دورية أكاديمية
Metabolic Alteration Analysis of Steroid Hormones in Niemann–Pick Disease Type C Model Cell Using Liquid Chromatography/Tandem Mass Spectrometry
العنوان: | Metabolic Alteration Analysis of Steroid Hormones in Niemann–Pick Disease Type C Model Cell Using Liquid Chromatography/Tandem Mass Spectrometry |
---|---|
المؤلفون: | Ai Abe, Masamitsu Maekawa, Toshihiro Sato, Yu Sato, Masaki Kumondai, Hayato Takahashi, Masafumi Kikuchi, Katsumi Higaki, Jiro Ogura, Nariyasu Mano |
المصدر: | International Journal of Molecular Sciences, Vol 23, Iss 8, p 4459 (2022) |
بيانات النشر: | MDPI AG, 2022. |
سنة النشر: | 2022 |
المجموعة: | LCC:Biology (General) LCC:Chemistry |
مصطلحات موضوعية: | Niemann–Pick disease type C, steroid hormones, LC–MS/MS, mitochondria, ammonium solution, metabolic change, Biology (General), QH301-705.5, Chemistry, QD1-999 |
الوصف: | Niemann–Pick disease type C (NPC) is an autosomal recessive disease caused by a functional deficiency of cholesterol-transporting proteins in lysosomes, and exhibits various clinical symptoms. Since mitochondrial dysfunction in NPC has recently been reported, cholesterol catabolism to steroid hormones may consequently be impaired. In this study, we developed a comprehensive steroid hormone analysis method using liquid chromatography/tandem mass spectrometry (LC–MS/MS) and applied it to analyze changes in steroid hormone concentrations in NPC model cells. We investigated the analytical conditions for simultaneous LC–MS/MS analysis, which could be readily separated from each other and showed good reproducibility. The NPC phenotype was verified as an NPC model with mitochondrial abnormalities using filipin staining and organelle morphology observations. Steroid hormones in the cell suspension and cell culture medium were also analyzed. Steroid hormone analysis indicated that the levels of six steroid hormones were significantly decreased in the NPC model cell and culture medium compared to those in the wild-type cell and culture medium. These results indicate that some steroid hormones change during NPC pathophysiology and this change is accompanied by mitochondrial abnormalities. |
نوع الوثيقة: | article |
وصف الملف: | electronic resource |
اللغة: | English |
تدمد: | 1422-0067 1661-6596 |
العلاقة: | https://www.mdpi.com/1422-0067/23/8/4459Test; https://doaj.org/toc/1661-6596Test; https://doaj.org/toc/1422-0067Test |
DOI: | 10.3390/ijms23084459 |
الوصول الحر: | https://doaj.org/article/f94298c1624e4abbaef0a10c99cb66cbTest |
رقم الانضمام: | edsdoj.f94298c1624e4abbaef0a10c99cb66cb |
قاعدة البيانات: | Directory of Open Access Journals |
تدمد: | 14220067 16616596 |
---|---|
DOI: | 10.3390/ijms23084459 |