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aortic valve stenosis/l tyrosine
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Black Aortic Valve Stenosis Reveals a Rare Disorder of Tyrosine Metabolism.
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Soluble FMS-Like Tyrosine Kinase-1 Is a Circulating Biomarker Associated With Calcific Aortic Stenosis.
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Aortic stenosis in cardiovascular ochronosis.
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Alkaptonuria (endogenous ochronosis) is a rare metabolic disorder caused by a deficiency of homogentisic acid oxidase, an enzyme responsible for the metabolic degradation of tyrosine. Patients with alkaptonuria commonly present with joint pain owing to degenerative arthritis. Other affected patients
Alkaptonuria-associated aortic stenosis.
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Alkaptonuria is an autosomal recessive disorder of tyrosine metabolism, which results in accumulation of unmetabolized homogentisic acid and its oxidized product in various tissues, including the heart. Cardiovascular involvement is a rare but serious complication of the disease. We present two
Choice of valve prosthesis in a rare clinical condition: aortic stenosis due to alkaptonuria.
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Alkaptonuria is a rare inherited disorder of tyrosine metabolism, which results in deposition of homogentisic acid in the connective tissues. The accumulation of homogentisic acid in connective tissue causes the syndrome known as ochronosis, which is typically manifested by skin pigmentation,
Aortic valve stenosis in alkaptonuric ochronosis.
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Alkaptonuria is a rare genetic disorder of tyrosine metabolism in which a bluish pigment accumulates in the connective tissues throughout the body, and causes degenerative changes. The most common clinical manifestation of ochronosis is arthropathy. Heart valves may also be affected, though cardiac
Aortic stenosis in endogenous ochronosis.
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Endogenous ochronosis, a rare inherited disease of tyrosine metabolism, is caused by a deficiency of the enzyme, homogentisic acid oxidase, and may lead to cardiovascular involvement seen most frequently as aortic valve stenosis. We report the case of a patient with generalized ochronosis who
Protein and 28S ribosomal RNA fractional turnover rates in the rat heart after abdominal aortic stenosis.
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The rate of synthesis of myocardial proteins and ribosomal ribonucleic acid (rRNA) was measured during the development of cardiac hypertrophy in rats using a continuous intracardiac infusion of 14C-tyrosine and 3H-uridine in unanaesthetised animals. Cardiac overload was induced by abdominal aortic
Alkaptonuric aortic stenosis: a case report.
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Alkaptonuria is a rare disease of phenylalanine, aromatic amino acids, and tyrosine metabolism. Because of a genetic deficiency of the enzyme homogentisic acid oxidase, an accumulation of homogentisic acid causes ochronotic pigment deposition. The most common clinical manifestations are arthropathy,
Aortic stenosis and vascular calcifications in alkaptonuria.
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Alkaptonuria is a rare metabolic disorder of tyrosine catabolism in which homogentisic acid (HGA) accumulates and is deposited throughout the spine, large joints, cardiovascular system, and various tissues throughout the body. In the cardiovascular system, pigment deposition has been described in
Increased Protein Tyrosine Phosphatase 1B (PTP1B) Activity and Cardiac Insulin Resistance Precede Mitochondrial and Contractile Dysfunction in Pressure-Overloaded Hearts.
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BACKGROUND
Insulin resistance in diabetes mellitus has been associated with mitochondrial dysfunction. Defects at the level of mitochondria are also characteristic of heart failure. We assessed changes in cardiac insulin response and mitochondrial function in a model of pressure overload-induced
Inflammation Drives Retraction, Stiffening, and Nodule Formation via Cytoskeletal Machinery in a Three-Dimensional Culture Model of Aortic Stenosis.
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In calcific aortic valve disease, the valve cusps undergo retraction, stiffening, and nodular calcification. The inflammatory cytokine, tumor necrosis factor (TNF)-α, contributes to valve disease progression; however, the mechanisms of its actions on cusp retraction and stiffening are unclear. We
Activation of the AKT Pathway in the Ascending Aorta With Bicuspid Aortic Valve.
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BACKGROUND
Dilatation of the ascending aorta affects those patients with bicuspid aortic valve (BAV), even after valvular surgery, possibly due to tissue fragility. The goal of the study was the molecular characterization of aorta with BAV compared to that with normal tricuspid aortic valve (TAV).
Aortic valve ochronosis: a rare manifestation of alkaptonuria.
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Alkaptonuric ochronosis is a heritable disorder of tyrosine metabolism, with various systemic abnormalities related to pigment deposition and degeneration of collagen and other tissues, including the heart and aorta. A 65-year-old woman with alkaptonuric ochronosis and a history of four joint
[Total and contractile protein synthesis during experimental cardiac hypertrophy].
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The fractional turnover rate of the total protein extracted from cardiac rat ventricles has been measured by the continuous infusion flow technique in unanesthesized animals using 3H-lysine as a marker. This turnover rate approximated 12 p. 100 per day, doubled after aortic stenosis or aortic
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