摘要:
The invention concerns the use of a compound of general formula (I) wherein: R0 represents in particular a hydrogen atom or an alkyl group; R1 represents in particular a hydrogen atom or an alkyl group; R2, R3 and R4 represent in particular, independently of one another, a hydrogen atom, a hydroxyl group, an alkoxy group or an acyloxy group, for preparing a medicine for treating lysosomal diseases related to a dysfunction of at least one lysosomal glycosidase enzyme.
摘要翻译:本发明涉及通式(I)的化合物的用途,其中:R 0表示特别是氢原子或烷基; R 1特别表示氢原子或烷基; R 2,R 3和R 4特别是彼此独立地表示氢原子,羟基,烷氧基或 用于制备用于治疗与至少一种溶酶体糖苷酶的功能障碍相关的溶酶体疾病的药物的酰氧基。
摘要:
An ionic polymer composite device and methods for fabricating the ionic polymer composite device are provided. The ionic polymer composite device includes two extended electrode layers, each extended electrode layer including at least one ionic polymer with a plurality of electrically conductive particles, and a dielectric layer including at least one sulfonated poly ether sulfone polymer or a derivative between the two extended electrode layers.
摘要:
Method for enhancing in a mammalian cell the activity of an enzyme associated with Gaucher Disease by administering a competitive inhibitor of glucocerebrosidase in an amount effective to enhance the activity of the enzyme. Preferred compounds for use in the method are imino sugars and related compounds. In particular, C8-12-alkyl derivatives of N-alkyl-deoxynojirimycin, isofagomine compounds, and calystegine compoiunds are effective to enhance glucocerebrosidase activity.
摘要:
Method for enhancing in a mammalian cell the activity of an enzyme associated with Gaucher Disease by administering a competitive inhibitor of glucocerebrosidase in an amount effective to enhance the activity of the enzyme. Preferred compounds for use in the method are imino sugars and related compounds. In particular, C8-12-alkyl derivatives of N-alkyl-deoxynojirimycin, isofagomine compounds, and calystegine compoiunds are effective to enhance glucocerebrosidase activity.
摘要:
A polymer electrolyte for a fuel cell is provided at low cost which has excellent mechanical characteristics, resistance to oxidation and high ion conductivity, and which hardly swells.The polymer electrolyte includes a block copolymer containing a hydrophilic segment and a hydrophobic segment. The hydrophilic segment contains a structural unit represented by the following chemical formula (1).
摘要:
Method for enhancing in a mammalian cell the activity of an enzyme associated with Gaucher Disease by administering a competitive inhibitor of glucocerebrosidase in an amount effective to enhance the activity of the enzyme. Preferred compounds for use in the method are imino sugars and related compounds. In particular, C8-12-alkyl derivatives of N-alkyl-deoxynojirimycin, isofagomine compounds, and calystegine compoiunds are effective to enhance glucocerebrosidase activity.
摘要:
A device for attaching and detaching a mounting board unit with respect to a device body in a substantially vertical posture includes a controller base which has a detachable mounting board fixed thereto and a guide portion engaging a guide rail provided on the device body. The guide portion guides the controller base when the mounting board unit is attached to and detached from the device body. Mounting boards having different shapes can be mounted on the unit of the same construction, and even a mounting board on which a heavy device such as a hard disk is mounted can be easily attached to and detached from the device body in a vertically arranged condition.
摘要:
An image forming apparatus having a pressing unit for pressing a fixing unit so that the fixing unit is secured to the apparatus body by the pressing force of the pressing unit. A projection portion on the fixing unit and an insertion slit in the apparatus body also secure the fixing unit, and thus, screws for securing the fixing unit to the apparatus body can be omitted and the fixing unit can easily be attached/detached. A discharge cover is rotatably mounted to the apparatus body and the fixing unit is inserted through an opening portion created when the discharge cover is opened.
摘要:
A fixing apparatus having a heating member maintained at a predetermined fixing temperature and an electric supply device for supplying an electric current to the heating member, wherein the electric supply device supplies an electric current at a second duty cycle larger than a first duty cycle after an electric current is supplied at the first duty cycle from the time the supply of the electric current is started to the heating member until a fixing temperature is reached.
摘要:
Method for enhancing in a mammalian cell the activity of an enzyme associated with Gaucher Disease by administering a competitive inhibitor of glucocerebrosidase in an amount effective to enhance the activity of the enzyme. Preferred compounds for use in the method are imino sugars and related compounds. In particular, C8-12-alkyl derivatives of N-alkyl-deoxynojirimycin, isofagomine compounds, and calystegine compounds are effective to enhance glucocerebrosidase activity.