Psychiatric burden in a cohort of adults with Niemann Pick type C disease: from psychotic symptoms to frontal lobe behavioral disorders
To describe Niemann-Pick type C (NP-C) behavioral symptoms (focusing on psychotic symptoms) and its relation to frontal lobe functioning. (Source: Orphanet Journal of Rare Diseases)
Source: Orphanet Journal of Rare Diseases - September 22, 2023 Category: Internal Medicine Authors: A. Morin, G. Carle, A. Ponchel, G. Fern ández-Eulate and Y. Nadjar Tags: Research Source Type: research
Secretory Production of 7-dehydrocholesterol by Engineered Saccharomyces cerevisiae
CONCLUSION: Selective secretion of different sterol intermediates was characterized in sterol over-produced strain and the extracellular export of 7-DHC developed in present study significantly improved the cell biosynthetic capacity, which offered a novel modification idea for 7-DHC de novo biosynthesis by S. cerevisiae cell factory. This article is protected by copyright. All rights reserved.PMID:37688450 | DOI:10.1002/biot.202300056 (Source: Biotechnology Journal)
Source: Biotechnology Journal - September 9, 2023 Category: Biotechnology Authors: Xia Ke Zi-Hao Pan Hong-Fei Du Yi Shen Ji-Dong Shen Zhi-Qiang Liu Yu-Guo Zheng Source Type: research
Secretory Production of 7-dehydrocholesterol by Engineered Saccharomyces cerevisiae
CONCLUSION: Selective secretion of different sterol intermediates was characterized in sterol over-produced strain and the extracellular export of 7-DHC developed in present study significantly improved the cell biosynthetic capacity, which offered a novel modification idea for 7-DHC de novo biosynthesis by S. cerevisiae cell factory. This article is protected by copyright. All rights reserved.PMID:37688450 | DOI:10.1002/biot.202300056 (Source: Biotechnology Journal)
Source: Biotechnology Journal - September 9, 2023 Category: Biotechnology Authors: Xia Ke Zi-Hao Pan Hong-Fei Du Yi Shen Ji-Dong Shen Zhi-Qiang Liu Yu-Guo Zheng Source Type: research
Secretory Production of 7-dehydrocholesterol by Engineered Saccharomyces cerevisiae
CONCLUSION: Selective secretion of different sterol intermediates was characterized in sterol over-produced strain and the extracellular export of 7-DHC developed in present study significantly improved the cell biosynthetic capacity, which offered a novel modification idea for 7-DHC de novo biosynthesis by S. cerevisiae cell factory. This article is protected by copyright. All rights reserved.PMID:37688450 | DOI:10.1002/biot.202300056 (Source: Biotechnology Journal)
Source: Biotechnology Journal - September 9, 2023 Category: Biotechnology Authors: Xia Ke Zi-Hao Pan Hong-Fei Du Yi Shen Ji-Dong Shen Zhi-Qiang Liu Yu-Guo Zheng Source Type: research
Secretory Production of 7-dehydrocholesterol by Engineered Saccharomyces cerevisiae
CONCLUSION: Selective secretion of different sterol intermediates was characterized in sterol over-produced strain and the extracellular export of 7-DHC developed in present study significantly improved the cell biosynthetic capacity, which offered a novel modification idea for 7-DHC de novo biosynthesis by S. cerevisiae cell factory. This article is protected by copyright. All rights reserved.PMID:37688450 | DOI:10.1002/biot.202300056 (Source: Biotechnology Journal)
Source: Biotechnology Journal - September 9, 2023 Category: Biotechnology Authors: Xia Ke Zi-Hao Pan Hong-Fei Du Yi Shen Ji-Dong Shen Zhi-Qiang Liu Yu-Guo Zheng Source Type: research
Chemical Synthesis and Biochemical Properties of Cholestane-5 α,6β-diol-3-sulfonate, A Non-hydrolysable Analogue of cholestane-5α,6β-diol-3β-sulfate
J Steroid Biochem Mol Biol. 2023 Sep 6:106396. doi: 10.1016/j.jsbmb.2023.106396. Online ahead of print.ABSTRACTCholestane-3β,5α,6β-triol (CT) is a primary metabolite of 5,6-epoxycholesterols (5,6-EC) that is catalyzed by the cholesterol-5,6-epoxide hydrolase (ChEH). CT is a well-known biomarker for Niemann-Pick disease type C (NP-C), a progressive inherited neurodegenerative disease. On the other hand, CT is known to be metabolized by the 11β-hydroxysteroid-dehydrogenase of type 2 (11β-HSD2) into a tumor promoter named oncosterone that stimulates the growth of breast cancer tumors. Sulfation is a major metabolic trans...
