol with a long unsaturated hydrocarbon chain. Sphingosine and its derivative sphinganine are the major bases of the sphingolipids in mammals. (Dorland, 28th ed)
A class of membrane lipids that have a polar head and two nonpolar tails. They are composed of one molecule of the long-chain amino alcohol sphingosine (4-sphingenine) or one of its derivatives, one molecule of a long-chain acid, a polar head alcohol and sometimes phosphoric acid in diester linkage at the polar head group. (Lehninger et al, Principles of Biochemistry, 2nd ed)
Salts and esters of the 14-carbon saturated monocarboxylic acid--myristic acid.
An enzyme that catalyzes the hydrolysis of sphingomyelin to ceramide (N-acylsphingosine) plus choline phosphate. A defect in this enzyme leads to NIEMANN-PICK DISEASE. EC 3.1.4.12.
A family of glycoprotein cofactors that are required for the efficient catabolization of SPHINGOLIPIDS by specific acid hydrolases such as GLUCOSYLCERAMIDASE; GALACTOCEREBROSIDASE; BETA-N-ACETYLHEXOSAMINIDASE; and CEREBROSIDE-SULFATASE.
GLYCEROL esterified with a single acyl (FATTY ACIDS) chain.
Inborn errors of metabolism characterized by defects in specific lysosomal hydrolases and resulting in intracellular accumulation of unmetabolized substrates.
Derivatives of PHOSPHATIDIC ACIDS that lack one of its fatty acyl chains due to its hydrolytic removal.
Phosphoric acid esters of dolichol.
A group of four homologous sphingolipid activator proteins that are formed from proteolytic cleavage of a common protein precursor molecule referred to as prosaposin.
Messages between computer users via COMPUTER COMMUNICATION NETWORKS. This feature duplicates most of the features of paper mail, such as forwarding, multiple copies, and attachments of images and other file types, but with a speed advantage. The term also refers to an individual message sent in this way.
Tumors or cancer of the human BREAST.
One of the mechanisms by which CELL DEATH occurs (compare with NECROSIS and AUTOPHAGOCYTOSIS). Apoptosis is the mechanism responsible for the physiological deletion of cells and appears to be intrinsically programmed. It is characterized by distinctive morphologic changes in the nucleus and cytoplasm, chromatin cleavage at regularly spaced sites, and the endonucleolytic cleavage of genomic DNA; (DNA FRAGMENTATION); at internucleosomal sites. This mode of cell death serves as a balance to mitosis in regulating the size of animal tissues and in mediating pathologic processes associated with tumor growth.
The decrease in the cell's ability to proliferate with the passing of time. Each cell is programmed for a certain number of cell divisions and at the end of that time proliferation halts. The cell enters a quiescent state after which it experiences CELL DEATH via the process of APOPTOSIS.
Organized services to provide information on any questions an individual might have using databases and other sources. (From Random House Unabridged Dictionary, 2d ed)
Information intended for potential users of medical and healthcare services. There is an emphasis on self-care and preventive approaches as well as information for community-wide dissemination and use.
A malignant form of astrocytoma histologically characterized by pleomorphism of cells, nuclear atypia, microhemorrhage, and necrosis. They may arise in any region of the central nervous system, with a predilection for the cerebral hemispheres, basal ganglia, and commissural pathways. Clinical presentation most frequently occurs in the fifth or sixth decade of life with focal neurologic signs or seizures.
The discipline concerned with using the combination of conventional ALLOPATHIC MEDICINE and ALTERNATIVE MEDICINE to address the biological, psychological, social, and spiritual aspects of health and illness.
Congenital structural abnormalities of the mouth and jaws, including the dentition.

Specific and sensitive assay for alkaline and neutral ceramidases involving C12-NBD-ceramide. (1/51)

A fluorescent analogue of ceramide, C12-NBD-ceramide, was found to be hydrolyzed much faster than 14C-labeled ceramide by alkaline ceramidase from Pseudomonas aeruginosa and neutral ceramidase from mouse liver, while this substrate was relatively resistant to acid ceramidase from plasma of the horseshoe crab. The radioactive substrate was used more preferentially by the acid ceramidase. It should be noted that C6-NBD-ceramide, which is usually used for ceramidase assays, was hardly hydrolyzed by any of the enzymes examined, compared to C12-NBD-ceramide. For the alkaline and neutral enzymes, the Vmax and k (Vmax/Km) with C12-NBD-ceramide were much higher than those with 14C-ceramide. In contrast, for the acid enzyme these parameters with C12-NBD-ceramide were less than half those with the radioisotope-labeled substrate. It is noteworthy that the labeling of ceramide with NBD did not itself reduce the Km of the alkaline enzyme, but did that of the neutral enzyme. It was also found that C12-NBD-ceramide was preferentially hydrolyzed by the alkaline and neutral enzymes, but not the acid one, in several mammalian cell lines. This study clearly shows that the attachment of NBD, but not dansyl, increases the susceptibility of ceramide to alkaline and neutral enzyme, and decreases that to acid enzymes. Thus the use of this substrate provides a specific and sensitive assay for alkaline and neutral ceramidases.  (+info)

Molecular cloning, sequencing, and expression of the gene encoding alkaline ceramidase from Pseudomonas aeruginosa. Cloning of a ceramidase homologue from Mycobacterium tuberculosis. (2/51)

We previously reported the purification and characterization of a novel type of alkaline ceramidase from Pseudomonas aeruginosa strain AN17 (Okino, N., Tani, M., Imayama, S., and Ito, M. (1998) J. Biol. Chem. 273, 14368-14373). Here, we report the molecular cloning, sequencing, and expression of the gene encoding the ceramidase of this strain. Specific oligonucleotide primers were synthesized using the peptide sequences of the purified ceramidase obtained by digestion with lysylendopeptidase and used for polymerase chain reaction. DNA fragments thus amplified were used as probes to clone the gene encoding the ceramidase from a genomic library of strain AN17. The open reading frame of 2,010 nucleotides encoded a polypeptide of 670 amino acids including a signal sequence of 24 residues, 64 residues of which matched the amino acid sequence determined for the purified enzyme. The molecular weight of the mature enzyme was estimated to be 70,767 from the deduced amino acid sequence. Expression of the ceramidase gene in Escherichia coli, resulted in production of a soluble enzyme with the identical N-terminal amino acid sequence. Recombinant ceramidase was purified to homogeneity from the lysate of E. coli cells and confirmed to be identical to the Pseudomonas enzyme in its specificity and other enzymatic properties. No significant sequence similarities were found in other known functional proteins including human acid ceramidase. However, we found a sequence homologous to the ceramidase in hypothetical proteins encoded in Mycobacterium tuberculosis, Dictyostelium discoideum, and Arabidopsis thaliana. The homologue of the ceramidase gene was thus cloned from an M. tuberculosis cosmid and expressed in E. coli, and the gene was demonstrated to encode an alkaline ceramidase. This is the first report for the cloning of an alkaline ceramidase.  (+info)

Overexpression of acid ceramidase protects from tumor necrosis factor-induced cell death. (3/51)

Tumor necrosis factor (TNF) signals cell death and simultaneously induces generation of ceramide. To evaluate the contribution of ceramide to TNF-dependent cell death, we generated clones of the TNF-sensitive cell line L929 that constitutively overexpress human acid ceramidase (AC). Ceramidase, in concert with sphingosine kinase, metabolizes ceramide to sphingosine-1-phosphate (SPP), an inducer of proliferation. In response to TNF, parental L929 cells display a significant increase in intracellular ceramide correlated with an "atypical apoptosis" characterized by membrane blebbing, DNA fragmentation and degradation of poly(ADP-ribose) polymerase despite a lack of caspase activity. These features are strongly reduced or absent in AC-overexpressing cells. Pharmacological suppression of AC with N-oleoylethanolamine restored the accumulation of intracellular ceramide as well as the sensitivity of the transfectants to TNF, implying that an enhanced metabolization of intracellular ceramide by AC shifts the balance between intracellular ceramide and SPP levels towards cell survival. Correspondingly, inhibition of ceramide production by acid sphingomyelinase also increased survival of TNF-treated L929 cells.  (+info)

Bcl-2 overexpression prevents apoptosis induced by ceramidase inhibitors in malignant melanoma and HaCaT keratinocytes. (4/51)

We examined the biological effects of the ceramide analogues (1S,2R)-2-N-myristoylamino-1-phenyl-1-propanol (D-e-MAPP) and (1R,2R)-2-N-myristoylamino-1-(4-nitrophenyl)-1,3-propandiol (D-NMAPPD) on human HaCaT keratinocytes and human melanoma cells. We could demonstrate that D-e-MAPP and D-NMAPPD are able to suppress acid ceramidase activity. The elevation of the endogenous level of ceramide is followed by induction of apoptosis and suppression of proliferation in HaCaT keratinocytes. Moreover, we recently identified a group of human melanoma cell populations which are heterogeneously susceptible to C2-ceramide-mediated apoptosis. Studies with these melanoma cells revealed correlation between ceramide-mediated apoptosis and D-NMAPPD-induced apoptosis, confirming the effect of this inhibitor on ceramide signaling in human melanoma cells. These findings suggest ceramidase inhibitors as a potential new therapeutical class of antiproliferative and cytostatic drugs.  (+info)

The reverse activity of human acid ceramidase. (5/51)

An overexpression system was recently developed to produce and purify recombinant, human acid ceramidase. In addition to ceramide hydrolysis, the purified enzyme was able to catalyze ceramide synthesis using [14C]lauric acid and sphingosine as substrates. Herein we report detailed characterization of this acid ceramidase-associated "reverse activity" and provide evidence that this reaction occurs in situ as well as in vitro. The pH optimum of the reverse reaction was approximately 5.5, as compared with approximately 4.5 for the hydrolysis reaction. Non-ionic detergents and zinc cations inhibited the activity, whereas most other cations were stimulatory. Of note, sphingomyelin also was very inhibitory toward this reaction, whereas the anionic lipids, phosphatidic acid and phosphatidylserine, were stimulatory. Of various sphingosine stereoisomers tested in the reverse reaction, only the natural, D-erythro form could efficiently serve as a substrate. Using D-erythro-sphingosine and lauric acid as substrates, the reaction followed normal Michaelis-Menten kinetics. The Km and Vmax values toward sphingosine were 23.75 microM and 208.3 pmol/microg/h, respectively, whereas for lauric acid they were 73.76 microM and 232.5 pmol/microg/h, respectively. Importantly, the reverse activity was reduced in cell lysates from a Farber disease patient to the same extent as the acid ceramidase activity. Furthermore, when 12-(N-methyl-N-(7-nitrobenz-2-oxa-1,3-diazol-4-yl)) (NBD)-conjugated lauric acid and sphingosine were added to cultured lymphoblasts from a Farber disease patient in the presence of fumonisin B (1), the conversion to NBD-ceramide was reduced approximately 30% when compared with normal cells. These data provide important new information on human acid ceramidase and further document its central role in sphingolipid metabolism.  (+info)

Stress-induced apoptosis is impaired in cells with a lysosomal targeting defect but is not affected in cells synthesizing a catalytically inactive cathepsin D. (6/51)

The role of cathepsin D in stress-induced cell death has been investigated by using ovine fibroblasts exhibiting a missense mutation in the active site of cathepsin D. The cathepsin D (lysosomal aspartic protease) deficiency did not protect cells against toxicity induced by doxorubicin and other cytotoxic agents, neither did it protect cells from caspase activation. Moreover, the cathepsin D inhibitor, pepstatin A, did not prevent stress-induced cell death in human fibroblasts or lymphoblasts. The possible role of lysosomal ceramide or sphingosine-mediated activation of cathepsin D in apoptosis was also excluded by using human cells either overexpressing or deficient in acid ceramidase. However, a normal lysosomal function seems to be required for efficient cell death, as indicated by the finding that fibroblasts from patients with mucolipidosis II were partially resistant to staurosporine, sphingosine and TNF-induced apoptosis, suggesting a key role of lysosomes in cell death.  (+info)

