Collagen in human aorta. Changes in the type III/I ratio and concentration of the reducible crosslink, dehydrohydroxylysinonorleucine in ascending aorta from healthy subjects of different age and patients with annulo-aortic ectasia.

The type III/I + III collagen ratio was studied in intima-medial samples of ascending aortas obtained from patients with the Marfan syndrome or other annulo-aortic ectasia (dilatation of the ascending aorta) and from control subjects, using electrophoretic analysis of cyanogen bromide peptides. The [3H]borohydride-reduced crosslinks of collagens were analysed by ion-exchange chromatography. Type III/I + III collagen ratios were twice as high in adult aortas as those found in skin samples of the same age.

Lysyl oxidase activity and synthesis of desmosines in cultured human aortic cells and skin fibroblasts: comparison of cell lines from control subjects and patients with the Marfan syndrome or other annulo-aortic ectasia.

The activity of lysyl oxidase, the cross-linking enzyme of elastin and collagen, was measured in culture media of human skin fibroblasts, human aortic medial smooth muscle cells (SMCs) and adventitial fibroblasts using [3H]lysine-labelled elastin substrate. In addition, biosynthesis of isodesmosine and desmosine, the cross-linking amino acids of elastin, was studied by metabolic labelling with [14C]lysine and subsequent amino acid chromatography of protein hydrolysates. Lysyl oxidase activity in culture media of skin fibroblasts and aortic smooth muscle cells increased with the growth of the cell population and was at the highest level in cultures of high cell density.

The borohydride-reducible compounds of human aortic elastin. Demonstration of a new cyclic amino acid in alkali hydrolysate, and changes with age and in patients with annulo-aortic ectasia including one with Marfan syndrome.

Human aortic elastin reduced with [3H]borohydride was analysed by ion-exchange chromatography after alkali or acid hydrolysis. Alkali hydrolysates of elastins contained a radioactive peak that was eluted between proline and leucine. This peak was not present in foetal elastin, but its proportion increased steadily during aging.

Elastin and collagen in the aortic wall: changes in the Marfan syndrome and annuloaortic ectasia.

Elastin and collagen concentrations were determined in intimal-medial samples of ascending aortas from healthy controls of different ages and from 20 patients with annuloaortic ectasia (AAE). Five patients had the Marfan syndrome. In controls the highest elastin concentrations (estimated from desmosine concentrations or insoluble residues after hot-alkali extraction) were found in children.

Xanthoma tendinosum in a normolipemic ectodermal dysplasia patient.

A female patient with congenital ectodermal dysplasia is described, who developed xanthoma tendinosum in her hands simulating rheumatoid arthritis. The serum lipids of this patient were normal. Both ectodermal dysplasia and xanthoma tendinosum in a normolipemic patient are rather uncommon and to our knowledge have not been previously reported in one and the same patient.

Desmosines in aneurysms of the ascending aorta (annulo-aortic ectasia).

Amino acid chromatography was used for determination of the elastin-specific amino acids desmosine and isodesmosine in acid hydrolyzates of intima-medial samples taken intraoperatively from aneurysms of human ascending aorta. Elastin concentration of the specimens was also estimated by hot alkali extraction followed by nitrogen determination of the extracted material and the insoluble residue. All patients studied had annulo-aortic ectasia i.

Type IIA hyperlipoproteinemic sera decrease the synthesis of hyaluronic acid by cultured human aortic smooth muscle cells.

The synthesis of sulphated glucosaminoglycans (S-GAGs) and hyaluronic acid (HA) by cultured human aortic smooth muscle cells (SMC) was measured in the presence of sera from type IIA hyperlipoproteinemic patients (7 males and 6 females) and their age- and sex-matched controls. Experiments with pooled sera showed that type IIA and control sera differed in their effects on the synthesis of GAGs at high serum concentrations with preincubation times of 24 and 48 h. Therefore, individual sera were tested at 15% with 24 h preincubation.

Collagen synthesis in cells cultured from v. Recklinghausen's neurofibromatosis.

Subcutaneous tumors of a patient with v. Recklinghausen's neurofibromatosis contained about 31% collagen calculated on the basis of lipid-free dry weight. Slices of the tumors synthesized collagen at a rate (4.