Source: Mol Biol Cell - September 8, 2023 Category: Molecular Biology Authors: Philippe de M édina Silia Ayadi R égis Soulès Bruno Payre Sandrine Rup-Jacques Sandrine Silvente-Poirot Mohammad Samadi Marc Poirot Source Type: research
Chemical Synthesis and Biochemical Properties of Cholestane-5 α,6β-diol-3-sulfonate, A Non-hydrolysable Analogue of cholestane-5α,6β-diol-3β-sulfate
J Steroid Biochem Mol Biol. 2023 Sep 6:106396. doi: 10.1016/j.jsbmb.2023.106396. Online ahead of print.ABSTRACTCholestane-3β,5α,6β-triol (CT) is a primary metabolite of 5,6-epoxycholesterols (5,6-EC) that is catalyzed by the cholesterol-5,6-epoxide hydrolase (ChEH). CT is a well-known biomarker for Niemann-Pick disease type C (NP-C), a progressive inherited neurodegenerative disease. On the other hand, CT is known to be metabolized by the 11β-hydroxysteroid-dehydrogenase of type 2 (11β-HSD2) into a tumor promoter named oncosterone that stimulates the growth of breast cancer tumors. Sulfation is a major metabolic trans...
Source: Mol Biol Cell - September 8, 2023 Category: Molecular Biology Authors: Philippe de M édina Silia Ayadi R égis Soulès Bruno Payre Sandrine Rup-Jacques Sandrine Silvente-Poirot Mohammad Samadi Marc Poirot Source Type: research
Bioinformatics analysis of carotid vulnerable plaques associated with the SARS-CoV-2 Pattern
Gene. 2023 Aug 31:147754. doi: 10.1016/j.gene.2023.147754. Online ahead of print.ABSTRACTThe rupture of carotid artery vulnerable plaque plays a critical role in ischemic stroke, and the widely spread new coronavirus in recent years plays a certain role in the development of human carotid artery vulnerable plaque, we screened out 27 differential expression genes (DEGs) of stable plaque and vulnerable plaque associated with the new coronavirus. Through the construction of the protein-protein interaction (PPI) network, the Cathepsin B (CTSB) and Niemann-Pick Disease Type 2 (NPC2) were identified as crucial expression genes, ...
Source: Gene - September 2, 2023 Category: Genetics & Stem Cells Authors: Tao Jiang Jiaming Huang Shupeng Li Qiushi Xu Tianding Zhang Xianwei Wang Dong Chen Source Type: research
Bioinformatics analysis of carotid vulnerable plaques associated with the SARS-CoV-2 Pattern
Gene. 2023 Aug 31:147754. doi: 10.1016/j.gene.2023.147754. Online ahead of print.ABSTRACTThe rupture of carotid artery vulnerable plaque plays a critical role in ischemic stroke, and the widely spread new coronavirus in recent years plays a certain role in the development of human carotid artery vulnerable plaque, we screened out 27 differential expression genes (DEGs) of stable plaque and vulnerable plaque associated with the new coronavirus. Through the construction of the protein-protein interaction (PPI) network, the Cathepsin B (CTSB) and Niemann-Pick Disease Type 2 (NPC2) were identified as crucial expression genes, ...