Upregulation of the human alkaline ceramidase 1 and acid ceramidase mediates calcium-induced differentiation of epidermal keratinocytes. (7/51)

Extracellular calcium (Ca2+(o)) potently induces the growth arrest and differentiation of human epidermal keratinocytes (HEKs). We report that Ca2+(o) markedly upregulates the human alkaline ceramidase 1 (haCER1) in HEKs; and its upregulation mediates the Ca2+(o)-induced growth arrest and differentiation of HEKs. haCER1 is the human ortholog of mouse alkaline ceramidase 1 that we previously identified. haCER1 catalyzed the hydrolysis of very long-chain ceramides to generate sphingosine (SPH). This in vitro activity required Ca2+. Ectopic expression of haCER1 in HEKs decreased the levels of D-e-C(24:1)-ceramide and D-e-C(24:0)-ceramide but elevated the levels of both SPH and its phosphate (S1P), whereas RNA interference-mediated knockdown of haCER1 caused the opposite effects on the levels of these sphingolipids in HEKs. Similar to haCER1 overexpression, Ca2+(o) increased the levels of SPH and S1P, and this was attenuated by haCER1 knockdown. haCER1 knockdown also inhibited the Ca2+(o)-induced growth arrest of HEKs and the Ca2+(o)-induced expression of keratin 1 and involucrin in HEKs. In addition, the acid ceramidase (AC) was also upregulated by Ca2+(o); and its knockdown attenuated the Ca2+(o)-induced expression of keratin 1 and involucrin in HEKs. These results strongly suggest that upregulation of haCER1 and AC mediates the Ca2+(o)-induced growth arrest and differentiation of HEKs by generating SPH and S1P.  (+info)

Population genetic analysis of the N-acylsphingosine amidohydrolase gene associated with mental activity in humans. (8/51)

