A FUSION PROTEIN ENZYME. USED AS A TARGETED ENZYME REPLACEMENT THERAPY DESIGNED TO ADDRESS THE UNDERLYING CAUSE OF HYPOPHOSPHATASIA (HPP) BY NORMALIZING THE GENETICALLY DEFECTIVE METABOLIC PROCESS, AND PREVENTING OR REVERSING THE SEVERE AND LIFE-THREATENING COMPLICATIONS OF LIFE-LONG DYSREGULATED MINERAL METABOLISM. COMPRISED OF THE SOLUBLE CATALYTIC DOMAIN OF HUMAN TISSUE NON-SPECIFIC ALKALINE PHOSPHATASE (SALP), THE HUMAN IMMUNOGLOBULIN G1 FC DOMAIN AND OF A DECA ASPARTATE PEPTIDE (D10). IT IS PRODUCED BY MAMMALIAN CELL CULTURE AND PURIFIED BY STANDARD BIOPROCESS TECHNOLOGY. A CLEAR COLOURLESS PRESERVATIVE-FREE SOLUTION AT 100MG/ML OR 40 MG/ML PACKAGED IN 2ML GLASS VIALS (SEVERAL FILL VOLUMES, BUT SAME VIAL). IT IS USED AS A FORMULATED MEDICINAL PRODUCT FOR SUBCUTANEOUS INJECTION. IT IS FORMULATED IN 25MM SODIUM PHOSPHATE, 150 MM SODIUM CHLORIDE AT PH 7.4. THE PREFORMULATED BDS IS SUPPLIED IN A 1L BAG AT A CONCENTRATION OF 100 MG/ML. THE BDS CAN BE FILLED AT THAT CONCENTRATION, OR DILUTED USING THE SAME FORMULATION BUFFER TO 40MG/ML. CAS NO. 1174277-80-5

CLASSIFICATION HAS BEEN DETERMINED IN ACCORDANCE WITH THE FOLLOWING:- GENERAL INTERPRETATIVE RULES (GIR)S GIR 1 HAS BEEN USED TO CLASSIFY THIS PRODUCT BY THE TERMS OF HEADING 3002 - HUMAN BLOOD; ANIMAL BLOOD PREPARED FOR THERAPEUTIC, PROPHYLACTIC OR DIAGNOSTIC USES; ANTISERA, OTHER BLOOD FRACTIONS AND IMMUNOLOGICAL PRODUCTS, WHETHER OR NOT MODIFIED OR OBTAINED BY MEANS OF BIOTECHNOLOGICAL PROCESSES; VACCINES, TOXINS, CULTURES OF MICRO-ORGANISMS(EXCLUDING YEASTS) AND SIMILAR PRODUCTS. GIR 5B HAS BEEN USED TO IDENTIFY THE TYPE OF PACKAGING GIR 6 HAS BEEN USED TO CLASSIFY THE GOODS TO SUBHEADING LEVEL 300210 - ANTISERA, OTHER BLOOD FRACTIONS AND IMMUNOLOGICAL PRODUCTS, WHETHER OR NOT MODIFIED OR OBTAINED BY MEANS OF BIOTECHNOLOGICAL PROCESSES CN CODE 30021098 - OTHER THAN ANTISERA, HAEMOGLOBIN, BLOOD GLOBULINS AND SERUM GLOBULINS. ALSO CLASSIFIED IN ACCORDANCE WITH: HSENS TO HEADING 3002 (2) (B)