Digestion of starch requires activities provided by 6 interactive small intestinal enzymes. Two of these are luminal endoglucosidases named a-amylases. Four are exo-glucosidases bound to the luminal surface of enterocytes. These mucosal activities were identified as 4 different maltases. Two maltase activities were associated with sucrase-isomaltase. Two remaining maltases, lacking other identi...

Glycogen storage disease type II (Pompe disease) causes death in infancy from cardiorespiratory failure due to acid alpha-glucosidase (GAA; acid maltase) deficiency. An AAV2 vector pseudotyped as AAV6 (AAV2/6 vector) transiently expressed high-level human GAA in GAA-knockout (GAA-KO) mice without reducing glycogen storage; however, in immunodeficient GAA-KO/SCID mice the AAV2/6 vector expressed...

1. The maltase, sucrase, isomaltase and palatinase activities of the chick small intestine are localized in particles that sediment when centrifuged at 100000g for 90min. 2. Solubilization of the particle-bound disaccharidases without loss of activity was achieved by digestion with papain. Trypsin was less effective and caused a preferential solubilization of the sucrase, isomaltase and palatin...

Pompe disease is a fatal genetic muscle disorder caused by a deficiency of acid a -glucosidase (GAA), a glycogen degrading lysosomal enzyme. GAA-deficient (AMD) Japanese quails exhibit progressive myopathy and cannot lift their wings, fly, or right themselves from the supine position (flip test). Six 4-wk-old acid maltase–deficient quails, with the clinical symptoms listed, were intravenously i...

The aim was to evaluate the activity of intestinal carbohydrases in patients with irritable bowel syndrome (IBS) symptoms food intolerance. Material and methods. 126 IBS (83 women 43 men, median age – 32.0 years (Q1–Q3: 27–38 years)) were examined. diagnosis established on basis Roman Criteria IV. A questionnaire conducted for all identify intolerances, according results which divided into thre...

1. The maltase of saliva and that of E. coli (B. coli communis) hydrolyze maltose but not alpha-methylglucoside or sucrose and are therefore to be considered glucomaltases. 2. Maltase is rapidly and completely inactivated and digested by trypsin.

Insectivorous/frugivorous passerine species studied so far lack the ability to modulate intestinal maltase activity, in contrast to galliformes. We tested for dietary modulation of small intestine (SI) enzymes including maltase in house sparrows to understand whether the difference between the galliformes on the one hand, and the passerines on the other, reflects a phylogenetic pattern (maltase...

Metabolic cardiomyopathies include amino acid, lipid and mitochondrial disorders, as well as storage diseases. A number of metabolic disorders are associated with both myopathy and cardiomyopathy. These include the glycogen storage diseases, ie, acid maltase deficiency (infantile, childhood, and adult onset), McArdle disease, and debrancher and brancher deficiencies. Disorders of lipid metaboli...

The interest of the experts on the aspects of a disorder can change over time for three reasons: 1. discovery of new therapies; 2. improved knowledge; 3. increased levels of scientific research. This concept particularly applies to the field of genetic muscular dystrophies, were we can distinguish disorders generally well known for a long time and disorders that have only recently attracted the...

1. The effects of Concanavalin A (Con A) on enzymes from the intestinal brush border were studied using membrane vesicles (BBMV) prepared from 3- and 6-week-old broiler chickens. 2. Maltase, sucrase, phytase, alkaline phosphatase and leucine aminopeptidase activities were assayed in BBMV in the absence (T0) or presence (T1) of Con A, or in the presence of casein (T2). Disaccharidase specific ac...