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Disease of the ornithine-proline pathway: delta 1-pyrroline-5-carboxylate reductase deficiency in the retina of retinal degeneration mice.

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In bovine ocular tissues, cornea and retina-including choroid provide the intracellular proline synthetic pathway from ornithine, but not from glutamate. In C3H retinal degeneration mice, P5C reductase activity in the retina and choroid was decreased to about one-third that of CRJ control mice in

Disease of ornithine-proline pathway: a delta 1-pyrroline-5-carboxylate reductase deficiency in the retina or retinal degeneration mice.

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Cloning, characterization, and expression of cDNAs encoding human delta 1-pyrroline-5-carboxylate dehydrogenase.

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Delta 1-pyrroline-5-carboxylate dehydrogenase (P5CDh; EC 1.5.1.12), a mitochondrial matrix NAD(+)-dependent dehydrogenase, catalyzes the second step of the proline degradation pathway. Deficiency of this enzyme is associated with type II hyperprolinemia (HPII), an autosomal recessive disorder

Pyrroline-5-carboxylate synthase senses cellular stress and modulates metabolism by regulating mitochondrial respiration

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Pyrroline-5-carboxylate synthase (P5CS) catalyzes the synthesis of pyrroline-5-carboxylate (P5C), a key precursor for the synthesis of proline and ornithine. P5CS malfunction leads to multiple human diseases; however, the molecular mechanism underlying these diseases is unknown. We found that P5CS

Regulation of proline biosynthesis: the inhibition of pyrroline-5-carboxylate synthase activity by ornithine.

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Mammalian cells have the capacity for proline biosynthesis from ornithine or glutamic acid. Using a radioisotopic assay, we have studied the regulation by ornithine of delta 1-pyrroline-5-carboxylate synthase, the enzyme that catalyzes the first step of proline biosynthesis from glutamic acid. In

Using MRI to measure in vivo free radical production and perfusion dynamics in a mouse model of elevated oxidative stress and neurogenic atrophy.

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Mitochondrial dysfunction, reactive oxygen species (ROS) and oxidative damage have been implicated to play a causative role in age-related skeletal muscle atrophy and weakness (i.e. sarcopenia). Mice lacking the superoxide scavenger CuZnSOD (Sod1^-/-) exhibit high levels of oxygen-derived

Hematogenous photosensitization. A mechanism for the development of age-related macular degeneration.

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Age-related macular degeneration (ARMD) is one of the leading causes of severe visual loss in the United States. Numerous risk factors have been investigated, but the pathogenesis of ARMD has remained elusive. The authors propose that ARMD develops as a direct result of photosensitization of the

Mitochondrial dysfunction in SOD1G93A-bearing astrocytes promotes motor neuron degeneration: prevention by mitochondrial-targeted antioxidants.

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Mitochondrial dysfunction and oxidative stress contribute to motor neuron degeneration in amyotrophic lateral sclerosis (ALS). Recent reports indicate that astrocytes expressing the mutations of superoxide dismutase-1 (SOD1) may contribute to motor neuron injury in ALS. Here, we provide evidence

Graphene Quantum Dots Protect against Copper Redox-Mediated Free Radical Generation and Cardiac Cell Injury.

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In this work, we investigated the effects of graphene quantum dots (GQDs) on copper redox-mediated free radical generation and cell injury. Using electron paramagnetic resonance (EPR) spectrometry in conjunction with 5,5-dimethyl-1-pyrroline N-oxide (DMPO) as a spin trap, we found that GQDs at a

Mass screening of urea cycle diseases: a new mass screening method of hyperornithinemia by using two coupling enzymes.

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We devised a new microfluorometric mass screening method for determining ornithine in only one blood disc of 3 mm in diameter (blood of 2-3 microliters) by use of two coupling enzymes, ornithine aminotransferase and delta'-pyrroline-5-carboxylate reductase. We could apply this method to some cases

Key role of cysteine residues and sulfenic acids in thermal- and H2O2-mediated modification of β-lactoglobulin.

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Oxidation results in protein deterioration in mammals, plants, foodstuffs and pharmaceuticals, via changes in amino acid composition, fragmentation, aggregation, solubility, hydrophobicity, conformation, function and susceptibility to digestion. This study investigated whether and how individual or

Prevention of ornithine cytotoxicity by proline in human retinal pigment epithelial cells.

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OBJECTIVE To investigate the relationship between ornithine-delta-aminotransferase (OAT) deficiency and ornithine accumulation and the specific degeneration of retinal pigment epithelial (RPE) cells in gyrate atrophy. METHODS Human RPE cells, human hepatoma cells, and human fibroblast cells were

Effect of cold storage and packaging material on the major aroma components of sweet cream butter.

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The major aroma compounds of commercial sweet cream AA butter quarters were analyzed by GC-olfactometry and GC-MS combined with dynamic headspace analysis (DHA) and solvent-assisted flavor evaporation (SAFE). In addition, the effect of long-term storage (0, 6, and 12 months) and type of wrapping

Hydroxyl free radical reactions with amino acids and proteins studied by electron spin resonance spectroscopy and spin-trapping.

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It has recently been shown that Fe(I) complexes of ADP or ATP generate OH radicals with H2O2 in a Fenton-type reaction. The OH radicals can be detected by using 5,5-dimethyl-1-pyrroline-N-oxide (DMPO) as a spin trap in electron spin resonance spectroscopy. All the biologically occurring amino acids,

NTP Technical Report on the metabolism, toxicity and predicted carcinogenicity of diazoaminobenzene (CAS No. 136-35-6).

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Diazoaminobenzene is used as an intermediate, complexing agent, and polymer additive. It is also an impurity in certain color additives used in cosmetics, food products, and pharmaceuticals. Diazoaminobenzene was selected for metabolism and toxicity studies based on the potential for worker exposure

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