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  • 1
    ISSN: 1432-0428
    Keywords: Keywords Circular dichroism ; collagen IV ; diabetes ; glycation ; nephropathy.
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Glycation of basement membrane collagen IV has been implicated as a major pathogenetic process leading to diabetic microvascular complications. To evaluate the relevance of carbohydrate-induced modifications on collagen IV in diabetic nephropathy, we isolated the cross-linking domains 7S and NC1 from the glomerular basement membrane (GBM) of patients with diabetes mellitus. Modifications characteristic for glycated proteins were identified when the domains from diabetic kidney were compared with the same domains from human placenta as an unmodified control. In both domains a marked formation of inter-and intramolecular cross links could be demonstrated by SDS-PAGE. Furthermore circular dichroism studies showed a decrease in helicity of the 7S domain from human diabetic kidneys of 13 %, indicating denaturation already at room temperature. Thermal transition profiles, showing a shift of the denaturation temperature towards a lower temperature, with loss of a distinct second melting point, confirmed this observation. Our data provide further evidence for a possible role of protein-modification by glycoxidative reactions in the onset of diabetic nephropathy in vivo. [Diabetologia (1998) 41: 1073–1079]
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1432-1440
    Keywords: Knorpel-Knochenumwandlung ; Osteoarthrose ; Chondrocyten ; Kollagensynthese ; Immunfluorescenz ; Cartilage-bone metamorphosis ; Osteoarthrosis ; Chondrocytes ; Collagen-synthesis ; Immunofluorescence
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary Synthesis of collagen by chondrocytes was studied by immunofluorescence using antibodies specific for type I, II and III collagen. The following tissues and culture conditions were chosen for this immunohistological study: normal articular cartilage, epiphyseal growth cartilage, cartilage undergoing osteoarthrotic degeneration, suspension culture and monolayer culture. While type II collagen is the unique collagen all over hyaline cartilage, type I collagen is produced by hypertrophic chondrocytes in the growth plate. In addition, chondrocytes in osteoarthrotic areas of articular cartilage synthesize type I collagen. Under in vitro culture conditions, chondrocytes initially produce type II collagen and synthesize later on type I collagen. The change of synthesis from type II to type I collagen is more rapid in monolayer than in suspension culture. It is concluded that the presence of matrix compounds and the cellmatrix interaction as well are necessary to maintain synthesis of type II collagen in chondrocytes. Alterations in the cell-matrix interactions are shown to occur in the hypertrophic zone of the epiphyseal growth plate, in cartilage undergoing osteoarthrotic degeneration as well as in chondrocytes grown in culture. Thus, change in the control of gene activity may subsequently lead to change in collagen synthesis. It is possible that the synthesis of type I collagen, which cannot fulfil the physiological function of a structural element in cartilageneous tissue, is a crucial factor in the process of osteoarthrosis.
    Notes: Zusammenfassung Unter Verwendung von spezifischen Antikörpern ist es möglich, mit Hilfe immunhistologischer Methoden die Verteilung der verschiedenen Kollagentypen und auch die Kollagensynthese einzelner Zellen zu verfolgen. Diese Methode wurde angewendet, um die Kollagensynthese von Chondrocyten im normalen Gelenkknorpel, Epiphysenknorpel der Wachstumsplatte, osteoarthrotischen Gelenkknorpel, sowie unter in vitro Kulturbedingungen zu verfolgen. Während im normalen Knorpel nur Typ II Kollagen synthetisiert wird, wurde festgestellt, daß die hypertrophierenden großen Chondrocyten an der Basis des Säulenknorpels auf die Synthese des Typ I Kollagens umschalten. Ähnliches gilt auch für die arthrotischen Zellen des Gelenkknorpels. Hier führt die Degeneration zu einer Umschaltung der Kollagensynthese von Typ II auf Typ I Kollagen. Dieses Phänomen kann auch in vitro nachvollzogen werden. In Suspensionskulturen, in denen Chondrocyten in Aggregaten eine knorpelähnliche Matrix aufzubauen vermögen, vollzieht sich der Umschaltungsprozeß langsamer als in Monolayerkulturen. Diese Beobachtungen zeigen, daß Chondrocyten zur Aufrechterhaltung ihrer Typ II Kollagensynthese eine spezielle Zell-Matrix-Wechselwirkung benötigen. Wird durch eine Veränderung der Knorpelmatrix (z.B. bei dem Degenerationsprozeß der Osteoarthrose) diese Wechselbeziehung gestört, kommt es zu einer Neuorientierung der Kollagensynthese und — in deren Folge — zur Umschaltung auf Typ I Kollagen.
