日期:
2020 年 — 2026 年
2020
2021
2022
2023
2024
2025
2026
影响因子:

Myelin Disruption, Neuroinflammation, and Oxidative Stress Induced by Sulfite in the Striatum of Rats Are Mitigated by the pan-PPAR agonist Bezafibrate

亚硫酸盐诱导的大鼠纹状体髓鞘破坏、神经炎症和氧化应激可被泛PPAR激动剂苯扎贝特减轻

Glänzel, Nícolas Manzke; Parmeggiani, Belisa; Grings, Mateus; Seminotti, Bianca; Brondani, Morgana; Bobermin, Larissa D; Ribeiro, César A J; Quincozes-Santos, André; Vockley, Jerry; Leipnitz, Guilhian

ITCH deficiency clinical phenotype expansion and mitochondrial dysfunction

ITCH 缺乏症临床表型扩增和线粒体功能障碍

Rachel Wolfe, Paige Heiman, Olivia D'Annibale, Anuradha Karunanidhi, Alyssa Powers, Marianne Mcguire, Bianca Seminotti, Steven F Dobrowolski, Miguel Reyes-Múgica, Kathryn S Torok, Al-Walid Mohsen, Jerry Vockley, Lina Ghaloul-Gonzalez

Nuclear Factor Erythroid-2-Related Factor 2 Signaling in the Neuropathophysiology of Inherited Metabolic Disorders

核因子E2相关因子2信号通路在遗传性代谢疾病神经病理生理学中的作用

Seminotti, Bianca; Grings, Mateus; Tucci, Paolo; Leipnitz, Guilhian; Saso, Luciano

Hypoglycemia symptoms and awareness of hypoglycemia in type 1 diabetes mellitus: cross-cultural adaptation and validation of the Portuguese version of three questionnaires and evaluation of its risk factors

1型糖尿病患者的低血糖症状和低血糖意识:三种问卷葡萄牙语版本的跨文化适应和验证及其风险因素评估

Stefenon, Paula; Silveira, André Luís Marques da; Giaretta, Luana Seminotti; Leitão, Cristiane Bauermann; Bauer, Andrea Carla

Reticular Dysgenesis and Mitochondriopathy Induced by Adenylate Kinase 2 Deficiency with Atypical Presentation

腺苷酸激酶 2 缺乏症引起的非典型表现的网状发育不全和线粒体病

Lina Ghaloul-Gonzalez, Al-Walid Mohsen, Anuradha Karunanidhi, Bianca Seminotti, Hey Chong, Suneeta Madan-Khetarpal, Jessica Sebastian, Catherine Walsh Vockley, Miguel Reyes-Múgica, Mark T Vander Lugt, Jerry Vockley

ETHE1 and MOCS1 deficiencies: Disruption of mitochondrial bioenergetics, dynamics, redox homeostasis and endoplasmic reticulum-mitochondria crosstalk in patient fibroblasts

ETHE1 和 MOCS1 缺陷:患者成纤维细胞中的线粒体生物能量学、动力学、氧化还原稳态和内质网-线粒体串扰

Mateus Grings, Bianca Seminotti, Anuradha Karunanidhi, Lina Ghaloul-Gonzalez, Al-Walid Mohsen, Peter Wipf, Johan Palmfeldt, Jerry Vockley, Guilhian Leipnitz

Mitochondrial energetics is impaired in very long-chain acyl-CoA dehydrogenase deficiency and can be rescued by treatment with mitochondria-targeted electron scavengers

极长链酰基辅酶A脱氢酶缺乏症会损害线粒体能量代谢,而使用线粒体靶向电子清除剂治疗可以挽救这种损害。

Seminotti, Bianca; Leipnitz, Guilhian; Karunanidhi, Anuradha; Kochersperger, Catherine; Roginskaya, Vera Y; Basu, Shrabani; Wang, Yudong; Wipf, Peter; Van Houten, Bennett; Mohsen, Al-Walid; Vockley, Jerry

Evaluation of mitochondrial bioenergetics, dynamics, endoplasmic reticulum-mitochondria crosstalk, and reactive oxygen species in fibroblasts from patients with complex I deficiency

评估复合物 I 缺乏症患者成纤维细胞中的线粒体生物能量学、动力学、内质网-线粒体串扰和活性氧

Guilhian Leipnitz, Al-Walid Mohsen, Anuradha Karunanidhi, Bianca Seminotti, Vera Y Roginskaya, Desiree M Markantone, Mateus Grings, Stephanie J Mihalik, Peter Wipf, Bennett Van Houten, Jerry Vockley

Increased glutamate receptor and transporter expression in the cerebral cortex and striatum of gcdh-/- mice: possible implications for the neuropathology of glutaric acidemia type I

gcdh-/-小鼠大脑皮层和纹状体中谷氨酸受体和转运蛋白表达增加:可能与I型戊二酸血症的神经病理学有关

Lagranha, Valeska Lizzi; Matte, Ursula; de Carvalho, Talita Giacomet; Seminotti, Bianca; Pereira, Carolina Coffi; Koeller, David M; Woontner, Michael; Goodman, Stephen I; de Souza, Diogo Onofre Gomes; Wajner, Moacir

Experimental evidence that methylmalonic acid provokes oxidative damage and compromises antioxidant defenses in nerve terminal and striatum of young rats

实验证据表明,甲基丙二酸会引发氧化损伤,并损害幼鼠神经末梢和纹状体的抗氧化防御能力。

Fernandes, Carolina Gonçalves; Borges, Clarissa Günther; Seminotti, Bianca; Amaral, Alexandre Umpierrez; Knebel, Lisiane Aurélio; Eichler, Paula; de Oliveira, Anderson Büker; Leipnitz, Guilhian; Wajner, Moacir