Tenth International Congress on Ceroid Lipofuscinoses

描述（由申请人提供）：为将于2005年6月8日至12日在芬兰赫尔辛基Hanasaari会议中心举行的第十届蜡样脂褐质病（Battens病）国际大会寻求会议支持。我们预计将有来自世界各地的130名参与者，并要求为美国的一些参与者提供支持。目前正在向欧盟寻求配套资金。会议中心可容纳92人，其余与会者可在附近的酒店住宿。它是一个理想的会议场所，因为与会者聚集在一起，但另一方面，它很容易从赫尔辛基到达。会议中心设备齐全，可供残疾人使用。这对于科学家的参与和父母及其坐轮椅的儿童出席和参加会议的能力都是至关重要的。儿童神经退行性疾病的Battens组涉及至少10种不同的基因，并且共同是儿童最常见的遗传性神经退行性疾病。他们的特点是视力丧失、癫痫发作和智力迟钝。所有这些都被认为涉及受损的溶酶体蛋白质降解，尽管只有两个涉及的基因，棕榈酰;蛋白硫酯酶和三肽基肽酶，已被确定为酶。CLN 3、5、6和8编码的蛋白质功能未知，本次会议的重点之一将是利用蛋白质组学，结合生物化学和细胞生物学，试图确定这种功能。其他潜在的令人兴奋的领域将包括脐带血干细胞的使用，基因治疗，营养治疗，药物治疗和使用转基因动物模型来开发有效的治疗方法。前九届大会汇集了医生、基础研究人员和家长，并为年轻研究人员，特别是女性研究人员提供了一个论坛，口头介绍他们的工作并参加热烈的讨论。赫尔辛基是第六届大会的所在地，其做法是在欧洲和美国之间交替举行会议，从而建立了国际合作研究，并促进了对这种毁灭性疾病的更好理解。

DESCRIPTION (provided by applicant): Conference support is sought for the Tenth International Congress on Ceroid Lipofuscinoses (Battens disease) to be held at the Hanasaari Congress Center, Helsinki, Finland from June 8th to 12th, 2005. We anticipate 130 participants from all over the world and support is requested for some of the US participants. Matching funds are being solicited from the EU. The Congress center has capacity to accommodate 92 persons and the rest of the participants can be accommodated at nearby hotels. It makes an ideal congress venue due to the fact that the participants are gathered together but on the other hand it is readily accessible from Helsinki. The Conference center is fully equipped for handicapped access. This is essential both for the participation of scientists and for the ability of parents and their wheelchair bound children to attend and participate in the meeting. The Battens group of childhood neurodegenerative disorders involve at least 10 different genes and collectively are the most common inherited neurodegenerative diseases of children. They are characterized by vision loss, seizures and mental retardation. All are believed to involve impaired lysosomal protein degradation although only two of the genes involved, palmitoyl;protein thioesterase and tripeptidylpeptidase, have been identified as enzymes. The proteins coded by CLN3, 5,6 and 8 are of unknown function and one focus of this meeting will be to use proteomics, in combination with biochemistry and cell biology, to try to determine this function. Other potentially exciting areas to be covered will include the use of cord blood-derived stem cells, gene therapy, nutritional therapy, drug therapy and the use of transgenic animal models to develop effective treatments. The previous 9 Congresses have brought together physicians, basic researchers and parents and have provided a forum for young researchers, especially females, to present their work orally and participate in lively discussions. Helsinki was the site of the 6th Congress and the practice has been to alternate the Conference between Europe and the USA, resulting in the establishment of international collaborative research and facilitating better understanding of this devastating disease.