| Autor: |
Bazin, Dominique, Rabant, Marion, Mathurin, Jérémie, Petay, Margaux, Deniset-Besseau, Ariane, Dazzi, Alexandre, Su, Yangyang, Hessou, Etienne P., Tielens, Frederik, Borondics, Ferenc, Livrozet, Marine, Bouderlique, Elise, Haymann, Jean-Philippe, Letavernier, Emmanuel, Frochot, Vincent, Daudon, Michel |
| Jazyk: |
English<br />French |
| Rok vydání: |
2021 |
| Předmět: |
|
| Zdroj: |
Comptes Rendus. Chimie, Vol 25, Iss S1, Pp 489-502 (2021) |
| Druh dokumentu: |
article |
| ISSN: |
1878-1543 |
| DOI: |
10.5802/crchim.135 |
| Popis: |
Medical literature indicates clearly that cystinuria and cystinosis, two severe genetic pathologies, are related to the presence of abnormal L-cystine deposits. While L-cystine adopts a hexagonal crystal morphology consistent with its crystallographic structure (hexagonal, $\mathrm{P}6_{1}22$ space group), abnormal deposits related to cystinosis display a rectangular shape. Because this is unexpected from the hexagonal crystallographic structure of L-cystine, we have investigated this inconsistency using SEM (scanning electron microscopy) and IR (infrared) spectroscopy at micrometre and nanometre scales. Our data clearly indicate the presence of both L-cysteine and L-cystine. Considering that L-cysteine crystals display a rectangular shape, and that a transition phase between L-cysteine and L-cystine is well known, we propose the following model for deposit evolution in cystinosis. The initial abnormal deposit consists of L-cysteine, with a rectangular crystal morphology. The micrometre scale rectangular crystallite shape is retained after the phase transition equilibrium between L-cysteine and L-cystine is established, with some crystalline L-cysteine still remaining. |
| Databáze: |
Directory of Open Access Journals |
| Externí odkaz: |
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