Pub Date : 2021-07-29DOI: 10.1201/9781003210016-17
Joan G. Clark
The connective tissue in normal lung confers mechanical strength and elastic recoil properties that allow the lung to undergo repeated cycles of expansion and relaxation. Pulmonary fibrosis develops as a consequence of a wide variety of insults to the lung. Heterogeneity among patients with pulmonary fibrosis may also be reflected in the activity and rate of progression of disease. Intraalveolar connective tissue may also be present, and in some instances, it is the predominant finding. In advanced stages of pulmonary fibrosis, the normal architecture of the lung is partially obliterated: the alveoli are replaced by dense, disorganized connective tissue, and the terminal airways are dilated and thickened, giving the lung a “honeycomb” appearance. The pivotal role of lung connective tissue in the pathogenesis of pulmonary fibrosis has been appreciated for some time. Diverse experimental approaches, utilizing model systems of varying levels of biological complexity, have been employed in investigations aimed at elucidating the biochemical basis of pulmonary fibrosis.
{"title":"The Molecular Pathology of Pulmonary Fibrosis","authors":"Joan G. Clark","doi":"10.1201/9781003210016-17","DOIUrl":"https://doi.org/10.1201/9781003210016-17","url":null,"abstract":"The connective tissue in normal lung confers mechanical strength and elastic recoil properties that allow the lung to undergo repeated cycles of expansion and relaxation. Pulmonary fibrosis develops as a consequence of a wide variety of insults to the lung. Heterogeneity among patients with pulmonary fibrosis may also be reflected in the activity and rate of progression of disease. Intraalveolar connective tissue may also be present, and in some instances, it is the predominant finding. In advanced stages of pulmonary fibrosis, the normal architecture of the lung is partially obliterated: the alveoli are replaced by dense, disorganized connective tissue, and the terminal airways are dilated and thickened, giving the lung a “honeycomb” appearance. The pivotal role of lung connective tissue in the pathogenesis of pulmonary fibrosis has been appreciated for some time. Diverse experimental approaches, utilizing model systems of varying levels of biological complexity, have been employed in investigations aimed at elucidating the biochemical basis of pulmonary fibrosis.","PeriodicalId":262745,"journal":{"name":"Connective Tissue Disease","volume":"59 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2021-07-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"131988968","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2021-07-29DOI: 10.1201/9781003210016-10
M. Lark, L. Culp
{"title":"Cell-Matrix Interactions: Biochemistry of Close and Tight-Focal Adhesive Contacts of Fibroblasts to Extracellular Matrices","authors":"M. Lark, L. Culp","doi":"10.1201/9781003210016-10","DOIUrl":"https://doi.org/10.1201/9781003210016-10","url":null,"abstract":"","PeriodicalId":262745,"journal":{"name":"Connective Tissue Disease","volume":"18 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2021-07-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"114673605","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2021-07-29DOI: 10.1201/9781003210016-16
R. Minor, J. Wootton, Donald F. Patterson, J. Uitto, Donald Bartel
{"title":"Genetic Diseases of Collagen in Animals","authors":"R. Minor, J. Wootton, Donald F. Patterson, J. Uitto, Donald Bartel","doi":"10.1201/9781003210016-16","DOIUrl":"https://doi.org/10.1201/9781003210016-16","url":null,"abstract":"","PeriodicalId":262745,"journal":{"name":"Connective Tissue Disease","volume":"1 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2021-07-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"129883676","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"The Microfibrillar Component of the Elastic Fibers Morphology and biochemistry","authors":"E. Cleary","doi":"10.1201/9781003210016-4","DOIUrl":"https://doi.org/10.1201/9781003210016-4","url":null,"abstract":"","PeriodicalId":262745,"journal":{"name":"Connective Tissue Disease","volume":"13 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2021-07-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"117245344","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2021-07-29DOI: 10.1201/9781003210016-12
D. S. Neblock, R. Berg
{"title":"Intracellular Degradation as a Modulator of Collagen Production","authors":"D. S. Neblock, R. Berg","doi":"10.1201/9781003210016-12","DOIUrl":"https://doi.org/10.1201/9781003210016-12","url":null,"abstract":"","PeriodicalId":262745,"journal":{"name":"Connective Tissue Disease","volume":"43 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2021-07-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"115966904","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"The Collagen Family of Proteins","authors":"R. Burgeson, N. Morris","doi":"10.1201/9781003210016-2","DOIUrl":"https://doi.org/10.1201/9781003210016-2","url":null,"abstract":"","PeriodicalId":262745,"journal":{"name":"Connective Tissue Disease","volume":"7 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2021-07-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"134164829","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2021-07-29DOI: 10.1201/9781003210016-24
A. J. Perejda
{"title":"Vascular and Cutaneous Complications of Diabetes Mellitus","authors":"A. J. Perejda","doi":"10.1201/9781003210016-24","DOIUrl":"https://doi.org/10.1201/9781003210016-24","url":null,"abstract":"","PeriodicalId":262745,"journal":{"name":"Connective Tissue Disease","volume":"40 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2021-07-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"117272557","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
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