Lysosomal cystine augments apoptosis and causes the phenotype in cystinosis
- VernacularTitle: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
- Author:
Jess G.THOENE
- Publication Type:Journal Article
- Keywords:
Cystinosis;
Cystine;
Lysosomes
- From:
Journal of Peking University(Health Sciences)
2005;37(1):8-9
- CountryChina
- Language:Chinese
-
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.
