A Key Component Determination on Forming Fairy Tofu from the Leaf of Premna Puberula (Verbenaceae).

A key component of Fairy Tofu from leaves of Premna puberula Pamp. was determined by immediate constituent analysis and prescription dismantlement analysis. The results showed that pectin in leaves of P. puberula was the key component as forming Fairy Tofu. It was because the highest component was pectin in Fairy Tofu except water, the pectin extracting directly from the leaves of P. puberula could be formed the jelly like Fairy Tofu, and the leaves which pectin was hydrolyzed by pectinase could not be made Fairy Tofu.

Clinical Characteristics of Childhood Pompe Disease / 대한소아신경학회지

PURPOSE: Pompe disease is one of the glycogen storage diseases caused by a deficiency of acid alpha-glycosidase. This enzyme defect results in lysosomal glycogen accumulation in many tissues and shows a various spectrum of clinical features from early infantile hypotonia to mild weakness. For the investigation of the clinical characteristics of Pompe disease, we reviewed 6 cases of childhood Pompe disease diagnosed by muscle biopsy and acid alpha-glycosidase assay. METHODS: We reviewed the medical records of 6 childhood Pompe disease patients in Seoul National University Children's Hospital, retrospectively from January 2001 to October 2006. RESULTS: The age of the symptom onset was 1 month to 11 years(mean 2.2 years) and the diagnosis was made at the age of 8 months to 14 years(mean 5.3 years). The patients showed delayed motor development, motor weakness, hypotonia, cardiomegaly, hypertrophic cardiomyopathy, hepatomegaly, recurrent pulmonary infections but the severity was very diverse. Liver transaminase and CK levels were elevated in all of the patients. Their muscle biopsy showed the characteristic accumulation of purple colored glycogen granules and the degeneration of myofibers. CONCLUSION: Childhood Pompe disease had various clinical features and severities depending on the age of onset, organ involvement and the rate of progression. Enzyme replacement therapy may modify the disease courses, so we need to diagnose earlier for the treatment at an optimal time.

Removal of human B-like antigen on cynomolgus monkey RBC by ?-galactosidase treatment / 中国输血杂志

Objective To observe the effects of enzymolysis of human B like antigen by ? galactosidase treatment on the morphology and function of cynomolgus monkey red blood cell (RBC) and to evaluate the safety of the enzyme treated RBC in animal transfusion. Methods RBC with human B like antigen was treated by recombined ? galactosidase and the effect was evaluated by absorption elution test. Meanwhile, morphology and function of the treated RBC were examined and compared with pre treatment RBC. ? galactosidase treated RBC was labeled with FITC and then infused to blood group A cynomolgus monkeys. Flow cytometry was used to detect the survival of donor RBC in recipient’s blood. Blood chemistry and urinalysis of recipients were performed before and after transfusion. Results The human B like antigen of cynomolgus monkey RBC was obliterated by ? galactosidase treatment and the treated RBC maintained normal morphology and function. Survival at 24 h after transfusion was 84.6% and 68.1%. T 1/2 were 7d and 8d versus 13d in the controls. There were no change in blood chemistry and urinalysis of the recipients. Conclusion Enzymolysis of cynomolgus monkey RBC does not affect the cellular function and morphology. Transfusion of the enzyme treated human B like RBC into human A like cynomolgus monkeys is safe.