期刊
AMINO ACIDS
卷 54, 期 8, 页码 1123-1133出版社
SPRINGER WIEN
DOI: 10.1007/s00726-022-03144-6
关键词
Biominerals; Computer simulations; Crystalline structure; Characterization; l-cysteine; l-cystine
资金
- Vrije Universiteit Brussel
- Flemish Supercomputer Center (VSC)
- FWO
- VUB
This paper investigates the characteristics of abnormal deposits related to genetic diseases cystinuria and cystinosis. The study compares the differences between l-cystine and l-cysteine using theoretical and experimental methods, providing a better characterization of these two genetic pathologies.
It is assumed that genetic diseases affecting the metabolism of cysteine and the kidney function lead to two different kinds of pathologies, namely cystinuria and cystinosis whereby generate l-cystine crystals. Recently, the presence of l-cysteine crystal has been underlined in the case of cystinosis. Interestingly, it can be strikingly seen that cystine ([-S-CH2-CH-(NH2)-COOH](2)) consists of two cysteine (C3H7NO2S) molecules connected by a disulfide (S-S) bond. Therefore, the study of cystine and cysteine is important for providing a better understanding of cystinuria and cystinosis. In this paper, we elucidate the discrepancy between l-cystine and l-cysteine by investigating the theoretical and experimental infrared spectra (IR), X-ray diffraction (XRD) as well as Raman spectra aiming to obtain a better characterization of abnormal deposits related to these two genetic pathologies.
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