Summary
Biochemical pathogenesis of the aortic connective tissue diseases (such as, Marfan's syndrome, dissecting aneurysm or aortic aneurysm) was examined by estimating glycoprotein, collagen and elastin contents in the aorta and the intramolecular cross-linking component (isodesmosine) and the intermolecular cross-linking components (cystine, histidinoalanine) in comparison with the control samples obtained from subjects with aortic regurgitation. The elastin content in the aorta and isodesmosine content obtained from the extract of the aortic sample found to be decreased. Ratio of cysteine residues (Cys/Cys-Cys) in the elastin fraction in disease increased. Content of histidinoalanine was found to be decreased. It may be suggested that elastin is maintained in its native nature and shape by intra- and inter-molecular cross-linking bridges, and they are readily denatured by various disease conditions. After elastin was solubilized by elastase, immunoreactive elastin content in those aortic diseases was found to be increased in the human connective tissue. Serum elastase and elastase-like activities tend to increase more than those in the control. These findings may suggest that the change in the structure of elastin would make more susceptible to elastase and other proteolytic enzymes. The reasonable hypothesis may be that molecular defect of fibillin or other constitutional structural glycoproteins produce deficient and functionally incompetent elastin associated microfibrils, and the defect of microfibrils cause to insufficient intra- and inter-molecular cross-links in elastin.
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References
Abraham PA, Perejde AJ, Carnes WH, Uitto J (1982) J Clin Invest 70: 1245–1252
Campa JS, Greenhalgh RM, Powell JT (1987) Atherosclerosis 65: 13–21
Dubick MA, Hunter GC, Perez-Lizano E, Mar G, Geokas MC (1988) Clin Chim Acta 177: 1–10
Fischer GM, Liaurado JG (1966) Circ Res 19: 394–399
Fujimoto D (1982) Biochem Biophys Res Commun 109: 1264–1269
Hino M, Fuyamada H, Hayakawa T, Nagatsu T, Oya H, Nakagawa Y, Takemoto T, Sakakibara S (1976) Clin Chem 22: 1256–1261
Hollister DW, Godfrey M, Sakai LY, Pyeritz RE (1990) N Engl J Med 323: 152–159
Indik I, Yeh H, Ornstein-Goldstein N, Rosenbloom J (1990) In: Sandell LJ, Boyd CD (eds) Extracellular matrix genes. Academic Press, New York, p 223
Inoue H, Seyama Y, Iikura Y, Yamashita S (1982) Jpn J Clin Chem 11: 29–37
Jamall IS, Finelli VN, Que-Hee SS (1981) Anal Biochem 112: 70–75
Katagiri K, Ito K, Miyaji M, Kakeuchi T, Yoshikane K, Sasaki M (1979) Clin Chim Acta 95: 401–404
Lee B, Godfrey M, Vitale E, Hori H, Mattei MG, Sarfarazi M, Tsipouras P, Ramirez F, Hollister DW (1991) Nature 352: 330–334
Maslen CL, Corson GM, Maddox BK, Glanville RW, Sakai LY (1991) Nature 352: 334–339
Mecham RP, Lange G (1982) Methods Enzymol 82: 744–759
Rasmussen BL, Brenger E, Sandberg LB (1975) Anal Biochem 64: 255–259
Rennard SI, Berg R, Martin GR, Foidart JM, Robey PG (1980) Anal Biochem 104: 205–214
Seyama Y, Usami E, Yamashita S (1982) Chem Pharm Bull (Tokyo) 30: 644–651
Seyama Y, Inoue H, Iwama M, Watanabe H, Irie M, Orimo H, Yamashita S (1987) Jpn J Clin Chem 16: 38–47
Seyama Y, Hayashi M, Usami E, Tsuchida H, Tokudome S, Yamashita S (1990) Jpn J Clin Chem 19: 53–61
Sueyoshi T, Miyata T, Iwanaga S, Toyooka T, Imai K (1985) J Biochem 97: 1811–1813
Toyooka T, Imai K (1983) J Chromatogr 282: 495–500
Yao K, Ubuka T, Masuoka M, Ikeda T (1989) Anal Biochem 179: 332–335
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Seyama, Y., Hayashi, M., Usami, E. et al. Change in elastin structure in human aortic connective tissue diseases. Amino Acids 3, 287–292 (1992). https://doi.org/10.1007/BF00806003
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DOI: https://doi.org/10.1007/BF00806003