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Biomedical subjects

R Grace

Publications and source records attributed to R Grace.

At least 37 records · Page 2Linked to original sources
PubMed1987

Cytochrome a3 deficiency in human achondroplasia.

Mitochondria prepared from tissue culture cells (skin fibroblasts) from normal subjects and subjects with homozygous achondroplasia were studied to determine the concentrations of cytochromes a and a3 in the preparations. Cytochrome a3 was markedly decreased (80%) in the achondroplastic preparations with cytochrome a present in normal amounts. Determination of total heme a (as the pyridine hemochromogen) in the normal and achondroplastic preparations demonstrated that the observed decrease in concentration of cytochrome a3 in the achondroplastic preparations was due to an absence of cytochrome a3 and not to a change in its absorbancy (extinction coefficient). The decreased concentrations of cytochrome a3 in the achondroplastic cells may decrease the reactivity or affinity of the mitochondrial oxidative systems for oxygen and result in the phenotypic expression of the disease.

Achondroplasia↗
PubMed1986

Studies of human achondroplasia: oxidative metabolism in tissue culture cells.

Mitochondria prepared from the first growth of cells (fibroblasts) from skin biopsies from homozygous (but not heterozygous) achondroplastic human subjects were unable to carry out oxidative phosphorylation. However, successive crops of cells gained the ability to phosphorylate with normal P:O ratios with pyruvate-malate and succinate as substrates. Concentrations of cytochromes a + a3 were markedly and significantly lower in homogenates of homozygous achondroplastic tissue culture cells than in homogenates of normal cells. Levels of cytochromes a + a3 in the heterozygous achondroplastic cells were intermediate between the levels in normal cells and the homozygous achondroplastic cells. Activities of the mitochondrial oxidative systems (NADH, succinic and cytochrome oxidases) were not significantly lower in the achondroplastic cell preparations than in normal cell preparations under standard assay conditions (saturation levels of oxygen).

Achondroplasia↗
PubMed1985

Maternal barbiturate administration and offspring response to shock.

Gravid Sprague-Dawley-derived rats were injected SC twice daily with either 20 or 40 mg/kg pentobarbital sodium (PT), sodium phenobarbital (PH), or the same volume of the saline vehicle on days 9-21 of pregnancy. Pair-feeding was employed. Vital, developmental, and activity measures were obtained on the neonates and locomotor activity was measured from 3-10 months of age. Avoidance was measured sequentially in a shuttle box, and in an operant chamber beginning at 3 months of age. The PH-80 dams gained less weight over the gestational period, and PH-80 and PH-40 offspring had more neonatal deaths. These male offspring were hyperactive at maturity, and PH-80 rats were initially slower to escape experimenter-initiated shock. PT exposure caused transient neonatal and juvenile hyperactivity. PT rats performed more poorly on both the conditioned avoidance and Sidman shock schedules, and had significantly lower brain: body weight ratios at 1 year of age. All four drug groups outperformed the saline offspring on subject-initiated shock schedules (punishment). Sex of offspring was determined on postnatal day 4 and the sex ratio was shifted towards male births with both drugs relative to controls.

Animals↗
PubMed1985

Pyridine nucleotide transhydrogenations in yeast.

Though previously described as very low or absent in yeast, we find significant pyridine nucleotide transhydrogenation (NADPH + acetyl pyridine-NAD+----NADP+ + acetyl pyridine-NADH) activity in yeast extracts when assayed at pH 8-9, and describe here the subcellular distribution and separation of the various molecular forms contributing to the total activity in two yeast species. Gentle subcellular fractionation reveals transhydrogenase activity only in the cytosolic fraction of both Saccharomyces cerevisiae and Candida utilis while intact mitochondria and microsomes are without activity. On sucrose gradient centrifugation, this soluble cytosolic activity proves to be primarily in a high-molecular-weight (greater than 10(6)) band which has salmon-colored fluorescence on uv illumination. Sonication of the particulate subcellular fractions solubilizes substantial transhydrogenase activity from mitochondria of C. utilis (but not from S. cerevisiae) which on sucrose gradients consists of both high (greater than 10(6))- and low-molecular-weight active fractions, each with yellow-green fluorescence. Ammonium sulfate fractionation and sucrose gradient centrifugation of protein solubilized from whole yeast of both species by vigorous homogenization with glass beads confirms the presence and fluorescence of these various molecular weight forms. The relationship of these activities to other enzymatic activities (especially the mitochondrial external NADH dehydrogenase) is discussed.

Ammonium Sulfate↗
PubMed1985

Iron deficiency in the rat: effects on energy metabolism in brown adipose tissue.

Mitochondria were prepared from the brown adipose tissue of control rats and animals made iron deficient by means of a low iron diet. The specific activities of the mitochondrial electron transport system (NADH, succinate and alpha-glycerophosphate oxidase activities) were markedly and significantly reduced in preparations of brown adipose tissue from the iron-deficient rats as compared with preparations from the control animals. In contrast, concentrations of the cytochrome pigments a + a3, and c + c1 were normal and cytochrome b was slightly reduced (18%) in the mitochondrial preparations from the iron-deficient animals. Treatment of the iron-deficient animals with triiodothyronine significantly increased the amount of brown fat present per kilogram of body weight in both control and iron-deficient rats, but did not significantly affect the specific activities of the mitochondrial electron transport system.

Adipose Tissue, Brown↗
PubMed1984

Iron deficiency in the rat: effects on oxidative metabolism in distinct types of skeletal muscle.

Studies performed on iron-deficient and control rats demonstrated that oxidative energy production (phosphorylation) by mitochondria from iron-deficient red and intermediate skeletal muscles was greatly reduced with pyruvate-malate, succinate, and alpha-glycerophosphate as substrates. Although phosphorylation was also decreased in iron-deficient white skeletal muscle with succinate and pyruvate-malate as substrates, no change was found with alpha-glycerophosphate as substrate.

Anemia, Hypochromic↗
PubMed1983

Iron deficiency in the rat: biochemical studies of fetal metabolism.

The effects of dietary-induced iron deficiency on fetal and maternal metabolism were studied in the rat. Concentrations of phenylalanine, but not tyrosine, were significantly elevated in plasma from iron-deficient maternal and fetal rats at day 20 of gestation with individual fetal plasma levels of phenylalanine as high as 10 mg per 100 ml. Concentrations of total 5-hydroxyindole compounds were significantly decreased in brain tissue from iron-deficient fetuses (day 20 of gestation), suggesting that synthesis of the compounds may be inhibited by iron deficiency. Mitochondrial NADH oxidase activity was markedly decreased (60%) in homogenates of fetuses at day 14 of gestation and may account for the high fetal resorption rate and small fetal size observed in the rat in iron deficiency.

Animals↗