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Glioblastoma is considered as the most aggressive form of brain tumor with overall survival less than fifteen months after diagnosis, even with multimodal therapy. It constitutes a major percentage of malignant brain tumors, with over 10,000 new cases reported every year in the USA. Comprehensive proteomic study of the disease in our lab has revealed that acid ceramidase is a novel target for possible drug development. Previous in Vitro study has found that acid ceramidase inhibitors (e.g. carmofur) have better efficacy than the standard chemotherapeutic drug available. This study aims to identify the role of acid ceramidase in glioblastoma and the efficacy of acid ceramidase inhibitors in suppressing the tumor, which may potentially lead to the development of a new chemotherapeutic agent for treating glioblastoma. Herein, we have worked towards the development of xenografted zebrafish (Danio rerio) glioblastoma model. For which, we have developed a zebrafish experimental microinjection protocol, where
TY - JOUR. T1 - Acid Ceramidase Deficiency in Mice Results in a Broad Range of Central Nervous System Abnormalities. AU - Sikora, Jakub. AU - Dworski, Shaalee. AU - Jones, E. Ellen. AU - Kamani, Mustafa A.. AU - Micsenyi, Matthew C.. AU - Sawada, Tomo. AU - Le Faouder, Pauline. AU - Bertrand-Michel, Justine. AU - Dupuy, Aude. AU - Dunn, Christopher K.. AU - Xuan, Ingrid Cong Yang. AU - Casas, Josefina. AU - Fabrias, Gemma. AU - Hampson, David R.. AU - Levade, Thierry. AU - Drake, Richard R.. AU - Medin, Jeffrey A.. AU - Walkley, Steven U.. PY - 2017/4/1. Y1 - 2017/4/1. N2 - Farber disease is a rare autosomal recessive disorder caused by acid ceramidase deficiency that usually presents as early-onset progressive visceral and neurologic disease. To understand the neurologic abnormality, we investigated behavioral, biochemical, and cellular abnormalities in the central nervous system of Asah1P361R/P361R mice, which serve as a model of Farber disease. Behaviorally, the mutant mice had reduced ...
Cellular senescence is linked to chronic age-related diseases including atherosclerosis, diabetes, and neurodegeneration. Compared to proliferating cells, senescent cells express distinct subsets of proteins. In this study, we used cultured human diploid fibroblasts rendered senescent through replicative exhaustion or ionizing radiation to identify proteins differentially expressed during senescence. We identified acid ceramidase (ASAH1), a lysosomal enzyme that cleaves ceramide into sphingosine and fatty acid, as being highly elevated in senescent cells. This increase in ASAH1 levels in senescent cells was associated with a rise in the levels of ASAH1 mRNA and a robust increase in ASAH1 protein stability. Furthermore, silencing ASAH1 in pre-senescent fibroblasts decreased the levels of senescence proteins p16, p21, and p53, and reduced the activity of the senescence-associated β-galactosidase. Interestingly, depletion of ASAH1 in pre-senescent cells sensitized these cells to the senolytics
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Sucrose non-fermenting protein (SNF1)-related kinase (SNRK) is a homologue of the yeast Snf1 kinase, mutants of which fail to thrive when provided with non-fermentable carbon sources. In the mouse lung SNRK expression is specific to capillary endothelial cells [63]. SNRK is phosphorylated and activated by the LKB1 serine/threonine-protein kinase that regulates cell polarity and functions also as a tumor suppressor [64]. In view of the vascular abnormalities found in LKB1 null mice LKB1 has been placed in the VEGF signalling pathway [65]. Together, these lines of evidence suggest that SNRK is also in the VEGF signalling pathway.. Monoamine oxidase A (MAOA) degrades amine neurotransmitters including serotonin. MAOA is located on the X chromosome and its upregulation in IPAH suggests a possible involvement in the development IPAH given the female prederiliction of the disease.. Acid ceramidase or N-acylsphingosine amidohydrolase (ASAH1) cleaves ceramide into sphingosine and a free fatty acid. Both ...
FUNCTION: This gene encodes acid ceramidase, an enzyme that plays a central role in ceramide metabolism. The encoded protein undergoes proteolytic processing to generate a heterodimeric enzyme comprised of alpha and beta subunits that catalyzes the hydrolysis of sphingolipid ceramide into sphingosine and free fatty acid. The homozygous disruption of this gene leads to embryonic lethality in mice whereas the heterozygous animals exhibit a progressive lipid storage disease phenotype. [provided by RefSeq, Oct 2015 ...
Carmofur (INN) or HCFU (1-hexylcarbamoyl-5-fluorouracil) is a pyrimidine analogue used as an antineoplastic agent. It is a derivative of fluorouracil, being a lypophilic-masked analog of 5-FU that can be administered orally. Carmofur prodrug is ingested and taken up in the intestine, overcoming the problem of 5-FU degradation by dihydropyrimidine dehydrogenase. Once inside a cell, carmofur prodrug is converted into 5-FU. The mechanism of action of carmofur prodrug is traditionally thought to be the generation of 5-FU. However, carmofur is a highly potent acid ceramidase (AC) inhibitor. Ceramide influences cancer cell survival, growth and death. Inhibition of AC activity sensitizes tumor cells to the effects of antineoplastic agents and radiation. Product marketing for carmofur started in 1981. Carmofur has also been used as adjuvant chemotherapy for curatively resected colorectal cancer patients in China, Japan, and Finland for many years. Trials and meta-analyses have confirmed that the drug is ...
ASAH2 Blocking Peptide for PRS4743; find Sigma-Aldrich-SBP4743 MSDS, related peer-reviewed papers, technical documents, similar products & more at Sigma-Aldrich
An authentic small animal model of Farber disease would greatly facilitate research into disease mechanisms and aid in the development of effective therapies. Unfortunately, a complete knock‐out model of Farber disease resulted in early embryonic lethality (Li et al, 2002) and a reduction of ACDase activity in the ovaries of a conditional knock‐out resulted in oocyte apoptosis (Eliyahu et al, 2012). These data highlight the difficulty in generating mouse models of certain LSDs, in particular the lipid storage disorders. These data also suggest that normal ceramide levels and metabolism are critical for early embryonic development.. Significant progress has been made towards effective therapies for LSDs through the use of recombinant enzyme replacement, bone marrow transplantation (BMT), small molecule drugs and gene therapy. It is likely that Farber disease would be responsive to similar approaches. However, the lack of an authentic animal model of Farber disease has hindered this ...
Conclusions A significant percentage of moderate Farber Disease patients are likely diagnosed as having JIA. Given this finding, it is important to increase awareness of Farber Disease in the pediatric rheumatology community. Differential diagnosis can be made by accounting for the comparatively early onset, progressive symmetric arthritis, presence of subcutaneous nodules at the joints, scalp and/or spine, and an unusual, hoarse cry or voice (due to nodule formation on the larynx) in FD patients. Based on experience with bone marrow transplantation in Farber Disease2, enzyme replacement therapy could resolve or significantly attenuate the arthritis phenotype in patients. Such therapy is currently under development. We propose that Farber Disease can account for specific cases diagnosed as JIA, and that clinically guided genetic screening of the JIA population will reveal patients who can benefit from disease-specific care and treatment.. ...
Farber disease (also known as Farbers lipogranulomatosis, ceramidase deficiency, Fibrocytic dysmucopolysaccharidosis, and Lipogranulomatosis[1]:545) is an extremely rare (80 cases reported worldwide to this day[2]) autosomal recessive lysosomal storage disease marked by a deficiency in the enzyme ceramidase that causes an accumulation of fatty material sphingolipids leading to abnormalities in the joints, liver, throat, tissues and central nervous system. Normally, the enzyme ceramidase breaks down fatty material in the bodys cells. In Farber Disease, the gene responsible for making this enzyme is mutated. Hence, the fatty material is never broken down and, instead, accumulates in various parts of the body, leading to the signs and symptoms of this disorder. ...
This invention provides a novel ganglioside ceramidase which has a substrate specificity at least for GD1a, GM1, GM2 and GM3 and acts at least on GD1a, GM1, GM2 and GM3 and catalyzes the reaction of hydrolysis of ganglioside to lysoganglioside and fatty acid.This invention further provides a process for producing the novel ganglioside ceramidase which comprises cultivating ganglioside ceramidase producing strain belonging to the genus Nocardia in a culture medium and collecting ganglioside ceramidase from the culture.
Most children with the classic form of Farbers disease die by age two, usually from lung disease. Those with a milder form of the disease may live in
Introduction. In this study, we evaluated the anticancer activity of DM102 [(2R,3Z)-N-(1-hydroxyoctadec-3-en-2-yl)pivalamide], a novel inhibitor of the enzyme acid ceramidase (AC), a key regulator of ceramide metabolism, in combination with C6-ceramide (C6-cer) in human breast cancer cells. Ceramide, a bioactive sphingolipid and potent enhancer of apoptosis, is known to play a role in chemo- and radiation-induced cell death. AC tempers ceramide-induced apoptosis by hydrolysis, producing sphingosine and free fatty acid, thus reducing intracellular ceramide levels and providing substrate for sphingosine kinase, which converts sphingosine to sphingosine 1-phosphate. AC has emerged as a putative anticancer target following reports of upregulation in prostate cancer and in some breast tumors. In the present work, we determined whether the introduction of an AC inhibitor would enhance the apoptosis-inducing effects of C6-cer in breast cancer cells.. Methods. Cultured breast cancer cells were ...
Sidney Farber (September 30, 1903 - March 30, 1973) was an American pediatric pathologist. He is regarded as the father of modern chemotherapy, and the Dana-Farber Cancer Institute is named after him. He was born in Buffalo, New York to Jewish parents Simon and Matilda (Goldstein) Farber. He was the third oldest of 14 siblings. He was the younger brother of the noted philosopher and University of Buffalo professor Marvin Farber (1901-1980). Sidney Farber graduated from SUNY Buffalo in 1923. In the mid-1920s, Jewish students were often refused admission to US medical schools, prompting him to go to Europe. As Farber was fluent in German, he undertook his first year of medical school at the Universities of Heidelberg and Freiburg in Germany. Having excelled in Germany, Farber entered Harvard Medical School as a second-year student and graduated in 1927. After graduate training in pathology at Peter Bent Brigham Hospital (the predecessor of Brigham and Womens Hospital) in Boston, Massachusetts, ...
Teijaro, J.R., Turner, D., Nam, Q., Wherry, E.J., LeFrançois, L. and Farber, D.L. (2011) Cutting Edge: Tissue-retentive lung memory CD4 T cells mediate optimal protective responses to influenza. J. Immunol. 187:5510-14.. Sathaliyawala, T., Kubota, M., Yudanin, N., Turner, D., Camp, P., Thome, J.C., Bickham, K.L., Lerner, H., Goldstein, M., Sykes, M., Kato, T and Farber, D.L. (2013) Distribution and compartmentalization of human circulating and tissue-resident memory T cells. Immunity. 38: 187-197. (Also see preview article in same issue by Neuenhahn and Busch, D.H., Whole body anatomy of human T cells. Immunity. 38:10-12.). Turner, D.L., Bickham, K.L., Farber, D.L. and Lefrancois, L. (2013) Splenic priming of virus-specific CD8 T cells following influenza infection. J. Virol. 87:4496-506. Turner, D.L., Bickham, K.L., Thome, J.J.T., Kim, C., DOvidio, F., Wherry, E.J. and Farber, D.L. (2014) Lung resident niches for the generation and maintenance of influenza-specific memory T cells. Mucosal ...
A Veterinary vehicle designed by Farber Specialty Vehicles and built for Kaufman County Animal Awareness on a Coach chassis. Best of all No CDL Required! This model is available in Gasoline or Diesel. Farber is here to help you with a custom solution to all your specialty vehicle needs. Call us today at (800) 331-3188.. ...
The KRAB-zinc finger proteins (KRAB-ZFPs) represent an extremely large, but poorly understood, family of transcriptional regulators in mammals. (Sera) cells, endogenous KRAB-ZFPs do not. Rather, KRAB-ZFPs and KAP1 relocalise to novel nucleoplasmic foci that we possess termed KRAB- and KAP1-connected (KAKA) foci. HP1s can also concentrate in these foci and there is a close spatial […]. ...
Visit Healthgrades for information on Dr. Charles Farber, MD Find Phone & Address information, medical practice history, affiliated hospitals and more.
Katy Farber is a teacher, author, and founder of the blog, Non-Toxic Kids. She is also the author of two books about education, Why Great Teachers Quit and How We Might Stop the Exodus and Change the World with Service Learning. Katy has written for various news, parenting, non-profit and educational publications, including Moms Clean Air Force, Educational Leadership, CNNs
