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[Neuroradiologic findings of glutaric aciduria type I].

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Glutaric aciduria type I is a rare disorder of organic acid metabolism caused by deficiency of glutaryl-CoA dehydrogenase, a mitochondrial enzyme. Improper degeneration of amino acids: tryptophan, lysine, and hydroxylysine, results in increased levels of glutaric acid, which typically becomes

Type II collagen-induced arthritis. A morphologic and biochemical study of articular cartilage.

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Articular cartilage was obtained from type II collagen-induced arthritic rat joints. Transmission electron microscopy showed a gradual degeneration of chondrocytes, disorganization of the collagenous extracellular matrix, and formation of microscars. Biochemical analyses indicated that type II

Immunolocalization of glutaryl-CoA dehydrogenase (GCDH) in adult and embryonic rat brain and peripheral tissues.

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Glutaryl-CoA dehydrogenase (GCDH) is a mitochondrial enzyme that is involved in the degradation of tryptophan, lysine and hydroxylysine. Deficient enzyme activity leads to glutaric aciduria type-I (GA-I). This neurometabolic disease usually manifests with acute encephalopathic crises and striatal

Long Lasting High Lysine Diet Aggravates White Matter Injury in Glutaryl-CoA Dehydrogenase Deficient (Gcdh-/-) Mice.

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Glutaric acidemia type I (GA-I) is a neurometabolic disease caused by deficient activity of glutaryl-CoA dehydrogenase (GCDH) that results in accumulation of metabolites derived from lysine (Lys), hydroxylysine, and tryptophan catabolism. GA-I patients typically develop encephalopatic crises with

[Glutaric aciduria type 1: an example of the importance of early detection of so-called cerebral organic aciduria].

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Glutaric aciduria type 1 (GA 1) is a preventable cause of acute brain damage in early childhood, leading to a severe dystonic-dyskinetic disorder. Typically between 6 and 18 months of age, a non-specific illness such as respiratory or gastrointestinal infection or immunization leads to

Neocartilage integration in temporomandibular joint discs: physical and enzymatic methods.

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Integration of engineered musculoskeletal tissues with adjacent native tissues presents a significant challenge to the field. Specifically, the avascularity and low cellularity of cartilage elicit the need for additional efforts in improving integration of neocartilage within native cartilage.

Putative role of lysyl hydroxylation and pyridinoline cross-linking during adolescence in the occurrence of osteoarthritis at old age.

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OBJECTIVE The collagen network in human articular cartilage experiences a large number of stress cycles during life as it shows hardly any turnover after adolescence. We hypothesized that, to withstand fatigue failure, the physical condition of the collagen network laid down at adolescence is of

Biochemical and ultrastructural changes in rabbit sclera after treatment with 7-methylxanthine, theobromine, acetazolamide, or L-ornithine.

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OBJECTIVE To examine a possible effect of 7-methylxanthine, theobromine, acetazolamide, or L-ornithine on the ultrastructure and biochemical composition of rabbit sclera. METHODS Groups of pigmented rabbits, six in each group, were dosed during 10 weeks with one of the substances under

The type of collagen made by the articular cartilage in joints of dogs with degenerative joint disease.

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The collagen in the degenerated articular cartilage from dysplastic canine hip joints was characterized as to type by examining cartilage directly and also after labeling cartilage slices with 14C-proline in vitro. Collagen analysis was by sodium dodecylsulfate-polyacrylamide gel electrophoresis of

[Collagen metabolism of skeletal muscles following injuries of the peripheral nerves].

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In order to elucidate the mechanisms of fibrosis and atrophy of skeletal muscles after peripheral nerve injuries, the biochemical changes in collagen from gastrocunemius muscles of adult albino rabbits have been analyzed after denervation of the proximal portion of sciatic nerve. The wet weight of

Early exercise advances the maturation of glycosaminoglycans and collagen in the extracellular matrix of articular cartilage in the horse.

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BACKGROUND Training at a very young age may influence the characteristics of the collagen network of articular cartilage extracellular matrix (ECM) in horses. OBJECTIVE To investigate whether increasing workload of foals results in significant changes in the biochemical composition of articular

Biochemistry and bioenergetics of glutaryl-CoA dehydrogenase deficiency.

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Glutaryl-CoA dehydrogenase (GCDH) is a central enzyme in the catabolic pathway of L-tryptophan, L-lysine, and L-hydroxylysine which catalyses the oxidative decarboxylation of glutaryl-CoA to crotonyl-CoA and CO2. Glutaryl-CoA dehydrogenase deficiency (GDD) is an autosomal recessive disease

The role of collagen in the quality and processing of fish.

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Collagen in the muscles of fish constitutes the main component of the connective tissue membranes joining individual myotomes and is responsible for the integrity of the fillets. The content of collagen in fish muscles is from about 0.2 to 1.4% and in squid mantel about 2.6%. Fish and invertebrata

Changes in the content and composition of collagen in the glaucomatous eye--basis for a new hypothesis for the genesis of chronic open angle glaucoma--a preliminary report.

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The pressure theory is still predominant in explaining the pathophysiology of the chronic open angle glaucoma. An insufficient drainage system resulting in an increased intraocular pressure is the basis for this theory. The pressure will exert an effect upon the optic disc which either directly on

Characterization of bone diagenesis by histology in forensic contexts: a human taphonomic study.

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The diagenesis of a bone in the postmortem period causes an identifiable deterioration in histology. This degradation is characterized by a collagenous alteration, which can be observed very early. In order to develop a method for determining a postmortem interval for medico-legal use, two ribs

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