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| 溶酶体内胱氨酸可促进细胞凋亡并导致胱氨酸贮积症的表型发生 | |
| 引用本文： | Jess G.THOENE. 溶酶体内胱氨酸可促进细胞凋亡并导致胱氨酸贮积症的表型发生[J]. 北京大学学报(医学版), 2005, 37(1): 8-9 |
| 作者姓名： | Jess G.THOENE |
| 作者单位： | Hayward Genetics |
| 摘 要： | Cystinosishasbeenknownasaclinicalentityforapproximately 10 0 years.Thenamederivesfromthehallmarkaccumulationofcystinewithinlysosomes .Cystineisthedisulfideaminoacidwhichiscapableofformingdisulfidebridgesbetweenadjacent peptidechains.Itisthelargestandleast… |
| 关 键 词： | 胱氨酸病 胱氨酸 溶酶体 |
Lysosomal cystine augments apoptosis and causes the phenotype in cystinosis |
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| Jess G.THOENE. Lysosomal cystine augments apoptosis and causes the phenotype in cystinosis[J]. Journal of Peking University. Health sciences, 2005, 37(1): 8-9 | |
| Authors: | Jess G.THOENE |
| Affiliation: | Hayward Genetics Center,Tulane University School of Medicine,New Orleans, LA 70112, USA |
| Abstract: | SUMMARY Nephropathic cystinosis is a lethal inborn error of metabolism that destroys kidney function by age 10 years. It is characterized by lysosomal cystine accumulation. How the cystine causes the phenotype is an open question. We propose that during apoptosis, permeablized lysosomes permit cystine to reach the cytosol where mixed disulfide formation occurs, augmenting apoptosis by interaction with a variety of pro-apoptotic proteins. |
| Keywords: | Cystinosis Cystine Lysosomes |
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