Academic Journal
Cystinuria and cystinosis are usually related to L-cystine: is this really the case for cystinosis? A physicochemical investigation at micrometre and nanometre scale
| العنوان: | Cystinuria and cystinosis are usually related to L-cystine: is this really the case for cystinosis? A physicochemical investigation at micrometre and nanometre scale |
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| المؤلفون: | 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 |
| المصدر: | Comptes Rendus. Chimie, Vol 25, Iss S1, Pp 489-502 (2021) |
| بيانات النشر: | Académie des sciences, 2021. |
| سنة النشر: | 2021 |
| المجموعة: | LCC:Biochemistry LCC:Physical and theoretical chemistry LCC:Mathematics |
| مصطلحات موضوعية: | Cystinuria, Cystinosis, FTIR, SEM, AFM-IR, OPTIR, Biochemistry, QD415-436, Physical and theoretical chemistry, QD450-801, Mathematics, QA1-939 |
| الوصف: | 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. |
| نوع الوثيقة: | article |
| وصف الملف: | electronic resource |
| اللغة: | English French |
| تدمد: | 1878-1543 |
| Relation: | https://comptes-rendus.academie-sciences.fr/chimie/articles/10.5802/crchim.135/; https://doaj.org/toc/1878-1543 |
| DOI: | 10.5802/crchim.135 |
| URL الوصول: | https://doaj.org/article/4b3277857219474299aea4c192710322 |
| رقم الانضمام: | edsdoj.4b3277857219474299aea4c192710322 |
| قاعدة البيانات: | Directory of Open Access Journals |
| تدمد: | 18781543 |
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| DOI: | 10.5802/crchim.135 |