alpha mannosidase/nekroos

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Bilateral patellar dislocation associated with alpha-mannosidase deficiency.

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Mannosidosis is an extremely rare genetic disease characterized by a deficiency of the lysosomal enzyme, alpha-mannosidase. This enzyme is necessary for cleavage of mannose from many glycoproteins. In the absence of this enzyme, mannose accumulates in cells throughout the body, including the joints

Differential augmentation by recombinant human tumor necrosis factor-alpha of neutrophil responses to particulate zymosan and glucan.

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Zymosan (Z) and its major insoluble carbohydrate component beta-linked glucan activate human neutrophils (PMN) through a trypsin-sensitive recognition mechanism. This mechanism is believed to involve the PMN CR3R. Both Z and glucan generated dose and time-dependent release of the secondary lysosomal

Measurement of alpha-glucosidase activity in serum from patients with cystic fibrosis or pancreatitis.

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We measured the activity of a non-lysosomal alpha-glucosidase with pH optimum near 6.0 in serum from a wide variety of patients, using the fluorogenic substrate, 4-methylumbelliferyl-alpha-D-glucopyranoside. Acutely ill patients with cystic fibrosis (CF) demonstrated significant increases in

Spontaneous and experimental glycoprotein storage disease of goats induced by Ipomoea carnea subsp fistulosa (Convolvulaceae).

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Spontaneous and experimental poisoning with the swainsonine-containing and calystegine-containing plant Ipomoea carnea subsp fistulosa is described. Three of 8 goats presenting with emaciation, weakness, symmetrical ataxia, posterior paresis, proprioceptive deficits, abnormal posture, abnormal

Effect of Bu-Zhong-Yi-Qi-Tang on deficiency of N-glycan/nitric oxide and islet damage induced by streptozotocin in diabetic rats.

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OBJECTIVE To investigate the effect of Bu-Zhong-Yi-Qi-Tang (Decoction for Reinforcing Middle Jiao and Replenishing Qi) on deficiency of N-glycan/nitric oxide (NO) and islet damage induced by injecting two medium doses of streptozotocin (STZ). METHODS Diabetes was induced by intraperitoneal injection

Release from intracellular enzymes from cutaneous cells after non-necrotizing damage by ultraviolet light.

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Experimental blisters were produced with suction on normal human skin and simultaneously on skin inflamed after exposure to middle wave ultraviolet light. Total proteins and marker enzymes for the plasma membrane, cytosol, lysosomes, peroxisomes, mitochondria, and microsomes were assayed in the

Reversible cellular damage by dimethyl sulfoxide reflected by release of marker enzymes for intracellular fractions.

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Irritative human skin reactions were induced by dimethyl sulfoxide (DMSO). Suction blisters were raised on these areas within 1.5 h after their induction and simultaneously on normal skin. The activities of marker enzymes for subcellular fractions in the suction blisters were determined. In suction

Kujigamberol interferes with pro-inflammatory cytokine-induced expression of and N-glycan modifications to cell adhesion molecules at different stages in human umbilical vein endothelial cells.

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Kujigamberol is the norlabdane compound isolated from Kuji amber and has recently been shown to prevent Ca2+-signal transduction and exert anti-allergy effects in vitro and in vivo. However, the anti-inflammatory activities of kujigamberol remain unclear. In the present study, we investigated the

Extracts of the rat tapeworm, Hymenolepis diminuta, suppress macrophage activation in vitro and alleviate chemically induced colitis in mice.

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Analysis of parasite-host interactions can reveal the intricacies of immunity and identify ways to modulate immunopathological reactions. We assessed the ability of a phosphate-buffered saline-soluble extract of adult Hymenolepis diminuta to suppress macrophage (human THP-1 cell line, murine

Increased activity of lysosomal acid hydrolases in the cell-free cerebrospinal fluid of bacterial meningitis.

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Because inflammation could affect lysosomal enzyme trafficking, resulting in increased enzyme release from the cells, tissue necrosis, or altered blood- and the brain-cerebrospinal fluid (CSF) barrier, the activity of four lysosomal enzymes in the cell-free CSF of 34 patients with bacterial

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