Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UNIPROT:Q86TM3 (cage)
 29,987 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Histopathologic evidence offered by a case of Morquio's disease and observed in the hearing organ showed widespread collagen damage, possibly initiated by the general hemorrhageous tendency, while the family history bore evidence of a genetic source. The clinical manifestations comprise striking dwarfism, beaking of the lumbar spine, sternal bulging, flaring of the rib cage, falt vertebrae, marked osteoporosis, prominent joints, semi-crouching stance, short nose. Roentgenograms showed retarded ossification.
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PMID:Morquio's disease and the hearing organ. 41 82

The aim of this study was to investigate the propensity to develop cardiac arrhythmias during an acute period of ischemia between normal and hypertrophied (by means of a swimming training regimen) rat hearts. We used the coronary artery ligation in vivo technique which induced the occurrence of cardiac arrhythmias in rats that was followed by the determination of the occluded zone size. This study was coupled to an in vitro study using a two-compartment tissue bath in which half of the ventricular preparation was exposed to normal conditions and the other to ischemic conditions (low pH, hypoxia, and hyperkalemia). We also measured the collagen content and the DNA/protein ratio of the hearts. Twenty-eight male Wistar rats submitted to an eight-week swimming training (SWT) and twenty-eight cage-confined matched rats were used for the studies. SWT resulted in a 14% decrease in mean body weight and an 8% increase in absolute heart weight. We also observed a resting bradycardia in the trained animals and blood pressure remained unchanged between the two groups. Collagen content was unchanged and DNA/protein ratio was lower in the left ventricle of trained animals. During a 30-min period of coronary artery ligation, SWT rats demonstrated fewer ischemia-induced arrhythmias as compared to controls. The size of the zone affected by the vasal occlusion was lower in trained animals. Electrophysiological data recorded in the two-compartment bath showed a marked prolongation of action potential duration and refractory period in the SWT rat hearts. During the 15-min period of in vitro ischemia there was a global alteration of all electrophysiological parameters which did not differ between the two groups. Our data support the hypothesis that resting bradycardia and decrease in ischemic zone size may be involved in the arrhythmogenic protection observed in hypertrophied hearts of swimming rats after an acute ligation of the left coronary artery. Our results also indicate that cardiac hypertrophy, as defined by quantitative changes in cardiac mass or by the electrophysiological alterations that are related to its development, is not necessarily associated with an increased risk for the occurrence of arrhythmias.
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PMID:Decreased susceptibility to arrhythmias in hypertrophied hearts of physically trained rats. 141 4

This study was carried out to test the hypothesis that improvements in ligament scar mechanical behavior during healing may be related, in part, to increases in collagen fibril diameters. Forty-eight adult female New Zealand White rabbits had standardized midsubstance gap injuries created in their right medial collateral ligaments (MCLs) and were allowed normal cage activity until sacrifice in groups of 12 at 3, 6, 14 or 40 weeks post-injury. Eight animals in each group had both MCLs tested biomechanically while 4 animals had transmission EM investigation of midsubstance collagen fibril diameters by a standardized protocol. Results of mechanical tests showed a three- to fourfold increase in scar strength and stiffness over the intervals of healing studied while there was no change in collagen mean fibril minimum diameters. These results demonstrate no correlation between material or structural properties of scar and collagen fibril diameters in this model of healing and suggest that other mechanisms for scar mechanical improvement under these conditions must be investigated.
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PMID:Collagen fibril diameters in the healing adult rabbit medial collateral ligament. 157 25

The campomelic syndrome is a skeletal dysplasia with a characteristic pattern of deformity involving the proximal and distal extremities, pelvic and shoulder girdles, thoracic cage and palate. Respiratory compromise often leads to death in early infancy. Etiology has not been determined although evidence suggests genetic heterogeneity in patients with campomelia. Cytogenetic analysis in the past have revealed an unexpectedly high incidence of a 46, XY karyotype in phenotypic females. We report here on a patient with a typical case of campomelic dysplasia in whom a de novo paracentric inversion of chromosome 17q was identified. Review of the genetic map of the inverted region identified potential "structural" genes including the Hox-2-homeobox gene and the collagen gene, COLIA1, which may be involved in the pathogenesis of campomelic syndrome.
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PMID:A chromosome 17q de novo paracentric inversion in a patient with campomelic dysplasia; case report and etiologic hypothesis. 167 32