Source: Gene - September 2, 2023 Category: Genetics & Stem Cells Authors: Tao Jiang Jiaming Huang Shupeng Li Qiushi Xu Tianding Zhang Xianwei Wang Dong Chen Source Type: research
Midline brain structures in adult niemann-pick type c disease: a cross-sectional study
CONCLUSIONS: Our findings suggest that adult NPC patients show some markers of early neurodevelopmental disturbance, matching findings seen in psychotic disorders. The differences in AI, but not CSP, suggest neurodevelopmental change may occur early in gestation rather than post-partum. The relationship with duration of illness suggests that there may be atrophy over time in these structures, consistent with prior analyses of grey matter regions in NPC.PMID:37612148 | DOI:10.1017/neu.2023.43 (Source: Acta Neuropsychiatrica)
Source: Acta Neuropsychiatrica - August 23, 2023 Category: Psychiatry Authors: Mark Walterfang Tsutomu Takahashi Maria A Di Biase Vanessa L Cropley Daiki Sasabayashi Michio Suzuki Dennis Velakoulis Christos Pantelis Source Type: research
Post-feeding transcriptomics reveals essential genes expressed in the midgut of the desert locust
In conclusion, the transcriptome datasets presented here will provide a useful and promising resource for studying the midgut physiology of S. gregaria, a socio-economically important pest species. (Source: Frontiers in Physiology)
Source: Frontiers in Physiology - August 10, 2023 Category: Physiology Source Type: research
Established and Emerging Lipid-Lowering Drugs for Primary and Secondary Cardiovascular Prevention
This article reviews established and emerging lipid-lowering drugs regarding their mechanism of action, development stage, ongoing clinical trials, side effects, effect on blood lipids and reduction in cardiovascular morbidity and mortality. We conducted a keyword search to identify studies on established and emerging lipid modifying drugs. Results were summarized in a narrative overview. Established pharmaceutical treatment options include the Niemann-Pick-C1 like-1 protein (NPC1L1) inhibitor ezetimibe, the protein convertase subtilisin-kexin type 9 (PCSK9) inhibitors alirocumab and evolocumab, fibrates as peroxisome prol...
Source: Cancer Control - July 24, 2023 Category: Cancer & Oncology Authors: Daniel Tobias Michaeli Julia Caroline Michaeli Sebastian Albers Tobias Boch Thomas Michaeli Source Type: research
Established and Emerging Lipid-Lowering Drugs for Primary and Secondary Cardiovascular Prevention
This article reviews established and emerging lipid-lowering drugs regarding their mechanism of action, development stage, ongoing clinical trials, side effects, effect on blood lipids and reduction in cardiovascular morbidity and mortality. We conducted a keyword search to identify studies on established and emerging lipid modifying drugs. Results were summarized in a narrative overview. Established pharmaceutical treatment options include the Niemann-Pick-C1 like-1 protein (NPC1L1) inhibitor ezetimibe, the protein convertase subtilisin-kexin type 9 (PCSK9) inhibitors alirocumab and evolocumab, fibrates as peroxisome prol...
Source: American Journal of Cardiovascular Drugs - July 24, 2023 Category: Cardiology Source Type: research
Novel application potential of cinaciguat in the treatment of mixed hyperlipidemia through targeting PTL/NPC1L1 and alleviating intestinal microbiota dysbiosis and metabolic disorders
We presented in vitro evidence from surface plasmon resonance analysis that cinaciguat interacted with PTL and NPC1L1. Furthermore, cinaciguat exhibited potent PTL-inhibition activity. Fluorescence-labeled cholesterol uptake analysis and confocal imaging showed that cinaciguat effectively inhibited cholesterol uptake. In vivo evaluation showed that cinaciguat significantly reduced the plasma levels of triglycerides and cholesterol, and effectively alleviated high-fat diet-induced intestinal microbiota dysbiosis and metabolic disorders. These results collectively suggest that cinaciguat has the potential to be further devel...
Source: Atherosclerosis - July 17, 2023 Category: Cardiology Authors: Ang Jia Hongfei Jiang Wenjing Liu Pengwei Chen Qi Xu Renshuai Zhang Jufeng Sun Source Type: research
Novel application potential of cinaciguat in the treatment of mixed hyperlipidemia through targeting PTL/NPC1L1 and alleviating intestinal microbiota dysbiosis and metabolic disorders
We presented in vitro evidence from surface plasmon resonance analysis that cinaciguat interacted with PTL and NPC1L1. Furthermore, cinaciguat exhibited potent PTL-inhibition activity. Fluorescence-labeled cholesterol uptake analysis and confocal imaging showed that cinaciguat effectively inhibited cholesterol uptake. In vivo evaluation showed that cinaciguat significantly reduced the plasma levels of triglycerides and cholesterol, and effectively alleviated high-fat diet-induced intestinal microbiota dysbiosis and metabolic disorders. These results collectively suggest that cinaciguat has the potential to be further devel...
Source: Atherosclerosis - July 17, 2023 Category: Cardiology Authors: Ang Jia Hongfei Jiang Wenjing Liu Pengwei Chen Qi Xu Renshuai Zhang Jufeng Sun Source Type: research
