Also glycogen storage disease type ii pompe disease is a defect in lysosomal metabolism as well although it is otherwise classified into e740 in icd 10 cystinosis is an lsd characterized by the abnormal accumulation of the amino acid cystine. General discussion lysosomal storage diseases are inherited metabolic diseases that are characterized by an abnormal build up of various toxic materials in the bodys cells as a result of enzyme deficiencies. What are lysosomal storage disorders gaucher disease is one of the most common lysosomal storage disorders lsds lsds are inherited disorders resulting from a lack of specific enzymes that break down certain lipids fats or carbohydrates sugars in the body cells. Lysosomal storage disorders are a group of more than 50 rare diseases they affect the lysosome a structure in your cells that breaks down substances such as proteins carbohydrates and old . Lysosomal storage diseases lsds are a group of over 70 diseases that are characterized by lysosomal dysfunction most of which are inherited as autosomal recessive traits
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