Gene/Protein Disease Symptom Drug Enzyme Compound
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 28,634 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The presence of the evacuation paths of aqueous humor at the level of the camerular angle makes it play an important role on open angle glaucoma pathology. The fundamental element in the evacuation of aqueous humor is the endothelial cell, who has a main contribution to the routing of aqueous humor using the phenomenon called micropinocytosis. With the age the number of endothelial cells diminishes, their function reduces and an atypical collagen appears, together with the thickness of the basal membrane, the agglutination of the trabecular blades and Schlemm channel collapse. The fact is that the raise of the evacuation resistance in open angle glaucoma must not be interpreted mechanically; a research from a biochemical and molecular point of view must be completed.
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PMID:[The resistance to drainage of the aqueous humor]. 828 12

Osteogenesis imperfecta (OI) Type II is a rare heritable disorder of bone matrix that results in catastrophic congenital skeletal dysplasia. Two cases of OI Type II had symmetric rhizomelic skeletal dysplasia apparent on ultrasound at 16 and 20 weeks' gestation. Histologic and histochemical studies performed on skeletal tissue from fetal autopsies showed the following: (1) abnormal growth plate tissue characterized by failure of formation of primary bony spongiosa; (2) persistence of calcified cartilage bars in the diaphysis; (3) metaphyseal microfractures; (4) abundant cartilaginous fracture callus; (5) absence of bony callus; (6) failure of formation of intramembranous cortical diaphyseal bone; (7) angulation of long bones in portions of the metadiaphyses bordered by fracture callus; and (8) mechanical failure of the perichondral ring of LaCroix with a normal fibrous ossification groove of Ranvier. These findings suggest that skeletal dysplasia in OI Type II results from the action of muscular forces on a skeleton weakened by a complex disorder of endochondral and intramembranous ossification. The paucity of primary metaphyseal trabeculae and subperiosteal cortical bone leads to pathologic fractures of the immature fiber bone and an imperfect attempt at fracture repair. Angulation and shortening of long bones occurs between numerous sites of focal endochondral fracture callus. Mechanical failure of the fibrous perichondral ring leads to further collapse and shortening without obvious functional impairment of the fibrous ossification groove. Perinatal lethal OI provides insight into how a molecular disorder predominantly of Type I collagen metabolism results in pathology of numerous tissues, leading to severe skeletal dysplasia without primarily affecting chondrogenesis.
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PMID:Skeletal dysplasia in perinatal lethal osteogenesis imperfecta. A complex disorder of endochondral and intramembranous ossification. 833

Sprague-Dawley rats were exposed for 6 h daily to 0.8 ppm of ozone and 14.4 ppm of nitrogen dioxide. Approximately 7 to 10 wk after the initiation of exposure, animals began to demonstrate respiratory insufficiency and severe weight loss. About half of the rats died between Days 55 and 78 of exposure; no overt ill effects were observed in animals exposed to filtered air, to ozone alone, or to nitrogen dioxide. Biochemical findings in animals exposed to ozone and nitrogen dioxide included increased lung content of DNA, protein, collagen, and elastin, which was about 300% higher than the control values. The collagen-specific crosslink hydroxy-pyridinium, a biomarker for mature collagen in the lung, was decreased by about 40%. These results are consistent with extensive breakdown and remodeling of the lung parenchyma and its associated vasculature. Histopathologic evaluation showed severe fibrosis, alveolar collapse, honeycombing, macrophage and mast cell accumulation, vascular smooth muscle hypertrophy, and other indications of severe progressive interstitial pulmonary fibrosis and end-stage lung disease. This unique animal model of progressive pulmonary fibrosis resembles the final stages of human idiopathic pulmonary fibrosis and should facilitate studying underlying mechanisms and potential therapy of progressive pulmonary fibrosis.
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PMID:A new model of progressive pulmonary fibrosis in rats. 834 14