This study investigated the resurfacing of full-thickness articular defects in the adult rabbit medial femoral condyle using a rib perichondrial graft. The graft was secured to a 4-mm-diameter bone core removed from the femoral condyle. Two postoperative treatment protocols were studied: one group had ad libitum cage activity (CAGE; n = 95) and the other group received two weeks of passive motion (PM; n = 73; eight hours per day, five days per week) followed by cage activity. Animals in both groups were killed at six, 12, 26, and 52 weeks. Repair tissue resembling hyaline cartilage formed in a majority of animals at all time periods and in both postoperative treatment groups. The overall success rates in which repair tissue formed were 58% in the CAGE group and 56% in the PM group. However, over time, a maturation of the neocartilage into nearly normal hyaline articular cartilage was noted with the percentage of Type II collagen increasing from 55% at six weeks to 82% at one year. The complex shear modulus of the repair tissue for both groups became similar to normal cartilage with increased healing time. There was no statistical difference in shear moduli between the two treatment modalities. These results show that cartilage repair tissue derived from rib perichondrium could mature into hyaline articular cartilage over time and would not degrade by one year after repair.
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PMID:Rib perichondrial autografts in full-thickness articular cartilage defects in rabbits. 173 24

The effects of motion and increased levels of stress on the biomechanical, biochemical, and morphological properties of healing medial collateral ligaments were assessed in a rabbit model. In one group, the medial collateral ligament of the left hindlimb was transected and allowed to heal with cage activity for either 6 or 12 weeks. In another group, the transected ligaments were permitted to heal for 4 weeks and then were placed under increased stress by inserting a stainless steel pin perpendicularly underneath the healing medial collateral ligament. The animals were allowed cage activity for an additional 2 or 8 weeks. The varus-valgus joint laxity and the stress-strain properties of the medical collateral ligament substance were obtained. Further, the quantity of total collagen, amount and ratio of the collagen cross-links, dihydroxylysinonorleucine and hydroxylysinonorleucine, and the histologic appearance of the healing medical collateral ligaments were evaluated for all groups. At 6 weeks, knees with a transected medial collateral ligament were twice as lax as the controls. However, joints with the stainless steel tension pin had varus-valgus values approximately 1.5 times those of the controls. At 12 weeks, joints with increased stress were not statistically different from the controls. The group that had healing with increased stress for 12 weeks produced the highest stress for a given strain compared to any other group. Also, the total collagen levels and the ratio of dihydroxylysinonorleucine/hydroxylysinonorleucine were the closest to normal of any transected group. Finally, qualitative histologic improvements were seen, including a more longitudinal arrangement of collagen fibers and decreased cellularity.
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PMID:The effects of increased tension on healing medical collateral ligaments. 189 48

The aim of this study was to test the hypothesis that deficiency of vitamin B6 would produce morphological characteristics of osteochondral lathyrism. To accomplish this goal, morphological characteristics of chick cartilage in which lathyrism was produced by two separate dietary regimens was compared to morphological changes encountered in vitamin B6 deficiency. Vitamin B6 deficiency should reduce activity of lysyloxidase needed for producing intermolecular cross-links. The question to be addressed was: would this latter deficiency impair collagen morphological features and secondarily other structures indirectly by reducing collagen molecular assembly? Failure of cross-linking of collagen in the positive controls was related to a lack of functional aldehyde cross-link intermediates which are blocked by homocysteine and aminoacetonitrile. Day-old-male Lohmann chicks were fed adequate (6 mg/kg) or vitamin B6-deficient diets. Cross-link defects were induced by homocysteine-rich diets (0.6% w/w) or a diet containing aminoacetonitrile (0.1% w/w). Animals were sacrificed at 6 weeks of age and Ossa tarsalia articular cartilage specimens, as well as the proximal end of tarsometatarsus were dissected from the tibial metatarsal joint, a major weight-bearing site. Light microscopic observations revealed reduction of subarticular trabecular bone formation, concurrent with overexpansion of the hypertrophic cell zone. Ultrastructural electron microscopy observation of articular fibro-cartilage indicated significant thickening of collagen fibers in vitamin B6 deficient birds, as well as the positive controls in comparison to that of cage-matched control birds. It was concluded that vitamin B6 deficient cross-linking may be responsible for the observed delay in bone development and aforementioned cartilage histological alterations.
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PMID:Morphological abnormalities in vitamin B6 deficient tarsometatarsal chick cartilage. 208 Apr 30

