Heritable disorders of connective tissue / Victor A. McKusick.
- Victor A. McKusick
- Date:
- 1960
Licence: Attribution-NonCommercial 4.0 International (CC BY-NC 4.0)
Credit: Heritable disorders of connective tissue / Victor A. McKusick. Source: Wellcome Collection.
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![32 Heritable Disorders oj Connective Tissue Table 2. Characteristics of Collagen (A Partial Tabulation) für Eiweiss- und Lederforschung] in Regensburg). Gustavson®^ has demonstrated that thermal shrinkage is the result of breaking of crosslinks in the collagen molecule which occur mainly through hydrogen bonding between hydroxyl groups of hydroxyproline and the keto-imide groups of adjacent helices. Gustavson^^i has further suggested the intriguing possibility that in vivo tanning may be the basis of some changes seen with aging and in pathologic states. The importance of collagen in pathology will be evident to a medical audience since the concept of collagen vascular diseases has seemingly gained such wide acceptance. Further medical implications appear to be represented by certain of the hereditary disorders of connective tissue under discussion here. These implications—biologic, economic, and medical—have been responsible for very extensive investigations of the nature of collagen^-^-'^'^'^^ by scientists of di¬ verse interests and perspectives. The result has been, in the past at least, a situation like the elephant that was examined by the six blind men. Recently several excellent symposia have effected a synthesis of the diverse bits of information. Neither the ultrastructure of collagen nor the mode of its formation,is established in full detail or beyond debate. A concept which perhaps is most con¬ sistent with the information available is as follows : The building blocks of collagen, the tropocollagen unit in the terminology of Gross, Highberger, and Schmitt,is elaborated by the cell, possibly intracellularly, and is extruded into the extracellular environment where ionic and other conditions favor its orderly](https://iiif.wellcomecollection.org/image/b18030154_0037.JP2/full/800%2C/0/default.jpg)