The goal of this study is to identify and validate a new biochemical marker for Farber disease from the plasma of affected patients. Read more about this study!
Overview (in German). Der Sphingolipidmetabolismus kann virale Infektionen und Immunantworten auf vielen Ebenen beeinflussen. In unserem Masernvirus (MV)-Infektionsmodell des zentralen Nervensystems (ZNS) beobachteten wir bereits die Folgen der sauren Sphingomyelinase (Asm)-Defizienz in Mäusen, nämlich eine verminderte Widerstandsfähigkeit gegenüber viraler Infektion, und entdeckten, dass sowohl genetische Defizienz, als auch pharmakologische Hemmung der Asm die Aktivität CD4+ Foxp3+ regulatorischer T-Zellen (Treg) erhöht (Hollmann et al., J. Immunol, 2016, 197:3130-3141). Nun wollen wir herausfinden, wie assoziierte Reaktionswege und Enzyme, für die teilweise Inhibitoren schon in klinischem Gebrauch sind, genutzt werden können, um den gegenteiligen Effekt zu erreichen, d.h. die antivirale Immunreaktion zu stärken. Vorläufige Daten weisen darauf hin, dass die MV-Infektion durch Inhibition der sauren Ceramidase (Acd) oder der Sphingosin-Kinase-1 gehemmt wird. In der kommenden ...
Im a default crier. I happy-cry, stress-cry, romantic-comedy-cry, regular-comedy-cry and, apparently, I even doctor-in-training-cry. Crying is a tough response, especially for someone who wants to take care of sick patients. My default reaction poses challenges for my future career. I had my first taste of this when a patient actor brought me to tears. An actor? Thats ridiculous, you might think. It was all pretend and yet, I broke down. Im ...
Team ZipCar, a group of cyclers put together for Dana Farber Cancer Institutes annual Pan Mass Challenge, a grueling bike-a-thon raising money for cancer research and treatment.
Farber Specialty Vehicles has been awarded U.S. Communities contract for Specialty Vehicles, Equipment and Accessories and any Related Equipment, Supplies and Services.
TY - JOUR. T1 - Regulation of steroidogenesis by p53 in macaque granulosa cells and H295R human adrenocortical cells. AU - Cherian-Shaw, Mary. AU - Das, Rituparna. AU - Vandevoort, Catherine A.. AU - Chaffin, Charles L.. PY - 2004/12. Y1 - 2004/12. N2 - Ovulation and formation of a functional corpus luteum in primates involve cascades of events, including increased progesterone synthesis and changes in granulosa cell proliferation. However, critical gaps remain in our understanding of how an ovulatory gonadotropin surge initiates these processes. To more fully elucidate changes in the cell cycle during luteal formation, the actions of the tumor suppressor p53 were examined. Rhesus macaque granulosa cells were isolated during controlled ovarian stimulation protocols before (nonluteinized) or after (luteinized) an ovulatory gonadotropin stimulus. Phosphorylated p53 protein was detected in the cytoplasm of granulosa cells before and after human chorionic gonadotropin (hCG) treatment, whereas ...
K00654 SPT; serine palmitoyltransferase [EC:2.3.1.50] K00654 SPT; serine palmitoyltransferase [EC:2.3.1.50] K00654 SPT; serine palmitoyltransferase [EC:2.3.1.50] K04708 E1.1.1.102; 3-dehydrosphinganine reductase [EC:1.1.1.102] K04710 CERS; ceramide synthetase [EC:2.3.1.24] K04710 CERS; ceramide synthetase [EC:2.3.1.24] K04710 CERS; ceramide synthetase [EC:2.3.1.24] K04710 CERS; ceramide synthetase [EC:2.3.1.24] K04710 CERS; ceramide synthetase [EC:2.3.1.24] K04710 CERS; ceramide synthetase [EC:2.3.1.24] K12348 ASAH1; acid ceramidase [EC:3.5.1.23] K12349 ASAH2; neutral ceramidase [EC:3.5.1.23] K01441 ACER1_2; alkaline ceramidase [EC:3.5.1.23] K01441 ACER1_2; alkaline ceramidase [EC:3.5.1.23] K04711 ACER3; dihydroceramidase [EC:3.5.1.-] K04712 DEGS; sphingolipid 4-desaturase/C4-monooxygenase [EC:1.14.19.17 1.14.18.5] K04712 DEGS; sphingolipid 4-desaturase/C4-monooxygenase [EC:1.14.19.17 1.14.18.5] K04714 SGMS; shingomyelin synthase [EC:2.7.8.27] K04714 SGMS; shingomyelin synthase [EC:2.7.8.27] ...
Ceramide plays an important role in regulating cell proliferation and apoptosis. Recent studies indicate that generation of ceramide in the intestine from sphingomyelin hydrolysis may be implicated in colon cancer development. The enzymes that catalyze the further hydrolysis of ceramide in the intestine have, however, not been well investigated. Our data reveal the existence of a ceramidase (EC 3.5.1.23) in rat intestinal mucosa with an optimal pH of 7.0. One milligram of mucosal protein is able to hydrolyze 44.0 ± 9.6 nmol of ceramide in 1 hr. The activity is low in the proximal duodenum and increases to a plateau in the proximal jejunum. The activity is then similar throughout the small intestine, until it declines in the distal part of ileum. Some activity is also detectable in the colon. The activity increases slightly in the presence of monomeric bile salt concentrations and sharply at the critical micellar concentration. Similar patterns were observed for both primary (taurocholate) and ...
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A Mammography vehicle designed by Farber Specialty Vehicles and built for Erie County Medical Center on a Rear Engine Farber Body chassis. Farber is here to help you with a custom solution to all your specialty vehicle needs. Call us today at (800) 331-3188.. ...
Farber (Japanese: ハーフェン Harfen) is a Valmese general and the boss of chapter 15 of Fire Emblem Awakening. Leading a band tasked with protecting Valm Harbor, Farbers group was, at the time of the arrival of Chrom and the Shepherds, cornering a resistance leader. Farber would eventually be defeated by the Shepherds.
In June 3, 1948, The New England Journal of Medicine published a study by Sidney Farber, MD, showing that a synthetic compound, 4-aminopteroylglutamic acid (aminopterin), could induce remissions in seriously ill children with acute leukemia.1 Although the study was small-just 16 children-10 showed clinical, hematologic, and pathologic evidence of improvement. Even though the childrens remissions were short-lived, Dr. Farbers findings were so groundbreaking-until then, no drug had proved effective against non-solid tumor cancers-they ushered in the era of chemotherapeutics, chemical agents that could stop cancer cells from growing and replicating, in effect killing them.. Aminopterin was later replaced by methotrexate, a similar but more effective folic acid antagonist with fewer side effects, which is still used today in the treatment of leukemia and other blood and solid tumor cancers. Nevertheless, the results of Dr. Farbers study earned him the title, the Father of Modern ...
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Temporary restorations often look almost as good as the final restoration. We want the patients to look at the temporary and say, Wow! This looks great and feels great. The final restoration will be better. but we need to work this all out in the temporary stage. Because temporary restorations look so good, patients sometimes delay getting a final crown or final bridge on their implants. The problem with that scenario is that bacteria can leak under the temporary restoration and that may affect the health of the implant in the long run. There is a set amount of time that a temporary restoration is designed to last, and people should not go beyond that timeframe. Typically six months is the longest amount of time that I would recommend that a patient keep a temporary restoration. They need to return to the office in a timely manner and get their new, permanent teeth.. Dr. Alan H. Farber. Periodontist. Offices in Hauppauge and Medford, NY. New Patients Welcome. www.longislandperio.com. ...
When you need extruded film and sheet, Farber Plastics Inc.is the supplier of choice. The Farber family has been extruding thermoplastic film and sheet since 1963, producing HIPS film and sheet since 1975. With a multi-million dollar expansion into PET (polyethylene terephthalate) roll stock in 2007, the company, with Lewis Farber as president, continues to grow and serve a variety of markets ...
The DF/HCC Connect:Science seminar series plans to involve speakers who are leaders in cancer biology, immune oncology and other fields from across the...
Scott has more than 38 years of experience in the areas of complex financial reporting, international trade, risk assessment and strategic financial transactions.
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This data article provides gene expression profiles, determined by using real-time PCR, of fibroblasts and keratinocytes treated with 0.01% and 0.001% extracts of neem plant (Azadirachta indica), local name Kohomba in Sri Lanka, harvested in Sri Lanka. For fibroblasts, the dataset includes expression profiles for genes encoding hyaluronan synthase 1 (HAS1), hyaluronan synthase 2 (HAS2), hyaluronidase-1 (HYAL1), hyaluronidase-2 (HYAL2), versican, aggrecan, CD44, collagen, type I, alpha 1 (COL1A1), collagen, type III, alpha 1 (COL3A1), collagen, type VII, alpha 1 (COL7A1), matrix metalloproteinase 1 (MMP1), acid ceramidase, basic fibroblast growth factor (bFGF), fibroblast growth factor-7 (FGF7), vascular endothelial growth factor (VEGF), interleukin-1 alpha (IL-1α), cyclooxygenase-2 (cox2), transforming growth factor beta (TGF-β), and aquaporin 3 (AQP3 ...
When David Hahns daughter was in high school, she had to complete an oral-history project on someone with an extraordinary life story.. For Hahn, one person immediately came to mind: Erich Farber.. He has a story that you really wont find from anyone else, said Hahn, chair of the Department of Mechanical and Aerospace Engineering at the University of Florida. He was truly remarkable.. Farber, who became a faculty member in the department in 1954 and eventually served as director of UFs Solar Energy and Energy Conversion Laboratory, passed away on April 9. He was 95.. He was a world-renowned expert, cutting-edge researcher and 36-year professor in solar-energy technologies. In addition, he earned military decorations in World War II and served as a mountain-climbing instructor and Boy Scout leader.. My dad had the ability to do just about anything, said his son, Webb Farber, and he did.. Erich Farber was passionate about researching and developing solar-energy technologies at a time ...
Stephen Farber is a very competent attorney who has been practicing law for 40 years. Mr. Farber is a Personal Injury lawyer specializing primarily in Motor Vehicle Accidents. He is licensed to practice in the state of Pennsylvania, the U.S. District Court, the Eastern District of Pennsylvania, and the Middle District of Pennsylvania.. Stephen graduated from Penn State University with a BA in Mathematics, and spent a year teaching Junior High School Math to save money for Law School. He earned his Juris Doctor from Temple University School of Law. While in Law School, Stephen had the rare opportunity to prosecute cases, as part of his coursework, when he attended a Prosecution Seminar taught by then District Attorney, Arlen Specter.. Mr. Farber joined Master Weinstein Moyer, P.C. in 1994, after spending several years at two prominent Philadelphia area law firms and seven years as a Partner in his own firm. In his earliest years as an attorney, Mr. Farber practiced Criminal Law in addition to ...
The epidermis is the outermost layer of skin that acts as a barrier to protect the body from the external environment and to control water and heat loss. This barrier function is established through the multistage differentiation of keratinocytes and the presence of bioactive sphingolipids such as c …
A Board Certified physician in Radiation Oncology, Dr. Leonard Farber has been in private practice for over a decade, recently serving as Chairman of Radiation Oncology at Staten Island University Hospital. Dr. Farber specializes in adult radiation oncology, with particular focus on breast cancer, prostate cancer and gastrointestinal malignancies, lymphomas, head and neck cancers and lung cancers. He is an expert in HDR brachytherapy for various types of cancers, including breast, skin and gynecologic malignancies, and intracranial and body radiosurgery.. After receiving his B.S. and B.A. at the State University of New York at Binghamton, Dr. Farber pursued his M.D. at the Mt. Sinai School of Medicine in New York. Following an internship in internal medicine at Beth Israel Medical Center in New York, he did his residency in Radiation Oncology at the Hospital of the University of Pennsylvania in Philadelphia, where he served as Chief Resident. Dr. Farber is a member of the American Society for ...
If not for the state-of-the-art medical equipment, the 15,000-square-foot Farber Center for Radiation Oncology could almost be mistaken for a spa.
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Volunteer Name: Adam Farber Project Location: Bulenga, Uganda Program Dates: July 2013 (2 weeks) Volunteered At: Orphanage and school What was your favorite memory of this trip? The kids. Theyre so great and so excited to have a guest teacher. Getting to know them over time and watching them learn was a great experience.  What was the most difficult thing you experienced? Figuring out how cautious to be with safety at night. As time went on it seemed fine to be out for a couple hours after dark if walking with a group, especially if with a local. For a later night in Kampala it was also easy to arrange a private driver for a reasonable fee. What was the one thing you wished you were better prepared for? Understanding what to worry about safety-wise. The quick answer is dont worry in Bulenga unless its been dark for a while and all the people have gone home. Of course there is no need to take unnecessary risks either, such as walking alone anytime at night or carrying lots of money or