We analyzed the expression and distribution of collagen types IV and VI, laminin and fibronectin during the development and regression of the mesonephros in human embryos and fetuses ranging from 6 to 12 weeks of gestation by indirect immunoperoxidase methods. Type IV collagen, laminin and fibronectin were detected along the glomerular, tubular and capsular basement membranes of developing and mature nephrons. Only type IV collagen and fibronectin were found in the mesangium. Type VI collagen formed a delicate interstitial fibrillar network and a continuous basement membrane-like structure along the mesonephric nephrons. Basement membranes (GBM) of developing and mature glomeruli showed a distinct continuous staining for this collagen. The mesangial matrix was rich in type VI collagen. Mesonephric involution started during the 8th week of gestation and coincided with a moderate expansion of mesangial matrix and progressive collapse of the capillary walls, while the tubules became thinner and shorter. Staining for all extracellular matrix glycoproteins studied showed GBM wrinkling, gradual disintegration of some capillary loops and glomerulosclerosis. The sclerotic glomeruli were strongly positive for type IV collagen and less positive for type VI collagen and fibronectin. Laminin was absent. Our results indicate that collagen types IV, VI, laminin and fibronectin may be involved in the development and regression of the human mesonephros.
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PMID:Distribution of extracellular matrix glycoproteins in the human mesonephros. 852 93

The clinical features, investigation and outcome in 24 patients with pregnancy-associated osteoporosis, followed for up to 24 years from the first pregnancy are described. Symptoms occurred most often in the first pregnancy (17 patients) at a mean age of 27 years (range 21-36); the most frequent was back pain in late pregnancy or post partum (n = 18); less common was hip (n = 5) or ankle (n = 1) pain. In most, symptoms improved soon after delivery. Four subjects had pre-existing disorders known to reduce bone density (corticosteroid therapy, heparin treatment, mild osteogenesis imperfecta and previous anorexia nervosa). Radiographs showed vertebral collapse or localized osteoporosis of the hip, with MRI evidence of oedema. Forearm bone mineral density (BMD) was sometimes normal, but spinal BMD (measured by DXA) was low. Bone biopsies in eleven patients showed features compatible with osteoblast failure. Except for the patient with mild osteogenesis imperfecta, cultured dermal fibroblasts synthesized and exported normal Type I collagen. In 14 subsequent pregnancies (10 patients) there was no recurrence in ten and mild symptoms in the remainder. Excluding one patient who had repeated osteoporotic fractures and vertebral collapse, the long-term prognosis was good.
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PMID:Pregnancy-associated osteoporosis. 859 46

Coronary capillaries are extensively tethered to adjacent myocytes by collagen fibers. The influence of this tethering in the beating heart is studied by structural mechanics as applied to the specific morphology of the capillary-myocyte system. The results show considerable effects of the tethering collagen fibers on the capillary deformation, especially during systole and in the deeper myocardial layers. The tethering fibers prevent total systolic collapse, being taut during systole but partially slack during diastole, in agreement with reported observations. At the deeper wall layers, the systolic/diastolic differences in capillary cross-sectional area are predicted to be more pronounced: about 30 and 50% area reduction in arterial and venous ends, respectively, compared with 10 and 20% increase of area in the subepicardial vessels. These predictions comply well with published, experimental data. A parametric investigation shows a variable effect of the capillary-myocyte distance on the dynamics of the capillary area, while the stiffnesses of both the fibers and wall membrane, and the extent of transmural transmission of intramyocardial pressure, have both considerable quantitative effects. These effects are found to be region dependent and vary along the capillary length and from diastole to systole. The results indicate that capillary tethering to the myocardial tissue has significant effect on its mechanics. Tethering should, therefore, be considered in analyzing the dynamics of coronary flow.
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PMID:Tethering affects the mechanics of coronary capillaries. 870 86

