Current Pathobiology Reports最新文献_第9页

Bioinductive Scaffolds—Powerhouses of Skeletal Muscle Tissue Engineering 生物诱导支架——骨骼肌组织工程的动力

Current Pathobiology Reports Pub Date : 2017-08-14 DOI: 10.1007/s40139-017-0151-9

H. Iyer, R. Galiano

引用次数: 0

Future Challenges in the Generation of Hepatocyte-Like Cells From Human Pluripotent Stem Cells 从人全能干细胞产生肝细胞样细胞的未来挑战

Current Pathobiology Reports Pub Date : 2017-07-31 DOI: 10.1007/s40139-017-0150-x

Richard Siller, Sebastian Greenhough, Santosh Mathapati, K. Si-Tayeb, G. Sullivan

引用次数: 1

Mitochondrial Fatty Acid Oxidation Disorders Associated with Cardiac Disease 与心脏病相关的线粒体脂肪酸氧化障碍

Current Pathobiology Reports Pub Date : 2017-07-24 DOI: 10.1007/s40139-017-0148-4

A. El-Gharbawy, A. Goldstein

引用次数: 2

Pathobiology of the Hepatic Glycogen Storage Diseases 肝糖原贮藏病的病理生物学研究

Current Pathobiology Reports Pub Date : 2017-06-06 DOI: 10.1007/s40139-017-0146-6

P. McKiernan

引用次数: 0

ER Stress and Autophagy in Obesity and Nonalcoholic Fatty Liver Disease 肥胖和非酒精性脂肪肝患者的ER应激与自噬

Current Pathobiology Reports Pub Date : 2017-06-02 DOI: 10.1007/s40139-017-0145-7

Nicholas R. L. Lind, Q. Qian, Ling Yang

引用次数: 0

An interspecies heart-to-heart: Using Xenopus to uncover the genetic basis of congenital heart disease. 种间心对心:利用非洲爪蟾揭示先天性心脏病的遗传基础。

Current Pathobiology Reports Pub Date : 2017-06-01 Epub Date: 2017-05-06 DOI: 10.1007/s40139-017-0142-x

Alexandra MacColl Garfinkel, Mustafa K Khokha

{"title":"An interspecies heart-to-heart: Using Xenopus to uncover the genetic basis of congenital heart disease.","authors":"Alexandra MacColl Garfinkel, Mustafa K Khokha","doi":"10.1007/s40139-017-0142-x","DOIUrl":"https://doi.org/10.1007/s40139-017-0142-x","url":null,"abstract":"Purpose of review: Given the enormous impact congenital heart disease has on child health, it is imperative that we improve our understanding of the disease mechanisms that underlie patient phenotypes and clinical outcomes. This review will outline the merits of using the frog model, Xenopus, as a tool to study human cardiac development and left-right patterning mechanisms associated with congenital heart disease.Recent findings: Patient-driven gene discovery continues to provide new insight into the mechanisms of congenital heart disease, and by extension, patient phenotypes and outcomes. By identifying gene variants in CHD patients, studies in Xenopus have elucidated the molecular mechanisms of how these candidate genes affect cardiac development, both cardiogenesis as well as left-right patterning, which can have a major impact on cardiac morphogenesis. Xenopus has also proved to be a useful screening tool for the biological relevance of identified patient-mutations, and ongoing investigations continue to illuminate disease mechanisms.Summary: Analyses in model organisms can help to elucidate the disease mechanisms underlying CHD patient phenotypes. Using Xenopus to disentangle the genotype-phenotype relationships of well-known and novel disease genes could enhance the ability of physicians to efficaciously treat patients and predict clinical outcomes, ultimately improving quality of life and survival rates of patients born with congenital heart disease.","PeriodicalId":37014,"journal":{"name":"Current Pathobiology Reports","volume":"5 2","pages":"187-196"},"PeriodicalIF":0.0,"publicationDate":"2017-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1007/s40139-017-0142-x","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"35649150","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 16

Autophagy and Ferroptosis - What's the Connection? 自噬和铁蛋白沉积--有什么联系？

Current Pathobiology Reports Pub Date : 2017-06-01 Epub Date: 2017-04-20 DOI: 10.1007/s40139-017-0139-5

Rui Kang, Daolin Tang

引用次数: 0

Using zebrafish to model liver diseases-Where do we stand? 用斑马鱼来模拟肝脏疾病——我们的研究进展如何?