    Type of Medium: Electronic Resource
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  • 3
    Electronic Resource
    Electronic Resource
    Springer
    Journal of molecular medicine 59 (1981), S. 91-93 
    ISSN: 1432-1440
    Keywords: Collagen synthesis ; Fibroblast cultures ; Osteogenesis imperfecta ; Kollagensynthese ; Fibroblastenkulturen ; Osteogenesis imperfecta
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Hautfibroblasten wurden von 13 Patienten mit Osteogenesis imperfecta gezüchtet und deren Kollagensynthese in vitro untersucht. Dabei fand sich bei 7 Patienten, die durch nur milde Manifestation der Erkrankung charakterisiert waren, eine Störung des Verhältnisses der Kollagentypen I und III. Fibroblasten von solchen Patienten mit einer schweren Form der Osteogenesis imperfecta synthetisierten die Kollagentypen I und III in einem normalen Verhältnis.
    Notes: Summary Skin fibroblasts were cultured from 13 patients with Osteogenesis imperfecta and collagen biosynthesis was investigated in vitro. In those patients characterised by only mild manifestations of the disease, the ratio of collagen types I and III was disturbed. By contrast, fibroblasts obtained from patients with Osteogenesis imperfecta of a more severe type synthesised collagen types I/III in a normal ratio.
    Type of Medium: Electronic Resource
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  • 4
    ISSN: 1432-1440
    Keywords: Constitutional connective tissue disorder ; fibroblast cultures ; collagen biosynthesis ; Konstitutionelle Bindegewebserkrankung ; Fibroblastenkulturen ; Kollagenbiosynthese
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Es wird über eine konstitutionelle Bindegewebserkrankung bei dem 10 Jahre alten Sohn blutsverwandter Eltern berichtet. Das klinische und röntgenologische Befundmuster unterscheidet sich von bisher bekannten Syndromen, insbesondere der Osteogenesis imperfecta und dem Marfan-Syndrom, mit denen gewisse Ähnlichkeiten bestehen. In Fibroblastenkulturen aus Hautbiopsien wurde die Kollagenbiosynthese untersucht. Dabei zeigte sich, daß die Fibroblasten unterschiedliche Typen von α1-Ketten synthetisieren, während α2-Ketten weder im Kulturmedium noch in Zellextrakten nachgewiesen werden konnten. Es wird vermutet, daß die Störung der Kollagenbiosynthese mit den klinischen und röntgenologischen Befunden der Erkrankung in Verbindung steht.
    Notes: Summary An apparently “new” connective tissue disorder is described in the 10 year old son of consanguineous parents. The patient showed clinical and radiographic abnormalities reminiscent of both osteogenesis imperfecta and Marfan syndrome. Collagen biosynthesis was analysed in fibroblast cultures from skin biopsies. Fibroblasts of the patient synthesized different types ofα1 chains. No α2 chains could be detected in cell extracts or in culture media suggesting a defect in the synthesis of these compounds. The peculiar pattern of clinical abnormalities may be causally related to the apparent defect in collagen synthesis.
