| These cells contained many lysosomal and autophagous bodies and damaged mitochondria. |
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| There is a strong argument that similar specialist centres should be designated for other lysosomal storage disorders. |
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| This enzyme, a lysosomal hydrolase, is involved in the catabolism of neutral glycosphingolipids. |
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| They are thought to represent lysosomal globules, composed of apoptotic bodies phagocytosed by the tumor cells. |
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| The leukocytes eventually destroy the pathogens by phagocytosis and lysosomal fusion that expose bacteria to lytic enzymes. |
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| The osmotic gradient across the membranes can drive an influx of water, which causes the lysosomal lysis. |
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| However, at closer examination the other organelles are certainly mentioned, as they are implicated in lysosomal, peroxisomal, or mitochondrial diseases. |
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| Intracellular bacteria are subjected to several bacteriocidal mechanisms in the macrophages such as free oxygen radicals and phagosome lysosomal activity. |
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| These diseases include Tuberous Sclerosis Complex, PEComas and hemangiomas, metabolic diseases, lysosomal storage diseases. |
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| But hyperexcretion of lysosomal enzymes could reflect much more complex mechanisms. |
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| The novel neuronal ceroid lipofuscinosis gene MFSD8 encodes a putative lysosomal transporter. |
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| The second approach is to attempt to correct the lysosomal defect by a cholinergic agonist, bethanechol chloride. |
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| The company develops drugs that treat rare genetic diseases known as lysosomal storage disorders. |
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| At 24 hr, the C particulates were often in small clumps and without evidence of surrounding lysosomal membrane. |
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| Mucopolysaccharidoses represent a rare lysosomal storage disorder that can be detected early in the paediatric age group by MRI of the brain. |
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| Morquio A syndrome is a rare, autosomal recessive lysosomal storage disease caused by a deficiency in N-acetylgalactosamine-6-sulfate sulfatase. |
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| There are about 40 different lysosomal storage disorders, like multiple sulfatase deficiency suffered by Pavan Tailor. |
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| Oligosaccharide analysis in urine by MALDI-TOF mass spectrometry for the diagnosis of lysosomal storage disorders. |
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| Magnesium levels in the cell can regulate glycosylation and protein processing modulating golgi body and lysosomal function. |
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| Cathepsin L, a lysosomal endopeptidase, is a member of the papain-like family of cysteine proteinases. |
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| The modified protein is resistant to degradation by lysosomal enzymes and accumulates intracellularly. |
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| Pretreatment with a combination of quercetin and alpha-tocopherol ameliorates adenosine triphosphatases and lysosomal enzymes in myocardial infarcted rats. |
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| Since inception in 1997, BioMarin has applied its proprietary enzyme technology to develop products for lysosomal storage diseases and for the treatment of serious burns. |
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| The relationship between lysosomal biomarker and organismal responses in an acute toxicity test with Eisenia fetida exposed to the fungicide copper oxychloride. |
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| As storage cells were observed in the bone marrow aspiration specimen and lysosomal sphingomyelinase activity was low, the patient was diagnosed with Niemann-Pick disease. |
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| Sebelipase alfa is a recombinant form of the human LAL enzyme under development by Synageva as an enzyme replacement therapy for LAL Deficiency, a lysosomal storage disorder. |
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| Imino sugar inhibitors for treating the lysosomal glycosphingolipidoses. |
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| A similar situation exists for some of the lysosomal sulfatases in which the fluorescent substrate 4-methylumbelliferyl sulfate can be hydrolyzed by a number of sulfatases. |
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| Lysosomal sialidase cleans sialic acid residues located in the last part of gangliosides, oligosaccharides and glycoproteins. |
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