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Acid ceramidase improves mitochondrial function and oxidative stress in Niemann-Pick type C disease by repressing STARD1 expression and mitochondrial cholesterol accumulation.

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第一作者： Sandra,Torres

第一单位： Cell Death and Proliferation, Institute of Biomedical Research of Barcelona (IIBB), CSIC, Barcelona, Spain; Liver Unit, Hospital Clinic I Provincial de Barcelona, IDIBAPS and CIBERehd, Barcelona, Spain.

作者： Sandra,Torres ^[1] ; Estel,Solsona-Vilarrasa ^[1] ; Susana,Nuñez ^[1] ; Nuria,Matías ^[1] ; Naroa,Insausti-Urkia ^[1] ; Fernanda,Castro ^[2] ; Mireia,Casasempere ^[3] ; Gemma,Fabriás ^[3] ; Josefina,Casas ^[3] ; Carlos,Enrich ^[4] ; José C,Fernández-Checa ^[5] ; Carmen,Garcia-Ruiz ^[6]

作者单位： Cell Death and Proliferation, Institute of Biomedical Research of Barcelona (IIBB), CSIC, Barcelona, Spain; Liver Unit, Hospital Clinic I Provincial de Barcelona, IDIBAPS and CIBERehd, Barcelona, Spain. ^[1] Cell Death and Proliferation, Institute of Biomedical Research of Barcelona (IIBB), CSIC, Barcelona, Spain. ^[2] Research Unit on BioActive Molecules (RUBAM), Departament de Química Orgànica Biològica, Institut d'Investigacions Químiques i Ambientals de Barcelona, Consejo Superior de Investigaciones Científicas (CSIC), Barcelona, Spain. ^[3] Departament de Biologia Cel·lular, Immunologia i Neurociències, Facultat de Medicina, Universitat de Barcelona, 08036, Barcelona, Spain; Centre de Recerca Biomèdica CELLEX, Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), 08036, Barcelona, Spain. ^[4] Cell Death and Proliferation, Institute of Biomedical Research of Barcelona (IIBB), CSIC, Barcelona, Spain; Liver Unit, Hospital Clinic I Provincial de Barcelona, IDIBAPS and CIBERehd, Barcelona, Spain; Research Center for ALPD, Keck School of Medicine, Univerisity of Southern California, Los Angeles, CA, USA. Electronic address: checa229@yahoo.com. ^[5] Cell Death and Proliferation, Institute of Biomedical Research of Barcelona (IIBB), CSIC, Barcelona, Spain; Liver Unit, Hospital Clinic I Provincial de Barcelona, IDIBAPS and CIBERehd, Barcelona, Spain; Research Center for ALPD, Keck School of Medicine, Univerisity of Southern California, Los Angeles, CA, USA. Electronic address: cgrbam@iibb.csic.es. ^[6]

关键词 Acid ceramiase Cholesterol Mitochondrial function NPC disease Oxidative stress

医学主题词酸性神经酰胺酶(Acid Ceramidase);动物(Animals);胆固醇(Cholesterol);人类(Humans);溶酶体(Lysosomes);小鼠(Mice);小鼠, 基因敲除(Mice, Knockout);线粒体(Mitochondria);尼曼-皮克病, C型(Niemann-Pick Disease, Type C);氧化性应激(Oxidative Stress);磷蛋白类(Phosphoproteins)

DOI 10.1016/j.redox.2021.102052

PMID 34175669

发布时间 2025-01-03

基金项目

P50 AA011999/AA/NIAAA NIH HHS/United States

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Acid ceramidase improves mitochondrial function and oxidative stress in Niemann-Pick type C disease by repressing STARD1 expression and mitochondrial cholesterol accumulation.

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Acid ceramidase improves mitochondrial function and oxidative stress in Niemann-Pick type C disease by repressing STARD1 expression and mitochondrial cholesterol accumulation.

Redox biology

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Redox biology

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