    Type of Medium: Electronic Resource
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  • 5
    Electronic Resource
    Electronic Resource
    Springer
    Journal of molecular medicine 59 (1981), S. 83-90 
    ISSN: 1432-1440
    Keywords: Collagen ; Marfan's syndrome ; Fibroblasts ; Aortic-aneurysm ; Kollagen ; Marfan-Syndrom ; Fibroblasten ; Aorten-Aneurysma
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Der Fallbericht einer 33jährigen Frau mit einem ausgeprägtem Marfan-Syndrom wird vorgestellt. Sie hatte typische ossäre, okuläre, kardiovaskuläre und auch pulmonale Veränderungen, auf deren Bedeutung näher eingegangen wird. Wegen einer schweren Aorteninsuffizienz bei einem Aneurysma der Aorta ascendens und einer beträchtlichen Mitralinsuffizienz wurde ein Doppelklappenersatz durchgeführt und eine Prothese der Aorta ascendens eingesetzt. Die Prognose, die diagnostischen und therapeutischen Möglichkeiten der lebensbedrohlichen kardiovaskulären Komplikationen bei Marfan-Syndrom werden diskutiert. Das bei der Operation gewonnene Aortengewebe wurde histologisch und biochemisch analysiert. Histologisch zeigte sich eine typische Medianekrose. Biochemisch (Fibroblastenkultur, Elektronenmikroskopie) fand sich eine veränderte Zusammensetzung der kollagenen Anteile. Sowohl in der Adventitia wie in der Media war Kollagen Typ I nahezu völlig zugunsten von Kollagen Typ III verschwunden. Diese Verschiebung galt in gleicher Weise für die Prokollagene. In der Haut der Patientin fand sich jedoch eine normale Verteilung von Prokollagen und Kollagen der Typen I und III. Die weitgehende Reduktion von Kollagen Typ I stellt möglicherweise die Ursache der Wandschwächung der Aorta dar, die die Ursache der Aneurysmabildung und der Aorteninsuffizienz ist.
    Notes: Summary 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 prothesis 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 electon 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.
    Type of Medium: Electronic Resource
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  • 6
    ISSN: 1432-1084
    Keywords: Key words: Liver metastases ; Interventional treatment modalities ; Alcohol injection ; Drug instillation ; Radiofrequency ablation ; Cryotherapy ; Laser-induced interstitial thermotherapy ; Regional chemoembolisation
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract. The liver is the most common site of metastatic tumour deposits. Hepatic metastases are the major cause of morbidity and mortality in patients with gastrointestinal carcinomas and other malignant tumours. The rationale and results for interventional therapeutic techniques in the treatment of liver metastases are presented. For the treatment of patients with irresectable liver metastases, alternative local ablative therapeutic modalities have been developed. Technique and results of local interventional therapies are presented such as microwave-, radiofrequency (RF)- and ultrasound ablation, and laser-induced interstitial therapy (LITT), cryotherapy and local drug administration such as alcohol injection, endotumoral chemotherapy and regional chemoembolisation. In addition to cryotherapy, all ablative techniques can be performed percutaneously with low morbidity and mortality. Cryotherapy is an effective and precise technique for inducing tumour necrosis, but it is currently performed via laparotomy. Percutaneous local alcohol injection results in an inhomogeneous distribution in liver metastases with unreliable control rates. Local chemotherapeutic drug instillation and regional chemoembolisation produces relevant but non-reproducible lesions. Laser-induced interstitial thermotherapy (LITT) performed under MRI guidance results in precise and reproducible areas of induced necrosis with a local control of 94 %, and with an improved survival rate. Interventional therapeutic techniques of liver metastases do result in a remarkable local tumour control rate with improved survival results.
