Acid sphingomyelinase
Ebolavirus Requires Acid Sphingomyelinase Activity and Plasma Membrane Sphingomyelin for Infection
... Acid sphingomyelinase (ASMase) converts the lipid sphingomyelin (SM) to phosphocholine and ceramide and has optimum activity at acidic pH. Normally, ASMase is located in lysosomes and endosomes, but ...
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Disease manifestations and burden of illness in patients with acid sphingomyelinase deficiency (ASMD)
... Keywords: Acid sphingomyelinase deficiency, ASMD, Burden of illness, Disease manifestations, Lysosomal storage disorder, Natural history, Niemann-Pick disease types A and B.. Background.[r] ...
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Essential role for acid sphingomyelinase-inhibited autophagy in melanoma response to cisplatin
... The sphingolipid metabolising enzyme Acid Sphingomyelinase (A-SMase) has been recently shown to inhibit melanoma progression and correlate inversely to tumour grade. In this study we have investigated the ...
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Identification of a distinct mutation spectrum in the SMPD1 gene of Chinese patients with acid sphingomyelinase-deficient Niemann-Pick disease
... Currently, the mainstay treatment for Niemann-Pick disease type A/B is symptomatic. Bone marrow trans- plantation has been performed on a small number of NPD patients and found to be beneficial only to NPD-B individuals ...
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Acid Sphingomyelinase Promotes Endothelial Stress Response in Systemic Inflammation and Sepsis
... of acid sphingomyelinase (SMPD1) with subsequent generation of the bioactive mediator ...Plasma-secreted sphingomyelinase activity, ceramide generation and lipid raft formation were measured in human ...
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Instigation of NLRP3 inflammasome activation and glomerular injury in mice on the high fat diet: role of acid sphingomyelinase gene
... as acid sphingomyelinase (Asm) or neutral sphingomyelinase (NSM) or by de novo synthesis via serine palmitoyltransferase (SPT) and ceramide synthase [8, ...the acid sphingomyelinase ...
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Acid Sphingomyelinase Deficiency Increases Susceptibility to Fatal Alphavirus Encephalomyelitis
... Sindbis virus (SV), an enveloped virus with a single-stranded, plus-sense RNA genome, is the prototype alphavirus in the Togaviridae family. In mice, SV infects neurons and can cause apoptosis of immature neurons. ...
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Intracerebral transplantation of mesenchymal stem cells into acid sphingomyelinase–deficient mice delays the onset of neurological abnormalities and extends their life span
... Types A and B Niemann-Pick disease (NPD) are lysosomal storage disorders resulting from loss of acid sphingomyelinase (ASM) activity. We have used a knockout mouse model of NPD (ASMKO mice) to evaluate the ...
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Levels of acid sphingomyelinase (ASM) in Caenorhabditis elegans in microgravity
... Both Amyotrophic Lateral Sclerosis (ALS) patients and astronauts in spaceflight suffer from muscle atrophy. Previous research suggests that the enzyme acid sphingomyelinase (ASM) may be involved in the ...
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Niemann Pick type B disease Identification of a single codon deletion in the acid sphingomyelinase gene and genotype/phenotype correlations in type A and B patients
... hydrolase, acid sphingomyelinase ...entire acid sphingomyelinase coding region from an Ashkenazi Jewish Type B patient was polymerase chain reaction- amplified, subcloned, and completely ...
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α-TEA-induced death receptor dependent apoptosis involves activation of acid sphingomyelinase and elevated ceramide-enriched cell surface membranes
... increased acid sphingomyelinase (ASMase) activity by 30 min, peaking at 4 hrs, which is correlated with ASMase translocation from cytosol to the cell surface ...
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Skin of atopic dermatitis patients shows disturbed β-glucocerebrosidase and acid sphingomyelinase activity that relates to changes in stratum corneum lipid composition
... A pivotal step in this ceramide conversion is the final process in which ceramide precursors – glucosylceramides and sphingomyelins – are extruded from the Stratum Granulosum (SG) keratinocytes into the extracellular ...
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Mevalonate inhibits acid sphingomyelinase activity, increases sphingomyelin levels and inhibits cell proliferation of HepG2 and Caco-2 cells
... reduced acid SMase activity by mevalonate ap- pears occurring mainly at post-translational ...of acid SMase mRNA by 20 % after incubation with the highest con- centration of mevalonate, the statistical ...
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Neutral sphingomyelinase-2, acid sphingomyelinase, and ceramide levels in COPD patients compared to controls
... Neutral sphingomyelinase-2 (nSMase-2) and acid SMase (aSMase), which hydrolyze sphingomyelin to produce ceramide, are activated by a range of cellular stresses, including inflammatory cytokines and ...
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Boswellic acid inhibits expression of acid sphingomyelinase in intestinal cells
... that acid SMase might play important roles in promoting the development of IBD, because inhi- bition of acid SMase resulted in significantly improve- ments of the inflammation in both animal and cell ...
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Chronic visceral acid sphingomyelinase deficiency (Niemann-Pick disease type B) in 16 Polish patients: long-term follow-up
... The chart review of patients’ medical records concerning the demographics, the first presented signs and symptoms, age at diagnosis, as well as biochemical (acid sphingomye- linase activity in leukocytes, cultured ...
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Acid sphingomyelinase as target of Lycium Chinense: promising new action for cell health
... The 16:0 SM, 18:1 SM, 24:0 SM, standards were pre- pared as reported by Lazzarini et al. [42]. Standards were dissolved in chloroform/methanol (9:1 vol/vol) at 10 μg/mL final concentration. The stock solutions were ...
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Liver acid sphingomyelinase inhibits growth of metastatic colon cancer
... These results, in addition to the increased tumor growth observed in Timp1–/– mice Figure 4F, suggest that the increased expression of TIMP1 in hMFs by metastatic tumors is involved in t[r] ...
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CLASSIFICATION, CHARACTERIZATION, TREATMENT, CARRIER DETECTION AND GENETIC BASIS OF NEIMAN-PICK DISEASE
... Neiman-pick disease is caused by lysosomal abnormality. As a result large complex molecules donot break down. These complex molecules start to gather in different organelles and cause additional attachment with the ...
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Cambinol, a novel inhibitor of neutral sphingomyelinase 2 shows neuroprotective properties
... Sphingolipids are a major component of eukaryotic cell membranes. They are enriched in lipid rafts, and are particularly abundant in the nervous systems of mammals [1]. Ceramides are a class of sphingolipid that play ...
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