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Glycogen storage disease type II - Wikipedia Glycogen storage disease type II (GSD-II), also called Pompe disease, and formerly known as GSD-IIa or Limb–girdle muscular dystrophy 2V, is an autosomal recessive metabolic disorder [1] which damages muscle and nerve cells throughout the body It is caused by an accumulation of glycogen in the lysosome due to a deficiency of the lysosomal acid alpha-glucosidase enzyme (GAA) The inability
Home - AMDA Pompe Pompe disease is one of a family of 49 rare genetic disorders known as Lysosomal Storage Diseases or LSDs Pompe disease is also known as Acid Maltase Deficiency or Glycogen Storage Disease type II
Pompe’s disease - Encyclopedia Britannica Pompe’s disease, hereditary defect in the body’s ability to metabolize glycogen, resulting in a muscle disorder that is usually fatal during the first year of life The defect responsible, absence of the enzyme alpha-1,4-glucosidase, is extremely rare, occurring in fewer than one in every 150,000
Pompes Japy Pompes Japy, manufacturer of hand pumps, electric pumps, pneumatic pumps, submersible pumps, motor pumps and accessories for all types of liquids A complete range of water, seawater and charged water, fuel pumps
Les différentes variantes de pompes : Notre guide complet Les différentes variantes de pompes Rentrons dans le vif du sujet ! Découvrez les différentes variantes de pompes en image Nous avons passé en revue les exercices de pompes les plus populaires et nous vous expliquons quel est l’intérêt de chacune de ces déclinaisons
Pompes | Sulzer En tant qu'un des principaux fabricants mondiaux de pompes, Sulzer offre une vaste gamme de produits pour des solutions de pompage configurées, sur mesure et standards, ainsi que de l'équipement auxiliaire indispensable Nous sommes connus pour nos produits à la pointe de la technologie, la fiabilité de notre performance et l'efficacité de nos solutions