| Literature DB >> 35296914 |
Yangyang Su1,2, Etienne P Hessou1, Estefania Colombo3, Gustavo Belletti3, Ali Moussadik1, Ivan T Lucas4, Vincent Frochot5,6, Michel Daudon5,6, Stéphan Rouzière7, Dominique Bazin8, Kezhi Li2, Paola Quaino3, Frederik Tielens9.
Abstract
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.Entities:
Keywords: Biominerals; Characterization; Computer simulations; Crystalline structure; L-cysteine; L-cystine
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Year: 2022 PMID: 35296914 DOI: 10.1007/s00726-022-03144-6
Source DB: PubMed Journal: Amino Acids ISSN: 0939-4451 Impact factor: 3.789