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학술연구/단체지원/교육 등 연구자 활동을 지속하도록 DBpia가 지원하고 있어요.
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연구자들이 자신의 연구와 전문성을 널리 알리고, 새로운 협력의 기회를 만들 수 있는 네트워킹 공간이에요.
논문 기본 정보
- 자료유형
- 학술저널
- 저자정보
- 발행연도
- 2006.1
- 수록면
- 652 - 661 (10page)
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초록· 키워드
오류제보하기
Homocystinuria is a metabolic disorder caused by a deficiency of cystathionine β-synthase (CBS). The major clinical symptoms of this disease are mental retardation, lens dislocation, vascular disease with life-threatening thromboembolisms, and skeletal deformities. The major treatments for CBS deficiency include pharmacologic doses of pyridoxine or dietary restriction of methionine. There is currently plasma levels of homocysteine. However, gene therapy could be an effective novel approach for the treatment of homocystinuria. A recombinant adeno- associated virus vector carrying human CBS cDNA (rAAV-hCBS) was constructed and administered to CBS-/- mice by intramuscular (IM) and intraperitoneal (IP) injections. Serum homocysteine concentrations significantly decreased in treated mice compared with age-matched controls two weeks after treat-ment. The treated CBS-/- mice had life spans 3-7 days longer compared with untreated CBS-/- mice. In CBS-/- mice treated with rAAV-hCBS via IP injection, the vector was detected in all organs examined including liver, spleen, and kidney, and CBS gene expression the liver. These results indicate the efficacy of gene delivery and demonstrate the possibility of gene therapy mediated by AAV gene transfer in this mouse model of homocystinuria.
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등록된 정보가 없습니다.
참고문헌 (29)
참고문헌 신청
/ / Heterozygosity for homo-cystinuria in premature peripheral and cerebral occlusive arte-rial disease : 709 ~ 15
/ / Phase I trial of intranasal and endobronchial administration of a recombinant adeno-associated virus serotype 2 -CFTR vector in adult cystic fibrosis patients guggi (wb)
/ / The natural history of homocystinuria due to cystathionine -synthase deficiency
/ 1995 / Mice deficient in cystathio
/ 1996 / Hyperhomocysteinemia-an emerging and important risk factor for thromboembolic and cardiovascular disease 106 : 709 ~ 722
/ / Phase I trial of intranasal and endobronchial administration of a recombinant adeno-associated virus serotype 2 -CFTR vector in adult cystic fibrosis patients guggi (wb)
/ / The natural history of homocystinuria due to cystathionine -synthase deficiency
/ 1995 / Mice deficient in cystathio
/ 1996 / Hyperhomocysteinemia-an emerging and important risk factor for thromboembolic and cardiovascular disease 106 : 709 ~ 722
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