This Includes the E1mzG27RsV0 download Naturalistic Philosophies of Experience: Studies in James, Dewey and Farber against Powerful Boss, Prim for Ms. Over one million sessions were various algorithm Blanche 2012 over a relevant executive that saw on September 29 at 7:03 language to oxygen. For the theology splendour Blanche variation, peripheries of not Red predicting download and exemplified times, evangelicals and languages been through significant Thousands, mixing in a coordination of characterisation around one practical text. The value was the advanced band components within the
And so to Connie. Yes, people on this thread are not flattering to Celia Farber (I would not use the word unkind myself). But theres a reason for that, in fact more than one. First, Ms Farber has done more than most to perpetuate the culture of death and destruction that is represented by the AIDS denialist movement in general, and the RA group in particular. When conduct so shameful occurs for so long, without any indications of repentance or regret, its perfectly appropriate to be critical. Second, even on this thread, and vastly more so on the denialists own Blogs, theres some extremely unkind things said about various people, including me, Seth and others, who take the time and trouble to fight the AIDS denialists. Perhaps Connie would wish to condemn also the abusive, defamatory, offensive, racist, sexist, homophobic and thoroughly nasty language that has been said about us, including by people who are closely linked to Ms Farber. There have been several occasions when she has ...
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Is too much big data causing analysis paralysis? Adam Silver suggests applying a clear data management structure to achieve a strong and sustainable ROI.
Our long-term goal is to define the mechanisms by which anti-cancer chemotherapeutic agents (ACCAs) induce anti-proliferative responses (proliferation inhibitio...
my husband was in Mercks PD-1 inhibitor ( keytruda) between Jan 2015 and June 2015. He was on the highest dose of 10mg/kg every 2 weeks. At the time, there was no clinical trial that would guarantee he gets a PD-1 inhibitor . There was only one that randomized between avastin or Opdivo blindly and we didnt want to get avastin at the time. Hence, we went with the keytruda study grouping solid tumors together at the highest dose. He never had a swelling. What happened was 2 month after enrolling, he started having subtle enhancements that the NO was not sure whether they are inflammation or recurrence. Things stayed this way with increased enhancements till in June we decided this cant be enhancement and he sat for a craniotomy that confirmed our doubts that this was definetly recurrence with minimal swelling or inflammation. The pathology was confirmed by Both Dana Farber and Johns Hopkins ...
Blogger Euan Semple recently highlighted a key point about Enterprise 2.0 adoption that ZDNets own Dan Farber also found worthy of note over the weekend. And that is that Enterprise 2.0 will happen in your organization entirely by itself, whether you encourage it, discourage it, or even consign it to benign neglect. Euan actually laid out three strategies in semi-tongue cheek form likely meant as a shot across the bow of complacent IT departments; whether you get out of the way, actively encourage it, or do absolutely nothing, Enterprise 2.0 platforms like blogs, wikis, and related social, emergent, freeform Web 2.0-style apps are coming to your company, and in fact are almost certainly there already.
Im here at an AttentionTrust luncheon, featuring a talk by Michael Goldhaber and some AttentionTrust and GestureBank related announcements. Dan Farber is here as well, so check his blog for related coverage (and his photos are included below).
Brownstein Hyatt Farber Schreck is a unique law firm. Walk into any of our offices and youll immediately recognize a different type of energy. Complacency doesnt have a place here. Flexibility and... ...
Petang semalam, mak sedara dia datang umah minta tunjukkan jalan ke Klinik Gigi di Seri Gombak.. so petang tu bawa la si Naufal jalan-jalan sekali.. so untuk asah skill mengambil gambar yang dah tumpul nie, ataicha bawa la kamera.. iya nak asah skill kena laa banyak ambik gambar.. baru dapat skill dalam mencari angle kan... tapi untuk awal-awal ni setting auto je ...
The ASAH1 Gene codes for acid ceramidase which is an enzyme found in lysosomes. The lysosome breaks down acid ceramidase and ... The lack of myelin resulting from the lack of acid ceramidase break down leads to nerve cell damage. Spinal muscular atrophies ... Park, Jae-Ho; Schuchman, Edward H. (December 2006). "Acid ceramidase and human disease". Biochimica et Biophysica Acta (BBA) - ... "Acid ceramidase deficiency associated with spinal muscular atrophy with progressive myoclonic epilepsy". Neuromuscular ...
... human acid ceramidase. Catalytic reactions and interactions with acid sphingomyelinase". The Journal of Biological Chemistry. ... Okino N, He X, Gatt S, Sandhoff K, Ito M, Schuchman EH (August 2003). "The reverse activity of human acid ceramidase". The ... Seelan RS, Qian C, Yokomizo A, Bostwick DG, Smith DI, Liu W (October 2000). "Human acid ceramidase is overexpressed but not ... Bär J, Linke T, Ferlinz K, Neumann U, Schuchman EH, Sandhoff K (March 2001). "Molecular analysis of acid ceramidase deficiency ...
However, carmofur is a highly potent acid ceramidase (AC) inhibitor. Ceramide influences cancer cell survival, growth and death ... "Acid ceramidase and its inhibitors: a de novo drug target and a new class of drugs for killing glioblastoma cancer stem cells ... "Acid ceramidase is a novel drug target for pediatric brain tumors". Oncotarget. 8 (15): 24753-24761. doi:10.18632/oncotarget. ... "Discovery of highly potent acid ceramidase inhibitors with in vitro tumor chemosensitizing activity". Scientific Reports. 3 ( ...
"Upregulation of the human alkaline ceramidase 1 and acid ceramidase mediates calcium-induced differentiation of epidermal ... Alkaline ceramidase 1 also known as ACER1 is a ceramidase enzyme which in humans is encoded by the ACER1 gene. ACER1 mediates ... Protein, Nucleic Acid, Enzyme. 47 (4 Suppl): 455-62. PMID 11915342. Houben E, Holleran WM, Yaginuma T, Mao C, Obeid LM, Rogiers ... Ito M, Okino N, Tani M, Mitsutake S, Mori K (Mar 2002). "[Molecular evolution of neutral ceramidase: signalling molecule and ...
In chronic myelogenous leukemia (CML), IRF8 regulates acid ceramidase to mediate CML apoptosis. IRF8 is highly expressed in ... "IRF8 regulates acid ceramidase expression to mediate apoptosis and suppresses myelogeneous leukemia". Cancer Res. 71 (8): 2882- ...
S1P is formed from ceramide, which is composed of a sphingosine and a fatty acid. Ceramidase, an enzyme primarily present in ... October 2000). "Oocyte apoptosis is suppressed by disruption of the acid sphingomyelinase gene or by sphingosine-1-phosphate ... Sphingosine Lipid signaling Sphingosine-1-phosphate receptor family (of 5) S1PR1 S1PR2 FTY720 Lysophosphatidic acid Mendelson, ... Progress in Nucleic Acid Research and Molecular Biology. 71: 493-511. doi:10.1016/s0079-6603(02)71049-0. ISBN 9780125400718. ...
2005). "Elevation of the level and activity of acid ceramidase in Alzheimer's disease brain". Eur. J. Neurosci. 20 (12): 3489- ... This gene encodes an N-acylethanolamine-hydrolyzing enzyme which is highly similar to acid ceramidase. Multiple transcript ... "Entrez Gene: ASAHL N-acylsphingosine amidohydrolase (acid ceramidase)-like". Human NAAA genome location and NAAA gene details ... Schulze H, Schepers U, Sandhoff K (2008). "Overexpression and mass spectrometry analysis of mature human acid ceramidase". Biol ...
... can be further hydrolyzed by acid ceramidase to form sphingosine and a free fatty acid, both of which are able to ... A ceramide is composed of sphingosine and a fatty acid. Ceramides are found in high concentrations within the cell membrane of ... Rotolo JA, Zhang J, Donepudi M, Lee H, Fuks Z, Kolesnick R (2005). "Caspase-dependent and -independent activation of acid ... Anandamide Ceramidase inhibitors Chemotherapeutic agents Fas ligand Endotoxin Homocysteine Heat Gamma interferon Ionizing ...
... and they identified acid ceramidase as the enzyme deficient in Farber's disease. In 1964, Moser returned to the Joseph P. ... Yasuo Kishimoto, who had helped Kuni Suzuki make the discovery of elevated very long chain fatty acids in Adrenoleukodystrophy ... Odone made an independent intellectual computation - namely that the use of erucic acid, which is C-22:1 (C-22 monounsaturated ... Bill Rizzo presented data that showed that adding oleic acid (C-18 monounsaturated) to fibroblast cultures of ALD patients ...
Fatty Acid Amide Hydrolase inhibitors), PEA (N-Acylethanolamide Acid Amidase inhibitors), and ceramide (Acid Ceramidase ... "Discovery of highly potent acid ceramidase inhibitors with in vitro tumor chemosensitizing activity". Science Reports 3:1035. ... "Selective N-acylethanolamine-hydrolyzing acid amidase inhibition reveals a key role for endogenous palmitoylethanolamide in ...
... acid ceramidase), an enzymatic product of ASAH1, gene mutated in Farber disease This disambiguation page lists articles ...
... acid ceramidase) 1 ASPH: encoding enzyme Aspartyl/asparaginyl beta-hydroxylase AZIN1: encoding protein Antizyme inhibitor 1 ... fatty acid binding protein 9, testis FAM167A: family with sequence similarity 167, member A FAM203B: family with sequence ...
... sialic acid storage disease Infantile free sialic acid storage disease Glycogen storage diseases Type II Pompe disease Type IIb ... These are LSDs: Sphingolipidoses Ceramidase Farber disease Krabbe disease Infantile onset Late onset Galactosialidosis ... Cystinosis is an LSD characterized by the abnormal accumulation of the amino acid cystine. Alternatively to the protein targets ... Chronic hexosaminidase A deficiency Glucocerebroside Gaucher disease Type I Type II Type III Sphingomyelinase Lysosomal acid ...
... ceramidase converts ceramide to sphingosine) and blocks the effects of TNF- and arachidonic acid on intracellular Ca2+ ... and heptadecanoic acid (C17H34O2; 17:0), elevated palmitoleic acid (POA; C16H30O2; 16:1, n-7), a conjugate acid of a ... where n-7 fatty acids are precursors for the production of omega-4 fatty acids like palmitolinoleic acid (16:2), and a ... The hydrolysis of NAE to free fatty acid (FFA) and ethanolamine (MEA) in animals, is catalyzed by fatty acid amide hydrolase ( ...
Presently, 7 human ceramidases encoded by 7 distinct genes have been cloned: acid ceramidase (ASAH1) - cell survival neutral ... Human neutral ceramidase (nCDase) is an enzyme that plays a critical role in colon cancer and there are currently no potent or ... Ceramidase at the US National Library of Medicine Medical Subject Headings (MeSH) EC 3.5.1.23 Biology portal v t e. ... Ceramidase (EC 3.5.1.23, acylsphingosine deacylase, glycosphingolipid ceramide deacylase) is an enzyme which cleaves fatty ...
NAAA is homologous to acid ceramidase (HUGO gene symbol: ASAH1), sharing 30% sequence identity at the amino acid level in ... its structural similarity to the more familiar acid ceramidase (AC) and functional similarity to fatty acid amide hydrolase ( ... NAAA's primary structure is nearly identical to that of acid ceramidase (AC), whose only difference is the substitution of Leu ... a novel member of the choloylglycine hydrolase family with structural and functional similarity to acid ceramidase". The ...
"Cooperative prosurvival activity by ERK and Akt in human alveolar macrophages is dependent on high levels of acid ceramidase ... Neutral ceramidase is an enzyme that in humans is encoded by the ASAH2 gene. GRCh38: Ensembl release 89: ENSG00000188611 - ... 2004). "Neutral ceramidase gene: role in regulating ceramide-induced apoptosis". Gene. 315: 113-22. doi:10.1016/S0378-1119(03) ... 2006). "Golgi alkaline ceramidase regulates cell proliferation and survival by controlling levels of sphingosine and S1P". ...
A brief, very comprehensible review is given in Gunstone, F. (1996) Fatty Acid and Lipid Chemistry, pp 43-44. Blackie Academic ... Finally, ceramide may be broken down by a ceramidase to form sphingosine. Sphingosine may be phosphorylated to form sphingosine ... Gangliosides have at least three sugars, one of which must be sialic acid. Inositol-containing ceramides, which are derived ... such as a fatty acid.[citation needed] Simple sphingolipids, which include the sphingoid bases and ceramides, make up the early ...
"Docosahexaenoic acid-derived fatty acid esters of hydroxy fatty acids (FAHFAs) with anti-inflammatory properties". Diabetes. 65 ... Sphingosine (Sph) is formed by the action of ceramidase (CDase) enzymes on ceramide in the lysosome. Sph can also be formed in ... Palmitic acid esters of hydroxy-stearic acids (PAHSAs) are among the most bioactive members able to activate G-protein coupled ... Docosahexaenoic acid ester of hydroxy-linoleic acid (DHAHLA) exert anti-inflammatory and pro-resolving properties. ...
Ceramidase. *Aspartylglucosaminidase. *Fatty acid amide hydrolase. *Histone deacetylase *Sirtuin. 3.5.2: Cyclic amides/. ...
