Aminoterminal extension peptides from type I procollagen normalize excessive collagen synthesis of scleroderma fibroblasts

Krieg, Thomas; Hörlein, D; Wiestner, Manfred; Müller, Peter

doi:10.1007/bf00446438

Cited by 83 publications

(28 citation statements)

References 16 publications

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“…Fibroblast cultures were established from skin biopsies of the upper arm of the patient and of healthy individuals. The cells were subcultured as previously described [21], and labeling experiments were performed by using the cultures in the third passage just prior to confluency. Ceils were pre-incubated for 24 h in Dulbecco's modified Eagle's medium containing sodium ascorbate (50 btg/mL), BAPN (100 l~g/mL), penicillin (400 U/mL) at 37 ° C, and subsequently labeled for 24 h in identical medium supplemented with 10 txCi/mL L-[2,3-]aH proline.…”

Section: Labeling Experiments In Organ and Celt Culturesmentioning

confidence: 99%

Case report and study of collagen metabolism in marfan's syndrome

et al. 1981

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The case report on a 33 year old woman with prominent features of Marfan's syndrome is presented. Characteristic signs were seen in the bones, the eyes, the cardiovascular system, and the lungs. Due to regurgitation of both the aortic and mitral valves and an aneurysm of the ascending aorta a double valve replacement was made, including a prosthesis of the aorta. The problems of early diagnosis and therapy of the life-threatening cardiovascular complications are discussed. Tissue specimens from the aorta were analysed histochemically and biochemically. Histology showed a typical necrosis of the media with cyst formation. Biochemical analysis by in vitro labeling of collagen in tissue explants and by electron microscopical evaluation showed proportions of type I and type III collagen which were significantly different from controls. In both the media and the adventitia the amount of type I collagen was drastically reduced as shown by quantitation of collagen and procollagen. Fibroblasts derived from the skin of the patient showed a normal content of type I and type III collagen. It is conceivable that the reduced content of type I collagen in the aortic wall is responsible for the weakness of the vessel wall causing formation of aneurysm and its sequelae.

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Section: Labeling Experiments In Organ and Celt Culturesmentioning

confidence: 99%

Case report and study of collagen metabolism in marfan's syndrome

et al. 1981

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“…This was later substantiated by several other investigators. Furthermore it became clear that overproduction of collagen could only be detected in some cell strains and only in early subcultures [26][27][28][29]. To a certain extent this phenomenon could be explained by a selection of fibroblast populations.…”

Section: Smentioning

confidence: 99%

Control of Fibrosis in Systemic Scleroderma.

Mauch¹,

Eckes²,

Hunzelmann³

et al. 1993

J Invest Dermatol

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Scleroderma is characterized by an excessive deposition of collagen in all involved organs. This is due to an overproduction of extracellular matrix (ECM) molecules following induction of gene expression, whereas there is no evidence that the composition of the connective tissue matrix is altered. Several in vivo studies and in vitro experiments suggest that a close interaction between inflammatory cells and fibroblasts is required for the initial activation of fibroblasts. TGF-beta presumably plays an important role, but other cytokines, e.g., PDGF or FGF, may also be involved. Many of the ECM molecules have been shown to interact closely with fibroblasts and provide signals that regulate fibroblast metabolism. The cellular response towards those signals is a further aspect of fibrosis that has attracted attention during recent years. The altered expression of receptor proteins on the cell surface of scleroderma fibroblasts for example might explain in part the lack of down-regulation of collagen synthesis in late phases of the disease. This review summarizes the alterations of connective tissue in scleroderma, and discusses the role of cytokines as well as the ECM for the regulation of fibroblast function and their implication for the development of fibrosis.

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“…Possible mediators of collagen regulation in vivo are the pro-peptides. The potential significance of this control in pathological conditions is supported by the observation that the increased synthesis observed in cultured fibroblasts from active scleroderma can be reduced to normal by adding amino terminal precursor sequences (KRIEG et al 1978). The same compound and some of its fragmentation products selectively inhibit the translation of collagen mRNA without affecting this process for other mRNAs (PAGLIA et al 1979).…”

Section: Ti Collagenmentioning

confidence: 99%