    Type of Medium: Electronic Resource
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  • 7
    Electronic Resource
    Electronic Resource
    Springer
    European radiology 9 (1999), S. 1479-1487 
    ISSN: 1432-1084
    Keywords: Key words: Interventional MRI ; Interstitial therapy ; MR-guided laser-induced interstitial thermotherapy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract. The rationale and results for interstitial therapies via interventional MRI in the treatment of tumors in various regions are presented. Different interstitial treatment techniques are presented based on varying technologies both for tumor ablation and treatment monitoring. Data are presented based on 335 patients, 29–84 years of age (mean age 59 years, 196 men and 139 women) with a total of 932 liver tumors, 16 head and neck tumors and 14 abdominal recurrent pelvic and lymphatic tumors. All lesions had been treated with MR-guided laser-induced interstitial thermotherapy (LITT) via 2516 laser applications and 1856 cannulations. Data in the literature are extremely varying depending on author experience, treatment technique, and the included patient material. In our patient material we were able to achieve a local tumor control of 96.7 % depending on the size of the tumorous lesion, the topographical relationship, and the applied laser parameters. The overall cumulative survival rate of patients with liver metastases was 45.74 months (median 40.97 months, 95 % confidence interval 31.42–50.52). The cumulative survival rate of the patient group with hepatic metastases of colorectal carcinoma was 42.71 months (median 39.33 months, 95 % confidence interval 33.26–45.37). In patients with head and neck tumors a relevant reduction in clinically relevant symptoms such as pain, swallowing disorders, or nervous compression was achieved in 11 of 15 patients treated with LITT. In 14 soft tissue tumors, such as pelvic tumor recurrence and lymph node metastases, a local tumor control was obtained in 68 % of lesions. Interstitial therapies under interventional MRI guidance, such as LITT, results in a high local tumor control with an improved survival rate.
    Type of Medium: Electronic Resource
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  • 8
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 125 (1977), S. 29-37 
    ISSN: 1432-1076
    Keywords: Osteogenesis imperfecta ; Collagen types ; Bone ; In vitro study ; Immunofluorescence
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Samples of bone from a patient with osteogenesis imperfecta were found to synthesize and contain type III collagen as well as type I collagen. Normal bone contains only type I collagen except in the lining cells of the bone marrow cavities. In the patient's tissue, type III collagen was localized in nonfibrillar structures in discrete areas of the bone. These and previous studies indicate that certain types of osteogenesis imperfecta may be caused by a failure of normal bone maturation and the sites in which the type III collagen is found appear to be defects in the bone.
    Type of Medium: Electronic Resource
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  • 9
    ISSN: 1432-1076
    Keywords: Osteogenesis imperfecta ; Joint contractures ; Collagen fibrils ; Mineralization
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We describe a male patient with osteogenesis imperfecta (OI) who was born with contractures of the knee, elbow and ankle joints. During the first 4 years he suffered from recurrent fractures. He has white sclerae, mild dentinogenesis imperfecta, multiple wormian bones, severe scoliosis and short stature. Morphological analysis of cortical bone revealed typical characteristics of OI including varying width of the osteoid, swollen mitochondria and a dilated endoplasmic reticulum of the osteoblasts. Collagen fibrils of the osteoid had a varying diameter, a feature not found in typical OI patients. Analysis of compact bone showed that the size of apatite crystals and the extractability of collagen with pepsin were markedly elevated compared to controls and other OI type III and IV patients. Lysyl hydroxylation of collagen from the organic bone matrix and the electrophoretic mobility of collagen α1(I)- and α2(I)-chains were normal. Our results provide evidence that this patient belongs to a subtype of OI. The biochemical studies indicate that the underlying defect involves defective fibril-formation of collagen type I leading to an altered mineralization of bone.
    Type of Medium: Electronic Resource
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  • 10
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 124 (1977), S. 101-111 
    ISSN: 1432-1076
    Keywords: Hutchinson-Gilford-syndrome ; Familial incidence ; Autosomal-recessive disorder ; Fibroblast cultures ; Collagen types ; Thymidine incorporation ; Immunofluorescence
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract This report relates the case histories of two sisters who demonstrated the typical symptoms of progeria at birth. One of these children had died previous to this study. The familial occurrence underlines the thesis that progeria is an autosomal-recessive disorder. The examination of the cultured skin fibroblasts from the younger child showed a clear decrease in cell growth. On the other hand, the immunfluorescent examination of skin biopsies and cultured skin fibroblasts revealed no atypical distribution of collagen types.
    Type of Medium: Electronic Resource
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