Gene transfer of master autophagy regulator TFEB results in clearance of toxic protein and correction of hepatic disease in alpha-1-anti-trypsin deficiency. - Wikidata - wikimedia - TriplyDB

Gene transfer of master autophagy regulator TFEB results in clearance of toxic protein and correction of hepatic disease in alpha-1-anti-trypsin deficiency.

Gene transfer of master autophagy regulator TFEB results in clearance of toxic protein and correction of hepatic disease in alpha-1-anti-trypsin deficiency.

http://www.wikidata.org/entity/Q41106554

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Update on Clinical Trials in Dry Age-related Macular Degeneration Autophagy in hepatocellular carcinomas: from pathophysiology to therapeutic response Ethanol-induced oxidant stress modulates hepatic autophagy and proteasome activity Capitalizing on the autophagic response for treatment of liver disease caused by alpha-1-antitrypsin deficiency and other genetic diseases Autophagy in hepatitis C virus-host interactions: potential roles and therapeutic targets for liver-associated diseases Autophagy and heterophagy dysregulation leads to retinal pigment epithelium dysfunction and development of age-related macular degeneration TFEB at a glance Transcriptional and epigenetic regulation of autophagy in aging Biphasic regulation of lysosomal exocytosis by oxidative stress TFE3 regulates whole-body energy metabolism in cooperation with TFEB.Lysosomal adaptation: how the lysosome responds to external cues Novel roles for the MiTF/TFE family of transcription factors in organelle biogenesis, nutrient sensing, and energy homeostasis Rag GTPases are cardioprotective by regulating lysosomal function.Innate host defense requires TFEB-mediated transcription of cytoprotective and antimicrobial genes Atg7 deficiency impairs host defense against Klebsiella pneumoniae by impacting bacterial clearance, survival and inflammatory responses in mice.Targeting intracellular degradation pathways for treatment of liver disease caused by α1-antitrypsin deficiency Signals from the lysosome: a control centre for cellular clearance and energy metabolism.Lysosomal dysfunction and impaired autophagy underlie the pathogenesis of amyloidogenic light chain-mediated cardiotoxicity.Mitophagy: therapeutic potentials for liver disease and beyond.Therapeutic targeting of misfolding and conformational change in α1-antitrypsin deficiency.Transition metals activate TFEB in overexpressing cells Enhancing Autophagy with Drugs or Lung-directed Gene Therapy Reverses the Pathological Effects of Respiratory Epithelial Cell Proteinopathy.Drosophila Mitf regulates the V-ATPase and the lysosomal-autophagic pathway.Molecular regulation of autophagy and its implications for metabolic diseases.Sustained knockdown of a disease-causing gene in patient-specific induced pluripotent stem cells using lentiviral vector-based gene therapy.Dry age-related macular degeneration: mechanisms, therapeutic targets, and imaging.Bile Duct Ligation Induces ATZ Globule Clearance in a Mouse Model of α-1 Antitrypsin Deficiency.Enhancing lysosomal biogenesis and autophagic flux by activating the transcription factor EB protects against cadmium-induced neurotoxicity.α1-antitrypsin deficiency and the hepatocytes - an elegans solution to drug discovery.Mysteries of α1-antitrypsin deficiency: emerging therapeutic strategies for a challenging disease.Liver autophagy: much more than just taking out the trash.Autophagy in hepatic fibrosis.Understanding macrophage diversity at the ontogenic and transcriptomic levels.Congenital disorders of autophagy: an emerging novel class of inborn errors of neuro-metabolism.Autophagy regulates hepatocyte identity and epithelial-to-mesenchymal and mesenchymal-to-epithelial transitions promoting Snail degradation Transcription factor EB: from master coordinator of lysosomal pathways to candidate therapeutic target in degenerative storage diseases.Activation of the c-Jun N-terminal kinase pathway aggravates proteotoxicity of hepatic mutant Z alpha1-antitrypsin.Autophagy in the liver: functions in health and disease.Autophagy modulation: a potential therapeutic approach in cardiac hypertrophy.In Silico Modeling of Liver Metabolism in a Human Disease Reveals a Key Enzyme for Histidine and Histamine Homeostasis.

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P2860

Gene transfer of master autophagy regulator TFEB results in clearance of toxic protein and correction of hepatic disease in alpha-1-anti-trypsin deficiency.

http://www.wikidata.org/entity/Q41106554

description

2013 nî lūn-bûn

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2013年论文

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2013年论文

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name

Gene transfer of master autoph ...... pha-1-anti-trypsin deficiency.

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Gene transfer of master autoph ...... pha-1-anti-trypsin deficiency.

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Gene transfer of master autoph ...... pha-1-anti-trypsin deficiency.

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EMBO Molecular Medicine

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Gene transfer of master autoph ...... pha-1-anti-trypsin deficiency.

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Donna Palmer

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Elena Polishchuk

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Francesco Vetrini

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Keith Blomenkamp

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Nunzia Pastore

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Philip Ng

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Pratibha Mithbaokar

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Roman Polishchuk

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Rosa Maria Sepe

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P2860

Guidelines for the use and interpretation of assays for monitoring autophagy

p62/SQSTM1 binds directly to Atg8/LC3 to facilitate degradation of ubiquitinated protein aggregates by autophagy

Coupling of a signal response domain in I kappa B alpha to multiple pathways for NF-kappa B activation

I kappa B epsilon, a novel member of the I kappa B family, controls RelA and cRel NF-kappa B activity.

A gene network regulating lysosomal biogenesis and function

Lifelong elimination of hyperbilirubinemia in the Gunn rat with a single injection of helper-dependent adenoviral vector

Small cargo proteins and large aggregates can traverse the Golgi by a common mechanism without leaving the lumen of cisternae

Impairment of starvation-induced and constitutive autophagy in Atg7-deficient mice

TFEB links autophagy to lysosomal biogenesis

A lysosome-to-nucleus signalling mechanism senses and regulates the lysosome via mTOR and TFEB

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10.1002/EMMM.201202046

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5

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Pasquale Piccolo

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