This enzyme is also called N-succinyl-L-alpha,epsilon-diaminopimelic acid deacylase. This enzyme participates in lysine ... Kindler SH, Gilvarg C (December 1960). "N-Succinyl-L-2,6-diaminopimelic acid deacylase". The Journal of Biological Chemistry. ...
"Nucleic Acids Research. 29 (16): 3439-47. doi:10.1093/nar/29.16.3439. PMC 55849. PMID 11504882.. ...
Rhizopus acid proteinase. Now EC 3.4.23.21, rhizopuspepsin EC 3.4.23.10: Endothia acid proteinase. Now EC 3.4.23.22, ... ceramidase EC 3.5.1.24: choloylglycine hydrolase EC 3.5.1.25: N-acetylglucosamine-6-phosphate deacetylase EC 3.5.1.26: N4-(beta ... polar-amino-acid-transporting ATPase EC 3.6.3.22: nonpolar-amino-acid-transporting ATPase EC 3.6.3.23: oligopeptide- ... Identical to EC 3.5.1.25, N-acetylglucosamine-6-phosphate deacetylase EC 3.5.1.81: N-acyl-D-amino-acid deacylase EC 3.5.1.82: N ...
Roca, X. (2003-11-01). "Intrinsic differences between authentic and cryptic 5' splice sites". Nucleic Acids Research. 31 (21): ... "A neutral ceramidase, NlnCDase, is involved in the stress responses of brown planthopper, Nilaparvata lugens (Stål)". ... Nucleic Acids Research. 37 (Web Server): W189-W192. doi:10.1093/nar/gkp320. ISSN 0305-1048. PMC 2703896. PMID 19429896. López- ... Nucleic Acids Res. 18 (10): 3015-9. doi:10.1093/nar/18.10.3015. PMC 330832. PMID 2349097. Senapathy, P. (1986). "Origin of ...
Unlike acid and neutral SMases in the intestinal tract that are rapidly inactivated by pancreatic trypsin, alk-SMase is ... neutral ceramidase, and sphingolipid metabolites". Pediatr. Res. 61 (1): 61-6. doi:10.1203/01.pdr.0000250534.92934.c2. PMID ... Besides, ursodeoxycholic acid and probiotic VSL#3 may stimulate the expression of ENPP7 in the intestine. GRCh38: Ensembl ... Liu F, Cheng Y, Wu J, Tauschel HD, Duan RD (April 2006). "Ursodeoxycholic acid differentially affects three types of ...
The most potent in vitro inhibitor of acid ceramidase, B-13, recently revealed to be virtually inactive towards lysosomal acid ... ω-position of the fatty acid moiety of B-13 led to a significant reduction of potency towards acid ceramidase from cellular ... Potent Inhibition of Acid Ceramidase by Novel B-13 Analogues. Denny Proksch, Jan Jasper Klein, and Christoph Arenz ... Indeed, inhibitors of acid ceramidase have been reported to induce cell death and to display potentiating effects to classical ...
... is a lysosomal cysteine hydrolase that catalyzes the conversion of ceramide into fatty acid and sphingosine. This reaction ... Role of acid ceramidase in resistance to FasL: therapeutic approaches based on acid ceramidase inhibitors and FasL gene therapy ... Acid ceramidase (AC) is a lysosomal cysteine hydrolase that catalyzes the conversion of ceramide into fatty acid and ... Complete Acid Ceramidase ablation prevents cancer-initiating cell formation in melanoma cells. *Michele Lai1,2,3,4. , ...
Compare Acid Ceramidase ELISA Kits from leading suppliers on Biocompare. View specifications, prices, citations, reviews, and ... Acid Ceramidase ELISA Kits. The ELISA (enzyme-linked immunosorbent assay) is a widely used application for detecting and ... Your search returned 15 Acid Ceramidase ELISA ELISA Kit across 3 suppliers. ...
... Marie-Elaine ... "Blockade of Lysosomal Acid Ceramidase Induces GluN2B-Dependent Tau Phosphorylation in Rat Hippocampal Slices," Neural ...
Acid ceramidase subunit beta. B, D. 253. Homo sapiens. Mutation(s): 1 Gene Names: ASAH1, ASAH, HSD-33, HSD33. EC: 3.5.1.23 (PDB ... Acid ceramidase subunit alpha. A, C. 131. Homo sapiens. Mutation(s): 2 Gene Names: ASAH1, ASAH, HSD-33, HSD33. EC: 3.5.1.23 ( ... Human acid ceramidase (AC) is a lysosomal cysteine amidase, which has received a great deal of interest in recent years as a ... Human acid ceramidase (AC) is a lysosomal cysteine amidase, which has received a great deal of interest in recent years as a ...
Markedly perturbed hematopoiesis in acid ceramidase deficient mice. Shaalee Dworski, Alexandra Berger, Caren Furlonger, Joshua ... Markedly perturbed hematopoiesis in acid ceramidase deficient mice. Shaalee Dworski, Alexandra Berger, Caren Furlonger, Joshua ... Markedly perturbed hematopoiesis in acid ceramidase deficient mice Message Subject (Your Name) has forwarded a page to you from ... Folate dietary insufficiency and folic acid supplementation similarly impair metabolism and compromise hematopoiesis ...
FD is caused by mutations in the ASAH1 gene, resulting in deficient acid ceramidase (ACDase) activity. Patients with ACDase ... Acid Ceramidase Deficiency in Mice Leads to Severe Ocular Pathology and Visual Impairment Am J Pathol. 2019 Feb;189(2):320-338. ... FD is caused by mutations in the ASAH1 gene, resulting in deficient acid ceramidase (ACDase) activity. Patients with ACDase ...
Here we report the identification of six novel mutations in the acid ceramidase gene causi … ... autosomal recessively inherited sphingolipid storage disorder due to the deficient activity of lysosomal acid ceramidase, ... Expression of the mutated acid ceramidase cDNAs in COS-1 cells and subsequent determination of acid ceramidase residual enzyme ... Molecular analysis of acid ceramidase deficiency in patients with Farber disease Hum Mutat. 2001 Mar;17(3):199-209. doi: ...
Abstract 5484: Targeted inhibition of acid ceramidase and C6-ceramide exposure induces synergistic killing in cancer cells. ... Abstract 5484: Targeted inhibition of acid ceramidase and C6-ceramide exposure induces synergistic killing in cancer cells ... Abstract 5484: Targeted inhibition of acid ceramidase and C6-ceramide exposure induces synergistic killing in cancer cells ... Abstract 5484: Targeted inhibition of acid ceramidase and C6-ceramide exposure induces synergistic killing in cancer cells ...
H9 cells, a human T cell lymphoma cell line, were cultured in medium with the acid ceramidase inhibitor N-oleoyl-ethanolamine ( ... extended treatment with an acid ceramidase inhibitor modulates the metabolism of ceramide and selects for a radiation-resistant ... extended treatment with an acid ceramidase inhibitor modulates the metabolism of ceramide and selects for a radiation-resistant ... extended treatment with an acid ceramidase inhibitor modulates the metabolism of ceramide and selects for a radiation-resistant ...
... 2016. *Author(s): Diamanti ...
We identified acid ceramidase (ASAH1), a lysosomal enzyme that cleaves ceramide into sphingosine and fatty acid, as being ... or treated with acid ceramidase inhibitor (ACi), with or without D+Q for 48 h, whereupon cell viability was assessed by direct ...
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... Academic Article * Authors Version ... Acid ceramidase (AC) catalyzes ceramide breakdown to sphingosine, the precursor for S1P. We report for the first time that AC ...
5′ splice site; ASAH1; Farber disease; acid ceramidase; exon skipping; exonic splicing enhancer; polypyrimidine tract ...
This study aims to identify the role of acid ceramidase in glioblastoma and the efficacy of acid ceramidase inhibitors in ... This model system will be used to study if acid ceramidase inhibitors can cross the blood-brain barrier, and their role in ... Comprehensive proteomic study of the disease in our lab has revealed that acid ceramidase is a novel target for possible drug ... Previous in Vitro study has found that acid ceramidase inhibitors (e.g. carmofur) have better efficacy than the standard ...
Novel Acid Ceramidase inhibitors, and their use as anti-tumor and anti-inflammatory agents. Novel anti-tumor and anti- ... Acid Ceramidase inhibitors, and their use as anti-tumor and anti-inflammatory agents. Anti-tumor and anti-inflammatory agents ... Acid ceramidase inhibitors and their use as medicaments. The present invention concerns, in a first aspect, compounds of ... Acid ceramidase inhibitors and their use as medicaments. The present invention relates to compounds of Formula I, its ...
Acid ceramidase breaks down fats called ceramides into a fat called sphingosine and a fatty acid. These two breakdown products ... Molecular basis of acid ceramidase deficiency in a neonatal form of Farber disease: identification of the first large deletion ... Mutations in the ASAH1 gene lead to severe reduction in acid ceramidase, typically to below 10 percent of normal. As a result, ... The ASAH1 gene provides instructions for making an enzyme called acid ceramidase. This enzyme is found in cell compartments ...
Lucki, N.C.; Sewer, M.B. Genistein stimulates MCF-7 breast cancer cell growth by inducing acid ceramidase (ASAH1) gene ... Chlorogenic Acid. Chlorogenic acid, a non-flavonoid polyphenol, is a quinic acid conjugate of caffeic acid found in high levels ... Caffeic Acid. Caffeic acid (3,4-dihydroxycinnamic acid) is a widespread hydroxycinnamic acid, naturally found in many plant ... Cinnamic acids. caffeic acid, chlorogenic acid, CAPE, tannic acid. Stilbenes. resveratrol, piceatannol, isorhapontigenin, ...
... acid ceramidase; multidrug resistance; autophagy melanoma; sphingolipids; ceramides; acid ceramidase; multidrug resistance; ...
Acid ceramidase of macrophages traps herpes simplex virus in multivesicular bodies and protects from severe disease Journal: ... Acid ceramidase of macrophages traps herpes simplex virus in multivesicular bodies and protects from severe disease ...
The acid ceramidase enzyme is required to help break down certain fatty substances - called ceramides - in the body. Because ... Acid ceramidase deficiency is characterized by a unique plasma cytokine and ceramide profile that is altered by therapy. ... Acid ceramidase and the treatment of ceramide diseases: the expanding role of enzyme replacement therapy. Biochim Biophys Acta ... Alterations in this gene result in a deficiency of the enzyme acid ceramidase. Enzymes are specialized proteins that act to ...
Acid Ceramidase Deficiency in Mice Results in a Broad Range of Central Nervous System Abnormalities. American Journal of ... Acid Ceramidase Deficiency in Mice Results in a Broad Range of Central Nervous System Abnormalities. / Sikora, Jakub; Dworski, ... Sikora, J., Dworski, S., Jones, E. E., Kamani, M. A., Micsenyi, M. C., Sawada, T., ... Walkley, S. U. (2017). Acid Ceramidase ... title = "Acid Ceramidase Deficiency in Mice Results in a Broad Range of Central Nervous System Abnormalities", ...
We tested single nucleotide polymorphisms (SNPs) in the acid ceramidase gene (ASAH1) for association with subject adherence and ... We tested single nucleotide polymorphisms (SNPs) in the acid ceramidase gene (ASAH1) for association with subject adherence and ... Genetic Variation in Acid Ceramidase Predicts Non-completion of an Exercise Intervention. ... Genetic Variation in Acid Ceramidase Predicts Non-completion of an Exercise Intervention.. Frontiers in Physiology, 9 (). http ...
Acid Ceramidase. Brain Neoplasms. Cell Line, Tumor. Glioblastoma. Humans. Immunohistochemistry. Lysophospholipids. Neoplasm ... Acid ceramidase confers radioresistance to glioblastoma cells. Oncol Rep 2017 Oct;38(4):1932-1940 PMID: 28765947 PMCID: ... This study demonstrates that GBMs acquire resistance to radiation via upregulation of acid ceramidase (ASAH1) and sphingosine‑1 ...
Title : Acid Ceramidase Inhibition Evokes Tau Hyperphosphorylation in Rat Hippocampal Slices: Contribution of NMDA Receptors. ... placed on a nylon mesh in a liquid-gas interface chamber and treated for a period of 3 hours with an acid ceramidase inhibitor ... Guy Massicotte and his colleagues will propose that ceramidase overexpression may eventually protect the human brain against ... 2-amino-5-phosphonovaleric acid (AP-5) and the GluN2B (but not the GluN2A) receptor antagonist RO25-6981. On the other hand, ...
... which can systemically deliver siRNA targeting the acid ceramidase (AC) gene for cancer therapy. This triblock copolymer, ... A biodegradable amphiphilic and cationic triblock copolymer for the delivery of siRNA targeting the acid ceramidase gene for ... siRNA delivery, Cancer therapy, Acid ceramidase, Micelleplex, Polyphosphoester, genetics, Animals, Apoptosis, Biocompatible ... which can systemically deliver siRNA targeting the acid ceramidase (AC) gene for cancer therapy. This triblock copolymer, ...
... acid sphingomyelinase and NPC1, respectively. While the primary cause of these diseases differs, both share common biochemical ... acid sphingomyelinase and NPC1, respectively. While the primary cause of these diseases differs, both share common biochemical ... ACDase, acid ceramidase; ASMase, acid sphingomyelinase; CLAH, congenital lipoid adrenal hyperplasia; ER, endoplasmic reticulum ... Lucki, N. C., Li, D., Bandyopadhyay, S., Wang, E., Merril, A. H., and Sewer, M. B. (2012). Acid ceramidase (ASAH1) represses ...
Effect of acid ceramidase over-expression on H295R adrenal carcinoma cells. Student Presenter: Dashon LaKing McCollum. Major: ... Over-expression of the enzyme acid ceramidase (AC) has been observed in some cancer cell lines and primary tumors. The ... Published studies have shown that ACTH can also alter acid ceramidase expression, suggesting a relationship between a stress ...
acid ceramidase. K12349 neutral ceramidase. Genes. .g1{display:none;}. HSA: 125981(ACER1) 340485(ACER2) 427(ASAH1) 56624(ASAH2) ...
  • FD is caused by mutations in the ASAH1 gene, resulting in deficient acid ceramidase (ACDase) activity. (nih.gov)
  • The ASAH1 gene provides instructions for making an enzyme called acid ceramidase. (medlineplus.gov)
  • Mutations in the ASAH1 gene lead to severe reduction in acid ceramidase, typically to below 10 percent of normal. (medlineplus.gov)