Current Pathobiology Reports Pub Date : 2017-06-01 Epub Date: 2017-05-03 DOI: 10.1007/s40139-017-0141-y

Duc-Hung Pham, Changwen Zhang, Chunyue Yin

{"title":"Using zebrafish to model liver diseases-Where do we stand?","authors":"Duc-Hung Pham, Changwen Zhang, Chunyue Yin","doi":"10.1007/s40139-017-0141-y","DOIUrl":"https://doi.org/10.1007/s40139-017-0141-y","url":null,"abstract":"Purpose of review: The liver is the largest internal organ and performs both exocrine and endocrine function that is necessary for survival. Liver failure is among the leading causes of death and represents a major global health burden. Liver transplantation is the only effective treatment for end-stage liver diseases. Animal models advance our understanding of liver disease etiology and hold promise for the development of alternative therapies. Zebrafish has become an increasingly popular system for modeling liver diseases and complements the rodent models.Recent findings: The zebrafish liver contains main cell types that are found in mammalian liver and exhibits similar pathogenic responses to environmental insults and genetic mutations. Zebrafish have been used to model neonatal cholestasis, cholangiopathies, such as polycystic liver disease, alcoholic liver disease, and non-alcoholic fatty liver disease. It also provides a unique opportunity to study the plasticity of liver parenchymal cells during regeneration.Summary: In this review, we summarize the recent work of building zebrafish models of liver diseases. We highlight how these studies have brought new knowledge of disease mechanisms. We also discuss the advantages and challenges of using zebrafish to model liver diseases.","PeriodicalId":37014,"journal":{"name":"Current Pathobiology Reports","volume":"5 2","pages":"207-221"},"PeriodicalIF":0.0,"publicationDate":"2017-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1007/s40139-017-0141-y","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"35567953","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 25

Non-canonical activation of NRF2: New insights and its relevance to disease. NRF2的非规范激活:新见解及其与疾病的相关性。

Current Pathobiology Reports Pub Date : 2017-06-01 Epub Date: 2017-04-19 DOI: 10.1007/s40139-017-0131-0

Matthew Dodson, Donna D Zhang

{"title":"Non-canonical activation of NRF2: New insights and its relevance to disease.","authors":"Matthew Dodson, Donna D Zhang","doi":"10.1007/s40139-017-0131-0","DOIUrl":"https://doi.org/10.1007/s40139-017-0131-0","url":null,"abstract":"Purpose of review: The goal of this review is to summarize the current knowledge in the field regarding the non-canonical activation of the NRF2 pathway. Specifically, we address what role p62 plays in mediating this pathway, which pathologies have been linked to the p62-dependent activation of NRF2, as well as what therapeutic strategies could be used to treat diseases associated with the non-canonical pathway.Recent findings: It has recently been shown that autophagic dysfunction leads to the aggregation or autophagosomal accumulation of p62, which sequesters KEAP1, resulting in prolonged activation of NRF2. The ability of p62 to outcompete NRF2 for KEAP1 binding depends on its abundance, or post-translational modifications to its key domains. Furthermore, the relevance of the p62-dependent activation of NRF2 in disease has been demonstrated in human hepatocellular carcinomas, as well as neurodegenerative diseases.Summary: These findings indicate that targeting p62, or the enzymes that modify it, could prove to be an advantageous strategy for treating diseases associated with autophagy dysregulation and prolonged activation of NRF2. Other therapeutic possibilities include restoring proper autophagic function, or directly inhibiting NRF2 or its targets, to restore redox and metabolic homeostasis. Future studies will help further clarify the mechanisms, regulation, and relevance of the non-canonical pathway in driving disease pathogenesis.","PeriodicalId":37014,"journal":{"name":"Current Pathobiology Reports","volume":"5 2","pages":"171-176"},"PeriodicalIF":0.0,"publicationDate":"2017-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1007/s40139-017-0131-0","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"35649148","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 26

Regulation of Exercise-Induced Autophagy in Skeletal Muscle. 运动诱导骨骼肌自噬的调控。

Current Pathobiology Reports Pub Date : 2017-06-01 Epub Date: 2017-04-20 DOI: 10.1007/s40139-017-0135-9

Altea Rocchi, Congcong He

{"title":"Regulation of Exercise-Induced Autophagy in Skeletal Muscle.","authors":"Altea Rocchi, Congcong He","doi":"10.1007/s40139-017-0135-9","DOIUrl":"10.1007/s40139-017-0135-9","url":null,"abstract":"Purpose of review: Physical exercise is a highly effective method to prevent several pathogenic conditions, such as obesity, type 2 diabetes and cardiovascular diseases, largely due to metabolic adaptations induced by exercise in skeletal muscle. Yet how exercise induces the beneficial effects in muscle remains to be fully elucidated. Autophagy is a lysosomal degradation pathway that regulates nutrient recycling, energy production and organelle quality control. The autophagy pathway is upregulated in response to stress during exercise and muscle contraction, and may be an important mechanism mediating exercise-induced health benefits.Recent findings: A number of studies have indicated that physical exercise induces non-selective autophagy and selective mitophagy in skeletal muscle in animal models and humans. The AMPK-ULK1 and the FoxO3 signaling pathways play an essential role in the activation of the upstream autophagy machinery in skeletal muscle during exercise. The autophagy activity is required for health benefits of exercise, as in different autophagy-deficient mouse lines exercise-induced effects are abolished.Summary: This review aims to summarize and highlight the most recent findings on the role of autophagy in muscle maintenance, the molecular pathways that upregulate autophagy during exercise, and the potential functions of exercise-induced autophagy and mitophagy in skeletal muscle.","PeriodicalId":37014,"journal":{"name":"Current Pathobiology Reports","volume":"5 2","pages":"177-186"},"PeriodicalIF":0.0,"publicationDate":"2017-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1007/s40139-017-0135-9","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"35629828","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 36