نتایج جستجو برای: alpha glucosidase

تعداد نتایج: 207190  

Journal: :Clinical chemistry 1980
J L Potter H B Robinson J D Kramer I A Schafter

We present a case of glycogen storage disease type II (Pompe's disease) with the classical clinical presentation and characteristic electrocardiographic changes of this disorder. An acid maltase (EC 3.2.1.20) determination in the peripheral leukocytes revealed normal activity; however, acid maltase activity was completely absent in a pre-mortem skeletal muscle biopsy. Post-mortem studies showed...

Journal: :Journal of Nutritional Science and Vitaminology 2006

Journal: :Yakugaku zasshi : Journal of the Pharmaceutical Society of Japan 2003
Masayuki Yoshikawa Yutana Pongpiriyadacha Akinobu Kishi Tadashi Kageura Tao Wang Toshio Morikawa Hisashi Matsuda

In the course of our characterization studies on anti-obese and antidiabetogenic principles in medicinal foodstuffs, we found that the methanolic extract from the stems of Salacia chinensis (Hippocerateaceae) showed potent anti-hyperglycemic effects in oral sucrose or maltose-loaded rats, inhibitory effects on intestinal alpha-glucosidase, rat lens aldose reductase, formation of Amadori compoun...

Journal: :Muscle & nerve. Supplement 1995
A J Reuser M A Kroos M M Hermans A G Bijvoet M P Verbeet O P Van Diggelen W J Kleijer A T Van der Ploeg

Glycogen storage disease type II (GSD II/glycogenosis type II/Pompe's disease/acid maltase deficiency) is caused by the deficiency of lysosomal alpha-glucosidase resulting in lysosomal accumulation of glycogen. The disease is inherited as an autosomal recessive trait and is clinically heterogeneous. Early and late onset phenotypes are distinguished. Insight in the molecular nature of the lysoso...

Journal: :Molecular Therapy: the Journal of the American Society of Gene Therapy 2009
Caterina Porto Monica Cardone Federica Fontana Barbara Rossi Maria Rosaria Tuzzi Antonietta Tarallo Maria Vittoria Barone Generoso Andria Giancarlo Parenti

In spite of the progress in the treatment of lysosomal storage diseases (LSDs), in some of these disorders the available therapies show limited efficacy and a need exists to identify novel therapeutic strategies. We studied the combination of enzyme replacement and enzyme enhancement by pharmacological chaperones in Pompe disease (PD), a metabolic myopathy caused by the deficiency of the lysoso...

Journal: :Gut 1987
B Lembcke C Löser U R Fölsch J Wöhler W Creutzfeldt

Intestinal adaptation (small intestinal weight and length, weight of the caecum and of the residual colon) to feeding different doses (0-5-50-500 mg/kg bw) of the absorbable, competitive alpha-glucosidase inhibitors BAY m 1099 and BAY o 1248 for three, seven, or 28 days was studied in rats. With the highest dose of either inhibitor, a significant and time dependent growth of the caecum was obse...

Journal: :Glycobiology 2000
M F Pelletier A Marcil G Sevigny C A Jakob D C Tessier E Chevet R Menard J J Bergeron D Y Thomas

Glucosidase II is an ER heterodimeric enzyme that cleaves sequentially the two innermost alpha-1,3-linked glucose residues from N-linked oligosaccharides on nascent glycoproteins. This processing allows the binding and release of monoglucosylated (Glc(1)Man(9)GlcNAc(2)) glycoproteins with calnexin and calreticulin, the lectin-like chaperones of the endoplasmic reticulum. We have isolated two cD...

2009
Michael Beck

Pompe disease is a lysosomal storage disorder characterized by muscle weakness and cardiomyopathy. It shows a broad variability regarding the clinical severity as well as the age of onset. In the past, two different recombinant enzyme preparations have been developed for the treatment of Pompe patients: alpha-glucosidase, produced in rabbit milk, and alpha-glucosidase, produced in Chinese hamst...

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