The activities of prolyl 4-hydroxylase (PH) and galactosylhydroxylysyl glucosyl-transferase (GGT), and the concentration of hydroxyproline (Hyp) were measured in the Achilles and tibialis anterior tendons of rats after periods of 0, 3, 7 and 14 days remobilization following a 7-day cast immobilization. PH, GGT and Hyp were also observed in the soleus and tibialis anterior muscles and the Achilles tendon 2, 5 and 10 days after a single bout of exhaustive exercise. During immobilization, the specific activity of PH decreased by 23% (P less than 0.05) and 54% (P less than 0.01) in the Achilles and tibialis anterior tendons. A less pronounced decrease was observed in GGT activity. During the remobilization experiment the specific activity of PH increased gradually, whereas GGT activity increased rapidly. The GGT activity was 61-66% (P less than 0.01) above the control value in the Achilles and tibialis anterior tendons after 3 days, decreasing to the control level after 14 days. Hyp concentration did not change significantly. In the exercise experiment, the specific activity of PH in the soleus muscle increased by 64% (P less than 0.05) 2 days after the prolonged exercise and then decreased gradually to the control level. There were no significant changes in PH or GGT activity in the tibialis anterior muscle. The specific activity of PH and GGT and Hyp concentration remained unchanged in the Achilles tendon. The results suggest that, in the tendon, restoring normal cage activity after immobilization results in acceleration of collagen synthesis. The results suggest also that collagen synthesis of the tendon is insensitive, but that of the soleus muscle is sensitive, to an acute increase in physical activity.
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PMID:The effects of remobilization and exercise on collagen biosynthesis in rat tendon. 216 21

The purpose of this study was to assess, morphologically and biochemically, the effect of hyaluronan (HA) on the early repair process of the anterior cruciate ligament (ACL). Following partial bilateral laceration in the midsubstance of the cruciate ligament, a single dose of HA (MW of 3.6 x 10(6] was injected in one knee and saline in the contralateral knee. Postsurgery, the rabbits were allowed normal (nonimmobilized) cage activity, and were killed after 4 (n = 11) and 12 (n = 10) weeks. The ligaments were evaluated by gross morphology and graded according to the degree of repair. We used grades 1,2, and 3 for uncovered, partially covered, and totally covered lacerations, respectively. Five of the HA-treated ligaments at each time studied were completely covered, compared to 0 at 4 weeks, and 1 at 12 weeks in the saline group. Paired evaluations of the lacerated ACLs showed that the HA-treated ligaments received a healing grade higher than the ligaments exposed to saline in 14 of the 21 animals. In the remaining animals, there was no difference between the sides. The repaired tissue of the ACLs was also examined by light and electron microscopy. When compared qualitatively with saline controls, HA-treated ligaments exhibited a more pronounced repair, with an increased angiogenesis and less inflammatory response. Biochemical analysis demonstrated a mean higher value of type III collagen in the HA-treated injured ACL than in saline-treated injured ACL (13.4 +/- 1.1% and 11.0 +/- 0.8%, respectively). This increased synthesis of type III collagen in the HA-treated injured ACL was statistically higher (p less than 0.05) when compared to the saline-treated injured ACL.
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PMID:The early effect of high molecular weight hyaluronan (hyaluronic acid) on anterior cruciate ligament healing: an experimental study in rabbits. 232 60

Exaggerated acute and late effects were observed in three of four women with pre-existing collagen vascular disease (CVD) within 2 years after definitive megavoltage radiation therapy for breast carcinoma. Five women with breast carcinoma, who developed CVD 3 months to 10 years after radiation therapy, had no complications. An abnormally severe reaction was observed during treatment of one patient with discoid lupus. The patient developed moist desquamation that persisted for a month, requiring early termination of treatment. One year after treatment, the patient developed paresthesias in the ipsilateral arm. A planned reduction of the prescribed dose in a second patient with progressive systemic sclerosis did not prevent intense erythema at the end of treatment, followed 14 months later by chest wall necrosis, which eventually required multiple surgeries including chest wall resections. The third patient, who had systemic lupus erythematosis, developed necrosis 2 years after treatment, which progressed over 12 years to osteoradionecrosis of the clavicle, sternum and rib cage. Multiple surgeries to repair the defect were complicated by flap necrosis and pleurocutaneous fistulas. The fourth patient died 6 months after radiotherapy without apparent sequelae. None of the patients had evidence of recurrent carcinoma. A history of collagen vascular disease appears to be a contraindication to breast conservation or for elective irradiation for breast cancer.
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PMID:Consequences of breast irradiation in patients with pre-existing collagen vascular diseases. 277 73


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