  • Alves MQ, Le Trionnaire E, Ribeiro I, Carpentier S, Harzer K, Levade T, Ribeiro MG. Molecular basis of acid ceramidase deficiency in a neonatal form of Farber disease: identification of the first large deletion in ASAH1 gene. (medlineplus.gov)
  • We tested single nucleotide polymorphisms (SNPs) in the acid ceramidase gene ( ASAH1 ) for association with subject adherence and physiologic benefit with exercise training in two well-characterised randomised, controlled 8-month exercise interventions: STRRIDE I ( n = 239) and STRRIDE II ( n = 246). (gwu.edu)
  • This study demonstrates that GBMs acquire resistance to radiation via upregulation of acid ceramidase (ASAH1) and sphingosine‑1-phosphate (Sph-1P). (mcw.edu)
  • The ASAH1 Gene codes for acid ceramidase which is an enzyme found in lysosomes. (wikipedia.org)
  • The ASAH1 gene encodes in humans the acid ceramidase enzyme. (wikipedia.org)
  • SMA-PME is caused by mutation in the ASAH1 gene encoding acid ceramidase. (jle.com)
  • Farber disease is a rare lysosomal storage disease caused by mutations in the ASAH1 gene, resulting in deficiency of the lysosomal enzyme acid ceramidase. (medindia.net)
  • ASAH1 is a lipid hydrolase that catalyzes the conversion of ceramide (cer) into sphingosine (SPH) and a free fatty acid. (ptglab.com)
  • Farber disease is a rare, autosomal recessively inherited sphingolipid storage disorder due to the deficient activity of lysosomal acid ceramidase, leading to the accumulation of ceramide in cells and tissues. (nih.gov)
  • Here we report the identification of six novel mutations in the acid ceramidase gene causing Farber disease: three point mutations resulting in single amino acid substitutions, one intronic splice site mutation resulting in exon skipping, and two point mutations also leading to occasional or complete exon skipping. (nih.gov)
  • Bär J, Linke T, Ferlinz K, Neumann U, Schuchman EH, Sandhoff K. Molecular analysis of acid ceramidase deficiency in patients with Farber disease. (medlineplus.gov)
  • Farber disease is a rare autosomal recessive disorder caused by acid ceramidase deficiency that usually presents as early-onset progressive visceral and neurologic disease. (elsevier.com)
  • Enzyvant is simultaneously conducting preclinical studies for RVT-801, a recombinant form of human acid ceramidase (rhAC), to enable a clinical trial of rhAC as an enzyme replacement therapy in patients with Farber disease. (medindia.net)
  • RVT-801 is a recombinant form of human acid ceramidase (rhAC) that Enzyvant is developing for potential use as an enzyme replacement therapy in acid ceramidase deficiency, manifesting as Farber disease. (medindia.net)
  • Indeed, inhibitors of acid ceramidase have been reported to induce cell death and to display potentiating effects to classical radio- or chemo therapy in a number of in vitro and in vivo cancer models. (hindawi.com)
  • Herein, we report a novel B-13-derived scaffold for more effective inhibitors of acid ceramidase. (hindawi.com)
  • Previous in Vitro study has found that acid ceramidase inhibitors (e.g. carmofur) have better efficacy than the standard chemotherapeutic drug available. (uwm.edu)
  • This study aims to identify the role of acid ceramidase in glioblastoma and the efficacy of acid ceramidase inhibitors in suppressing the tumor, which may potentially lead to the development of a new chemotherapeutic agent for treating glioblastoma. (uwm.edu)
  • This model system will be used to study if acid ceramidase inhibitors can cross the blood-brain barrier, and their role in tumor suppression using imaging microscopy and mass spectrometric techniques. (uwm.edu)
  • Peripherally restricted fatty acid amide hydrolase (FAAH) inhibitors. (iit.it)
  • Antisense expression of Osr9-lox1 (as-r9lox1) decreased the amount of wound-induced (Z)-3-hexenal but increased levels of striped stem borer (SSB)-induced linolenic acid, JA, SA and trypsin protease inhibitors. (jove.com)
  • These entities named FIASMAs (Functional Inhibitors of Acid SphingoMyelinAse), therefore, can be potentially used to treat diseases associated with enhanced activity of ASM, such as Alzheimer's disease, major depression, radiation- and chemotherapy-induced apoptosis and endotoxic shock syndrome. (wikisource.org)
  • Our lab research efforts are focused on sphingolipids, pancreatic/prostate cancer, acid ceramidase, mitochondriotropic ceramides, lysomotropic acid ceramidase inhibitors. (musc.edu)
  • Several investigations have shown that acid ceramidase inhibitors are potential antiproliferative and cytostatic drugs for cancer chemotherapy. (inserm.fr)
  • The combinatorial chemistry approach for the discovery of acid ceramidase inhibitors requires the availability of a high-throughput enzyme assay. (inserm.fr)
  • The assay was validated for the identification and characterization of acidic ceramidase inhibitors by using several alpha-ketoamide ceramide analogues, whose inhibitory activity had been previously described. (inserm.fr)
  • However, introduction of weakly basic groups at the ω -position of the fatty acid moiety of B-13 led to a significant reduction of potency towards acid ceramidase from cellular extracts. (hindawi.com)
  • Acid ceramidase (AC) is a lysosomal cysteine hydrolase that catalyzes the conversion of ceramide into fatty acid and sphingosine. (nature.com)
  • AC tempers ceramide-induced apoptosis by hydrolysis, producing sphingosine and free fatty acid, thus reducing intracellular ceramide levels and providing substrate for sphingosine kinase, which converts sphingosine to sphingosine 1-phosphate. (aacrjournals.org)
  • Acid ceramidase breaks down fats called ceramides into a fat called sphingosine and a fatty acid. (medlineplus.gov)
  • The acid ceramidase enzyme is required to help break down certain fatty substances - called ceramides - in the body. (rarediseases.org)
  • Adiponectin receptors (ADIPORs) are integral membrane proteins controlling glucose and lipid metabolism by mediating, at least in part, a cellular ceramidase activity 1 which catalyzes the hydrolysis of ceramide to produce sphingosine and a free fatty acid (FFA). (pubmedcentralcanada.ca)
  • Adiponectin is a hormone secreted mainly from adipocytes 3 that stimulates glucose utilization 4 and fatty-acid oxidation 5 . (pubmedcentralcanada.ca)
  • The lysosome breaks down acid ceramidase and the fatty acid component is then used to produce myelin. (wikipedia.org)
  • AC is a cysteine amidase that degrades the sphingolipid messenger ceramide, into sphingosine and fatty acid. (iit.it)
  • The encoded protein catalyzes the synthesis and degradation of ceramide into sphingosine and fatty acid. (wikipedia.org)
  • Determination of Double Bond Positions in Polyunsaturated Fatty Acids using the Photochemical Paternò-Büchi Reaction with Acetone and Tandem Mass Spectrometry. (lipidmaps.org)
  • FUNCTION: May hydrolyze the sphingolipid ceramide into sphingosine CC and free fatty acid (By similarity). (univ-lyon1.fr)
  • Elongation of Very Long chain fatty acids-4 (ELOVL4) protein is a member of the ELOVL family of fatty acid elongases that is collectively responsible for catalyzing formation of long chain fatty acids. (frontiersin.org)
  • ELOVL4 is the only family member that catalyzes production of Very Long Chain Saturated Fatty Acids (VLC-SFA) and Very Long Chain Polyunsaturated Fatty Acids (VLC-PUFA) with chain lengths ≥28 carbons. (frontiersin.org)
  • The mouse ASAHL gene encodes N-acylethanolamine-hydrolyzing Acid Amidase (NAAA), a fatty acid amidase with maximal activity at acidic pH (1). (novusbio.com)
  • ADIPOQ-binding activates a signaling cascade that leads to increased AMPK activity, and ultimately to increased fatty acid oxidation, increased glucose uptake and decreased gluconeogenesis. (tcdb.org)
  • Stearic acid, also called octadecanoic acid, is one of the useful types of saturated fatty acids that comes from many animal and vegetable fats and oils. (hmdb.ca)
  • This compound belongs to the class of organic compounds known as long-chain fatty acids. (hmdb.ca)
  • These are fatty acids with an aliphatic tail that contains between 13 and 21 carbon atoms. (hmdb.ca)
  • Altered glucose ( 10 ), fatty acid ( 11 ), and amino acid metabolism ( 12 ) are reported in obese people, including an inability to adjust to fuel availability ( 13 , 14 ). (diabetesjournals.org)
  • Generation of free fatty acids from phospholipids regulates stratum corneum acidification and integrity. (springer.com)
  • By screening for novel paqr-2 suppressors, we identified mutations in genes involved in phosphatidylcholine synthesis ( cept-1, pcyt-1 and sams-1 ) and fatty acid metabolism ( ech-7 , hacd-1 , mdt-15 , nhr-49 and sbp-1 ). (prolekare.cz)
  • We then show genetic evidence that paqr-2 , phosphatidylcholines, sbp-1 and Δ9-desaturases form a cold adaptation pathway that regulates the increase in unsaturated fatty acids necessary to retain membrane fluidity at low temperatures. (prolekare.cz)
  • This model is supported by the observations that the paqr-2 suppressors normalize the levels of saturated fatty acids, and that low concentrations of detergents that increase membrane fluidity can rescue the paqr-2 mutant. (prolekare.cz)
  • For example, adaptation to cold in Drosophila correlates with changes in the phospholipid composition of cellular membranes, favoring a higher abundance of shorter and/or unsaturated fatty acids to maintain membrane fluidity [5] - [8] . (prolekare.cz)
  • Expression of the mutated acid ceramidase cDNAs in COS-1 cells and subsequent determination of acid ceramidase residual enzyme activity demonstrated that each of these mutations was the direct cause of the acid ceramidase deficiency in the respective patients. (nih.gov)
  • Alterations in this gene result in a deficiency of the enzyme acid ceramidase. (rarediseases.org)
  • Since Hsp70.1- BMP binding is indispensable for activating acid sphingomyelinase and producing ceramide to stabilize lysosomal membrane, dysfunction of Hsp70.1 and BMP presumably causes storage of sphingomyelin and deficiency of ceramide at the lysosomal membrane, leading to its destabilization in the postischemic CA1 neurons. (omicsonline.org)
  • 1991,"Molecular Basis of Acid Sphingomyelinase Deficiency in a Patient With Niemann-Pick Disease Type A." Biochem. (patentgenius.com)
  • Wasserstein MP, Aron A, Brodie SE, Simonaro C, Desnick RJ, McGovern MM. Acid sphingomyelinase deficiency: prevalence and characterization of an intermediate phenotype of Niemann-Pick disease. (medscape.com)
  • Niemann-Pick type A/B (NPA/B) and type C diseases Niemann-Pick type C (NPC) are progressive LSD caused by loss of function of distinct lysosomal-residing proteins, acid sphingomyelinase and NPC1, respectively. (frontiersin.org)
  • Niemann-Pick type A and B (NPA and NPB) diseases are caused by deficits in the activity of acid sphingomyelinase (ASMase), an enzyme that regulates lysosomal sphingomyelin (SM) homeostasis, while Niemann-Pick type C (NPC) disease is caused by mutations in NPC1 and NPC2 genes, resulting in functional defects in the lysosomal proteins NPC1 and NPC2, involved in cholesterol efflux from lysosomes. (frontiersin.org)
  • Accordingly, using the monkey brain tissues after in-vivo and in-vitro oxidative stresses , we studied molecular modifications of Hsp70.1 and its counterpart bis(monoacylglycero) phosphate (BMP), because these molecules are closely related to the lysosomal membrane stability by regulating acid sphingomyelinase. (omicsonline.org)
  • Barbon, 2005, "AAV8-mediated hepatic expression of acid sphingomyelinase corrects the metabolic defect in the visceral organs of a mouse model of Niemann-Pick disease," Mol. (patentgenius.com)
  • 2009, "Alterations of myelin-specific proteins and sphingolipids characterize the brains of acid sphingomyelinase-deficient mice, an animal model of Niemann-Pick disease type A." J. Neurochem. (patentgenius.com)
  • 1991, "Regional assignment of the human acid sphingomyelinase gene (SMPD1) by PCR analysis of somatic cell hybrids and in situ hybridization to 11. (patentgenius.com)
  • 2001, "Analysis of the lung pathology and alveolar macrophage function in the acid sphingomyelinase-deficient mouse model of Niemann-Pick disease. (patentgenius.com)
  • 2009, "Intracerebroventricular infusion of acid sphingomyelinase corrects CNS manifestations in a mouse model of Niemann-Pick A disease. (patentgenius.com)
  • Dodge, 2005, "Gene transfer of human acid sphingomyelinase corrects neuropathology and motor deficits in a mouse model of Niemann-Pick type A disease," PNAS 102(49):17822-7. (patentgenius.com)
  • We describe a hitherto unknown feature for 27 small drug-like molecules, namely functional inhibition of acid sphingomyelinase (ASM). (wikisource.org)
  • Acid sphingomyelinase (ASM, EC 3.1.4.12) is a lysosomal glycoprotein that catalyses the hydrolysis of sphingomyelin into ceramide and phosphorylcholine. (wikisource.org)
  • Alkaline sphingomyelinase (Alk-SMase) and neutral ceramidase (N-CDase) in the intestinal microvillar membrane are responsible for dietary sphingomyelin digestion. (lu.se)
  • Apoptotic effects of selected strains of lactic acid bacteria on a human T leukemia cell line are associated with bacterial arginine deiminase and/or sphingomyelinase activities. (springer.com)
  • Roles and regulation of secretory and lysosomal acid sphingomyelinase. (medscape.com)
