Copper Trafficking and Extracellular Superoxide Dismutase Activity

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Copper trafficking and extracellular superoxide dismutase activity: kinky hair, kinky vessels.

In 1962, Menkes et al1 first described a pediatric disorder characterized by hypopigmented brittle hair, doughy skin, connective tissue fragility, failure to thrive, progressive neurological damage, and various defects of the arterial wall. This was termed “kinky hair syndrome” and later was found to be associated with a disturbance in copper metabolism due to mutations in the ATP7A gene. The A...

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Extracellular Superoxide Dismutase

Extracellular-superoxide dismutase is a tetrameric copper-containing glycoprotein that previously has been demonstrated to be the major superoxide dismutase of human extracellular fluids. The occurrence of this enzyme in various human tissues that were removed from two accidental death victims and in 19 different human cultured cell lines was determined. All investigated tissues were found to c...

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Gene transfer of extracellular superoxide dismutase increases superoxide dismutase activity in cerebrospinal fluid.

BACKGROUND AND PURPOSE Copper-zinc superoxide dismutase (CuZnSOD) is expressed intracellularly, while extracellular SOD (EC-SOD) is released from cells. The purpose of this study was to determine whether gene transfer of CuZnSOD increases SOD activity predominantly in tissues, and gene transfer of EC-SOD increases SOD activity in cerebrospinal fluid (CSF). We also determined whether heparin or ...

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Prenatal hypoxia decreases lung extracellular superoxide dismutase expression and activity.

Extracellular superoxide dismutase (EC-SOD), which scavenges extracellular superoxide (O.), is highly regulated in the developing lung. In the prenatal rabbit, EC-SOD is predominantly intracellular and inactive, and postnatally, active EC-SOD is secreted. We hypothesized that prenatal hypoxia would delay the normal postnatal secretion of active EC-SOD in the lung. Pregnant New Zealand White rab...

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Inactive extracellular superoxide dismutase disrupts secretion and function of active extracellular superoxide dismutase.

Extracellular superoxide dismutase (EC-SOD) is an antioxidant enzyme that protects cells and tissues from extracellular damage by eliminating superoxide anion radicals produced during metabolism. Two different forms of EC-SOD exist, and their different enzyme activities are a result of different disulfide bond patterns. Although only two folding variants have been discovered so far, five foldin...

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ژورنال

عنوان ژورنال: Hypertension

سال: 2008

ISSN: 0194-911X,1524-4563

DOI: 10.1161/hypertensionaha.108.117770