A rapid assay to measure collagen synthesis in cell cultures.

Limited pepsin digestion and precipitation of resistant parts of proteins with perchloric acid on glass-fiber filters has been used as a rapid way to determine the radioactive collagen secreted into fibroblast culture media. The specificity of the pepsin cleavage was tested by digesting [14C]- or [3H]proline- and [3H]tyrosine-labeled procollagens. Radioactivities obtained with this method were comparable with those obtained with collagenase digestions or hydroxyproline determinations.

Massive cutaneous hyalinosis. A newly recognized disease.

A disease characterized by massive tumorous cutaneous hyalinosis has been studied histologically, immunologically, and biochemically. The precipitated hyalin material differed from amyloid in being Congo-red-negative and ultrastructurally nonfibrillary. In lipoid proteinosis, massive hyalin deposits have not been encountered and the clinical course is distinct from the course of massive cutaneous hyalinosis.

Serum lipoprotein composition, hormones, and the synthesis of glycosaminoglycans by human aortic smooth muscle cells.

The synthesis of hyaluronic acid (HA) and sulphated glycosaminoglycans (S-GAGs) by cultured human aortic smooth muscle cells (SMC) was measured in the presence of human sera with high LDL-cholesterol concentration (type IIA hyperlipoproteinemic patients) or high HDL-cholesterol concentration (marathon runners and lumberjacks). In addition, the effect of some hormones on GAG-synthesis was studied. As compared to normolipidemic controls, type IIA hyperlipoproteinemic sera decreased markedly the synthesis of HA without affecting the synthesis of S-GAGs.

Effect of sera from hyperlipidemic subjects and high-density lipoproteinemic runners on the synthesis of DNA and glycosaminoglycans by cultured human aortic smooth muscle cells.

Effects of hyperlipidemic, normolipidemic and high-density-lipoproteinemic (HD lipoproteinemic) sera from active runners were studied on cultured human aortic smooth muscle cells. The synthesis of DNA, hyaluronic acid and sulphated glycosaminoglycans (GAGs) in the presence of the various sera was measured by the incorporation of [3H]-thymidine and [3H]-glucosamine. The HD lipoproteinemic sera from runners stimulated the synthesis of DNA and sulphated GAGs less than normolipidemic and hyperlipidemic sera.

High density lipoproteinemia due to vigorous physical work inhibits the incorporation of [3H]thymidine and the synthesis of glycosaminoglycans by human aortic smooth muscle cells in culture.

The effect of sera from normolipidemic men engaging in normal physical activity and from high density lipoproteinemic lumberjacks engaging in vigorous physical activity on the incorporation of [3H]thymidine and the synthesis of glycosaminoglycans by human aortic smooth muscle cells in culture was measured. At high concentration (15%) high density lipoproteinemic serum inhibited significantly (P less than 0.001) the incorporation of thymidine.

Identification of glutamine as a hepatic factor which influence the synthesis of collagen by freshly isolated fibroblasts.

Earlier we have found that 35,000 g supernatants of liver homogenates from both normal and hypercholesterolaemic rats stimulate the synthesis of collagen by freshly isolated fibroblasts. Supernatants from the fatty livers of hypercholesterolaemic rats showed greater stimulation. In the present study we fractionated the 35,000 g supernatants using gel filtration, ion-exchange chromatography, paper electrophoresis and paper chromatography.

Energy metabolism of experimental wounds at various oxygen environments.

Energy metabolism of healing tissue was studied in experimental wounds of rats chronically breathing 11% O2, air or 55% O2. Increasing oxygen supply elevated both PO2 and PCO2 in the wound tissue. At the early phases of healing hypoxic wounds contained less DNA than normoxic or hyperoxic tissues.

Effect of changes in inspired oxygen tension on wound metabolism.

This work was prompted by earlier findings of the beneficial effect of increased oxygen supply on wound healing. Enzyme activities in the limiting step of glycolysis, citric acid cycle and pentose phosphate cycle were determined in cellulose sponge implants of rats chronically, breathing 12% O(2), air or 55% O(2.) Respiratory gas tensions and concentrations of pyruvate and lactate were measured in wound fluid aspirated from the implants.