We have used polarized light (POL) to monitor changes in the organization of the articular cartilage collagen network and matrix proteoglycans (PGs) after intra-articular injection of chymopapain (CP). POL viewing of sirius red stained sections revealed a loss of normal birefringence suggesting an apparent collapse of the collagen network following intra-articular CP. After 21 days, knees injected with 2.0 mg CP showed no return of normal birefringence, however, normal birefringence was noted in knees injected with only 0.2 mg CP. POL viewing of toluidine blue stained sections revealed a severe loss of matrix PGs followed by PG restoration in animals injected with 0.2 mg CP. The most important inference from the data is that articular cartilage can recover from enzyme-induced alterations in the spatial collapse of its fibrillar network. This is an important finding since it has often been inferred that damage to the collagen network leads invariably to progressive articular cartilage destruction.
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PMID:Alteration and recovery of the spatial orientation of the collagen network of articular cartilage in adolescent rabbits following intra-articular chymopapain injection. 890 75

Renal glomerular fibrosis was observed in a 1-year-old spayed female Japanese domestic cat that showed clinically advanced renal failure. In the glomeruli, increased homogeneous materials were stained strongly with aniline blue by Masson's trichrome and positive for anti-type III collagen antibody by immunohistochemical staining, causing mesangial sclerosis and capillary collapse. By electron microscopy, randomly arranged fibrils were observed in the expanded subendothelial and mesangial areas, and the fibrils showed periodicity characteristic of collagen fibers in longitudinal sections. These findings of glomerular lesions closely resemble those of human "collagenofibrotic glomerulonephropathy," which has recently been described as a new type of glomerulonephropathy.
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PMID:Renal glomerular fibrosis in a cat. 895 29

Electroporation provides a useful method for loading fibroblasts with fluorescent probes for the cytoskeleton, but the possible deleterious effects of this loading technique on cell motility are unknown. We have used conventional and confocal microscopy of living cells and immunohistochemistry to examine the migration and cytoskeleton of chick embryo corneal fibroblasts electroporated while cultured within collagen gels. Fibroblasts cultured in collagen (1 mg/ml) are successfully electroloaded (0.5-1.0 kVcm-1/960 microF in DMEM/F12/20 mM Hepes, pH 7.2) with dextran (4-150 kDa) and immunoglobulin, but subsequently display uncoordinated pseudopodia and hence are unable to migrate effectively in any one direction. The lack of directed movement is due to depolymerization of microtubules and/or a perinuclear collapse of vimentin filaments, seemingly caused by millimolar levels of Ca2+ ions derived from culture medium following electroporation. Fibroblasts loaded in a buffer which resembles intracellular fluid (< or = 10 microM Ca2+) maintain their cytoskeleton and continue to migrate, when returned to culture medium within 10 min. Using this novel approach, we have loaded fibroblasts migrating through extracellular matrix (ECM) with rhodamine phalloidin and monitored the behavior of the labeled actin cortex by confocal microscopy. During migration phalloidin-actin accumulates near the base of pseudopodia and at the rear of the cell where it is subsequently left behind. We conclude that electroporation is a valuable technique for loading fibroblasts to study migration within ECM, provided that the conditions used support stability of the tubulin cytoskeleton.
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PMID:Effects of electroporation on the tubulin cytoskeleton and directed migration of corneal fibroblasts cultured within collagen matrices. 895 5

Insoluble collagen has been utilized as a base material for parenteral drug carrier systems. Information on its physicochemical properties was obtained by focussing on thermoanalytical methods. On the way from the raw material to the matrices, the acidic aqueous dispersion represents an important intermediate state. DSC and FTIR revealed its complete denaturation at 43 degrees C. Dense homogeneous collagen matrices were prepared by air-drying at 25 degrees C and became denatured at 103.5 degrees C, far above normal storage temperatures. Dielectrical Thermal Analysis demonstrated transitions in the dielectrical storage and loss moduli, reflecting the dissipation of electrical energy and increased molecular mobility caused by collapse of the triple helical structure. Cross-linking of the collagen dispersion with glutaraldehyde induced no alteration in the thermoanalytical properties of dry matrices. However, in the swollen state, after incubation of the devices in phosphate buffer the transition temperature increased from 50 to 70 degrees C as cross-linking was intensified. This indicated stronger interactions between the collagen fibre structures. Dissolution tests with cytochrome c-loaded matrices showed that higher amounts of the model protein were trapped inside the matrices as more glutaraldehyde was added.
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PMID:Basic thermoanalytical studies of insoluble collagen matrices. 896 25


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