  • To determine the efficacy and safety of treatment with Cereton (alfoscerate) in patients with mild cognitive impairment syndrome (MCI) and its effects during and after treatment on phosphatidyl-choline, sphingomyelin, and ceramide (a sphingolipids metabolite) contents and the activity of genes controlling the synthesis of enzymes (sphingomyelinase and ceramidase) involved in the sphingomyelin and ceramide metabolism. (springer.com)
  • Plasma phosphatidylcholine, sphingomyelin, and ceramide were estimated by thin layer chromatography, while expression of the sphingomyelinase and ceramidase genes was determined using the reverse transcriptase polymerase chain reaction. (springer.com)
  • Expression of the genes (acid sphingomyelinase and ceramidase) controlling ceramide metabolism decreased in most patients during Cereton treatment. (springer.com)
  • Doxepin mitigates noise induced neuronal damage in primary auditory cortex of mice via suppression of acid sphingomyelinase/ceramide pathway. (ptglab.com)
  • Inhibition of the lysosomal acid ceramidase can increase cellular ceramide levels and thus induce apoptosis. (hindawi.com)
  • In ceramide metabolism, ceramidases catalyze the breakdown of ceramide to sphingosine and the inhibition of ceramidase activity results in the intracellular accumulation of ceramide leading to apoptosis. (aacrjournals.org)
  • Herein, we report that modulation of ceramide metabolism by extended treatment with a ceramidase inhibitor allows cells to acquire resistance to radiation-induced apoptosis in a radiosensitive human T cell lymphoma cell line. (aacrjournals.org)
  • 3) A salvage pathway generates ceramides by recycling sphingosine via CerS, as the sphingosine is produced by the hydrolysis of ceramide catalyzed by ceramidase (CDase) [ 4 ]. (clinsci.org)
  • Metabolic labeling studies in fibroblasts of four patients showed that even though acid ceramidase precursor protein was synthesized in these individuals, rapid proteolysis of the mutated, mature acid ceramidase occurred within the lysosome. (nih.gov)
  • Both acidic ceramidase (aCDase) and neutral ceramidase (nCDase) activities declined after L- and H-UVB, but returned to normal only in L-UVB cells, with decreased CDase activities or mRNA or protein levels being sustained in H-UVB cells. (biomedsearch.com)
  • Heterozygous inheritance of a different set of autosomal dominant ELOVL4 mutations that leads to a full-length protein with single amino acid substitutions causes spinocerebellar ataxia 34 (SCA34), a late-onset neurodegenerative disease characterized by gait ataxia and cerebellar atrophy. (frontiersin.org)
  • In order to determine how E2 works, the scientists then used a variety of advanced technologies to identify the protein that E2 binds to, which is known as acid ceramidase 1 (abbreviated as ASAHI). (yonsei.ac.kr)
  • E2 binds to the protein acid ceramidase, which sets off a chain of cellular processes that suppress angiogenesis. (yonsei.ac.kr)
  • We show that 9-cis retinoic acid, a molecule important in cellular differentiation and the product of ALDH1A1 catalysis in vitamin A metabolism, stimulates the accumulation of pigment, TYR and MITF mRNAs, and TYR protein in melanoma cells and primary human epidermal melanocytes. (escholarship.org)
  • Journal Article] Molecular cloning and functional analysis of zebrafish neutral ceramidase. (nii.ac.jp)
  • Journal Article] Conserved amino acid residues in the COOH-terminal tail are indispensable for the correct folding and localization, and enzyme activity of neutral ceramidase. (nii.ac.jp)
  • O-glycosylation of mucin-like domain retains the neutral ceramidase on the plasma membranes as a type II integral membrane protein'Journal of Biological Chemistry. (nii.ac.jp)
  • Neutral ceramidase secreted by endothelial cells is released in part associated with caveolin-1. (nii.ac.jp)
  • A neutral ceramidase homologue from Dictyosterium discoideum exhibits an acidic pH optimum'Biochemical Journal. (nii.ac.jp)
  • Genomic structure and promoter analysis of the mouse neutral ceramidase gene. (nii.ac.jp)
  • SIMILARITY: Belongs to the neutral ceramidase family. (univ-lyon1.fr)
  • An acid SMase (aSMase) functions in lysosomes, while the neutral SMases (nSMases) are active at the plasma membrane. (caymanchem.com)
  • In the intestinal tract of control rats, the activity of Alk-SMase was profoundly higher than those of acid and neutral Wases. (lu.se)
  • Three different ceramidases (acid, neutral and alkaline) have been identified and appear to play distinct roles in sphingolipid metabolism. (jle.com)
  • The most potent in vitro inhibitor of acid ceramidase, B-13, recently revealed to be virtually inactive towards lysosomal acid ceramidase in living cells. (hindawi.com)
  • In contrast, a number of weakly basic B-13 analogues have been shown to accumulate in the acidic compartments of living cells and to efficiently inhibit lysosomal acid ceramidase. (hindawi.com)
  • In this study, we evaluated the anticancer activity of DM102 [(2R,3Z)-N-(1-hydroxyoctadec-3-en-2-yl)pivalamide], a novel inhibitor of the enzyme acid ceramidase (AC), a key regulator of ceramide metabolism, in combination with C6-ceramide (C6-cer) in human breast cancer cells. (aacrjournals.org)
  • These results suggest that the absence of intracellular accumulation of ceramide following irradiation, through the modulation of ceramide metabolism using an acid ceramidase inhibitor for extended periods of time in culture, represents one potential mechanism for the development of radioresistance. (aacrjournals.org)
  • A recent study has shown that deregulation of sphingolipid metabolism may contribute to the development of radiation resistance through upregulation of acid ceramidase ( 2 ). (aacrjournals.org)
  • Cloning of an alkaline ceramidase from Saccharomyces cerevisiae. (uniprot.org)
  • an acid SMase and an alkaline ceramidase activity. (lu.se)
  • 2009) Acid ceramidase upregulation in prostate cancer: role in tumor development and implications for therapy . (musc.edu)
  • Lipid metabolic studies were performed using [ 3 H]palmitic acid. (aacrjournals.org)
  • Acid ceramidase, an enzyme of the sphingolipid metabolic pathway, hydrolyzes the antiproliferative sphingolipid ceramide, to sphingosine, which is a substrate for sphingosine kinases ( 3 ). (aacrjournals.org)
  • From a biochemical perspective, we observed that Tau phosphorylation at the Ser262 epitope was highly increased in hippocampal slices pre-incubated with the ACI, an effect totally prevented by the global NMDA receptor antagonist D/L(-)-2-amino-5-phosphonovaleric acid (AP-5) and the GluN2B (but not the GluN2A) receptor antagonist RO25-6981. (neurologycongress.com)
  • This improvement in insulin sensitivity was not accompanied by changes in skeletal muscle mitochondrial oxidative capacity or oxidant emissions, nor were there changes in skeletal muscle ceramide, diacylglycerol, or amino acid metabolite levels. (diabetesjournals.org)
  • The cDNA contains a 2061 bp open reading frame, which encodes an enzyme of 686 amino acid residues, with a calculated molecular mass of 77.6 kDa. (jove.com)
  • The results of these alterations are reflected in the encoded amino acids sequence of the proteins or in any targeted binding site in the DNA sequence. (els.net)
  • 1) De novo synthesis begins in the cytosolic layer of the endoplasmic reticulum (ER) with the condensation of the amino acid serine and palmitoyl-coenzyme A (CoA) via serine palmitoyltransferase (SPT), generating 3-ketosphinganine. (clinsci.org)
  • Melanin, a complex polymer derived from the amino acid tyrosine, is the primary determinant of hair, skin and eye color in humans. (escholarship.org)
  • Recently, the pleiotropic actions of adiponectin were linked to the ceramide signaling pathway as ADIPOR1 and ADIPOR2 activation was shown to lower ceramide levels via the activation of a ceramidase activity measured in crude cell lysates 1 . (pubmedcentralcanada.ca)
  • Has also reverse hydrolysis activity, catalyzing synthesis of phytoceramide and dihydroceramide from palmitic acid and phytosphingosine or dihydrosphingosine. (uniprot.org)
  • As the biological effects of 9-cis retinoic acid are mediated by two classes of nuclear receptors, the retinoic acid receptors (RARs) and the retinoid X receptors (RXRs), we searched for and identified a putative RAR/RXR binding site within the promoter of MITF-A, an isoform of MITF not known previously to play a role in melanin synthesis. (escholarship.org)
  • Similarly, hydrolysis occurred in intact cultured cells that overexpressed acidic ceramidase. (inserm.fr)
  • Furthermore, we provide hints for an introduction of basic functional groups at an alternative site of the B-13 scaffold that do not interfere with acid ceramidase inhibition in vitro . (hindawi.com)
  • Site‐directed mutagenesis (SDM) aims to introduce precise alterations in any coding or noncoding deoxyribonucleic acid (DNA) sequence, usually in vitro . (els.net)
  • The invention relates to the field of molecular biology.The invention relates to the field of molecular biology.More particularly, the invention relates to a methodand a kit for the colorimetric detection of a targetnucleic acid in a sample in which the target nucleicacid is present in mixture with other sample materials,including other acids nucleics that differ from thetarget nucleic acid for the base sequence. (iit.it)
  • Our results suggest a new target for Tau dephosphorylation and provide an insight into the potential therapeutic effects of brain ceramidase in Alzheimer's disease and other Tau-related pathologies.Acknowledgements - This work was supported by the Canadian Research Chair in Molecular Neuropharmacology (Michel Cyr) and by the National Sciences and Engineering Council of Canada (Guy Massicotte). (neurologycongress.com)
  • Molecular cloning and characterization of a full-length complementary DNA encoding human acid ceramidase. (wikipedia.org)
  • The Molecular Medicine of Acid Ceramidase. (exponent.com)
  • The molecular physiology of uric acid homeostasis. (harvard.edu)
  • The catabolism of ceramide in mammals occurs in lysosomes through the activity of ceramidase. (jle.com)
  • Because we found that ADIPOR1 also possesses an intrinsic ceramidase activity, we suspect that the two distinct structures may represent key steps of the ADIPORs enzymatic activity. (pubmedcentralcanada.ca)
  • Ceramide is further metabolized to sphingosine and sphingosine-1-phosphate by acid ceramidase (AC, EC 3.5.1.23) and sphingosine kinases. (wikisource.org)
  • In contrast, two known polymorphisms had no effect on acid ceramidase activity. (nih.gov)
  • It is unclear whether the level of acid ceramidase activity is related to the severity of the disorder. (medlineplus.gov)
  • Here, we determined crystal structures of ADIPOR2 bound to a FFA molecule and demonstrate that ADIPOR2 possesses an intrinsic basal ceramidase activity enhanced by adiponectin. (pubmedcentralcanada.ca)
  • The ceramidase activity is low, however, and further studies will be required to fully characterize ADIPORs enzymatic parameters and their substrate specificity. (pubmedcentralcanada.ca)
  • The reverse activity of human acid ceramidase. (nii.ac.jp)
  • Critical evaluation of probiotic activity of lactic acid bacteria and their effects. (springer.com)
  • Zebrafish acid ceramidase: Expression in Pichia pastoris GS115and biochemical characterization. (annals.org)
  • Characterization of Coding Variation in the urate Transporter GLUT9, a Key Determinant of Serum Uric Acid: Absence of Gain-of-Function Phenotypes [Abstract] J Am Soc Nephrol. (harvard.edu)
  • NAAA is a member of the choloylglycine hydrolase family of enzymes, and is structurally similar to acid ceramidase (1). (novusbio.com)
  • Acid ceramidase (AC) catalyzes ceramide breakdown to sphingosine, the precursor for S1P. (unh.edu)
  • UWM Undergraduate Research Symposium: Role of Acid Ceramidase In Xenografted Zebrafish Glioblastoma Model. (uwm.edu)
  • Human acid ceramidase (AC) is a lysosomal cysteine amidase, which has received a great deal of interest in recent years as a potential target for the development of new therapeutics against melanoma and glioblastoma tumors. (rcsb.org)
  • H9 cells, a human T cell lymphoma cell line, were cultured in medium with the acid ceramidase inhibitor N-oleoyl-ethanolamine (NOE), starting at a concentration level of 10 μM, with gradual increases to an NOE concentration of 100 μM. (aacrjournals.org)
  • Guy Massicotte and his colleagues will propose that ceramidase overexpression may eventually protect the human brain against Alzheimer's disease pathology by slowing-down Tau hyperphosphorylation, secretion and aggregation. (neurologycongress.com)
  • Insulin: Genetic and Physiological Influences on Human Uric Acid Homeostasis. (harvard.edu)
  • Treatment with DM102 (10 μM) for 24 hr increased levels of 3 H-palmitic acid-labeled ceramide by 2-fold, compared to controls, in MDA-MB 231 breast cancer cells. (aacrjournals.org)
  • Acid ceramidase confers radioresistance to glioblastoma cells. (mcw.edu)
  • Lipoxygenase metabolites of arachidonic acid as second messengers for presynaptic inhibition of Aplysia sensory cells. (iit.it)
  • Methods and Results - Transverse rat hippocampal slices were prepared with a McIlwain tissue chopper, placed on a nylon mesh in a liquid-gas interface chamber and treated for a period of 3 hours with an acid ceramidase inhibitor (ACI). (neurologycongress.com)