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Biomedical subjects

L Ose

Publications and source records attributed to L Ose.

At least 127 records · Page 7Linked to original sources
PubMed1983

Autosomal dominant partial lipodystrophy associated with Rieger anomaly, short stature, and insulinopenic diabetes.

We describe the clinical findings and natural history of an autosomal dominant form of partial lipodystrophy found in four affected individuals from three generations in the same family. The lipodystrophy was present from infancy/early childhood, involved primarily the face and local areas on the buttocks, and was nonprogressive. Affected individuals also had the Rieger anomaly, midface hypoplasia, short stature, retarded bone age, and hypotrichosis. An affected woman developed insulinopenic diabetes mellitus at 39 yr and another had glucose intolerance at 55 yr.

Adult↗
PubMed1981

The binding of high density lipoproteins to isolated rat hepatocytes.

The association of 125I-labelled rat high density lipoproteins (125-I-HDL) to suspended rat hepatocytes was studied at 4 degrees C. 125 I-HDL associated to isolated hepatocytes by two processes-one of high and one of low affinity. The cell-association of 125I-HDL exhibited saturation kinetics and was inhibited to varying degrees with both rat and human lipoproteins such as VLDL, LDL and HDL but not by lipoprotein deficient serum or asialo-fetuin. The cell-association of 125I-HDL did not require divalent cations and could be reduced by pronase treatment of the cells. The binding site was clearly different from the receptor for LDL in extrahepatic cells since heparin and apolipoprotein E did not compete and cholesterol ester labelled HDL. The number of binding sites for HDL at 4 degrees C was 2.2 X 10(6) per cell and the association constant (Ka) 8.2 X 10(6) (mol/l)-1. Experiments with HDL labelled with [3H] cholesterol by means of lecithin: cholesterol acyltransferase (LCAT, EC 2.3.1.43) in the cholesterol ester moiety suggested that the same mechanism was responsible for the cell-association of HDL prepared this way.

Animals↗
PubMed1981

Binding, uptake, and degradation of 125I-labelled high-density lipoproteins in isolated non-parenchymal rat liver cells.

Binding, uptake, and degradation of 125I-labelled HDL were measured in isolated rat non-parenchymal cells in vitro. The binding exhibited saturation kinetics and was inhibited to various degrees with other unlabelled lipoproteins such as VLDL, LDL, and HDL, but the uptake was not reduced by heat and formaldehyde-denatured albumin. The binding could be reduced by pronase treatment of the cells. Acetylated 125I-labelled HDL were more effectively taken up and degraded by non-parenchymal cells than unmodified 125I-labelled HDL. The Kupffer cells were able to take up two to three times as much HDL per cell as the endothelial cells. 125I-labelled HDL bind to the plasma cell membrane and can be dissociated by addition of unlabelled HDL. Relatively more labelled HDL dissociate at 4 degrees C than at 37 degrees C. The non-parenchymal cells also internalize 125I-labelled HDL at 37 degrees C, and the lysosomotropic drug chloroquine inhibits partly the degradation of accumulated 125I-labelled HDL.

Animals↗
PubMed1980

The intracellular distribution of high density lipoproteins taken up by isolated rat hepatocytes.

The subcellular distribution of 125I-labelled HDL taken up by rat hepatocytes in vivo and in vitro has been studied with subcellular fractionation techniques: differential centrifugation and isopycnic centrifugation in sucrose gradients. 125I-labelled HDL bind to plasma membranes both in vivo and in vitro and part of the membrane-bound 125I-labelled HDL can be dissociated by the addition of unlabelled HDL. The hepatocytes also internalize 125I-labelled HDL. The 125I-labelled HDL accumulate, however, at different intracellular sites in the in vivo and in vitro situation. The subcellular distribution pattern of 125I-labelled HDL taken up by the cells in vivo is similar to that of the lysosomal marker enzyme acid phosphatase. Peak activity was found at a density of 1.20 g/ml. In vitro 125I-labelled HDL accumulate in an organelle with a medium density of about 1.13 g/ml. This distribution was similar to that of the plasma membrane marker 5'-nucleotidase. The subcellular distribution of radioactivity taken up in vivo was changed to lower density by incubating the cells with chloroquine, a drug known to render the lysosomes more boyant. Chloroquine had no effect on the distribution of 125I-labelled HDL taken up by hepatocytes in vitro.

5'-Nucleotidase↗
PubMed1979

Uptake and degradation of 125I-labelled high density lipoproteins in rat liver cells in vivo and in vitro.

1. The uptake of 125I-labelled high density lipoproteins (HDL) in various organs of the rat was determined after an intravenous injection. The uptake of 125I-labelled polyvinylpyrrolidone in the same organs was determined in order to assess uptake by fluid endocytosis. The uptake/organ was highest for the liver. The adrenals showed the highest uptake/unit weight of the organs studied. The liver, the kidneys and the spleen showed comparable values for uptake/g of tissue. The uptake of 125I-labelled HDL exceeded by far that of 125I-labelled polyvinylpyrrolidone in the liver, the kidneys, the spleen and the adrenals, indicating that the uptake of 125I-labelled HDL was mediated by adsorptive endocytosis. 2. The in vivo uptake of 125I-labelled HDL was determined in purified hepatocytes and non-parenchymal cells prepared by collagenase perfusion of livers from animals after intravenous injections of 125I-labelled HDL. When expressed per cell, the hepatocytes and the non-parenchymal liver cells took up about the same amount of 125I-labelled HDL. 3. The in vitro uptake and degradation of 125I-labelled HDL in isolated rat hepatocytes was studied. The uptake at increasing concentrations of 125I-labelled HDL was saturable indicating uptake mediated through binding sites. 125I-labelled HDL were easily degraded by contaminating proteases from the perfusate. 4. Subcellular fractionation by isopycnic centrifugation indicated that the accumulation of 125I-labelled HDL did not take place in the lysosomes, but rather on the plasma membrane and possibly in the endosomes (phagosomes). 5. 125I-labelled HDL were internalized into the cells and degraded in the lysosomes. Leupetin and chloroquine, inhibitors of the lysosomal function effectively inhibited the formation of 125I-labelled acid-soluble radioactivity by the cells. Chloroquine, but not the protease inhibitor leupeptin, reduced the hydrolysis of the cholesteryl ester moiety of HDL.

Animals↗
PubMed1977

A heritable syndrome of craniosynostosis, short thin hair, dental abnormalities, and short limbs: cranioectodermal dysplasia.

Five children are reported with dolichocephaly (with sagittal suture synostosis in three), sparse, slow-growing, fine hair, epicanthal folds, hypodontia and/or microdontia, short span, brachydactyly and brachypodia, and narrow thoraces. Radiologic abnormalities were noted chiefly in the skull, chest, and limbs. Intelligence was normal. The pattern of inheritance is compatible with an autosomal recessive trait.

Abnormalities, Multiple↗
PubMed1977

Prophylactiv use of propranolol in the Marfan syndrome to prevent aortic dissection.

Twenty-five patients, 16 men and 9 women, 5-59 years of age with the Marfan syndrome and cardiac complications were started on propranolol over a 6-year period. Propranolol was used as a beta-adrenergic blocking agent to reduce myocardial contractility in an attempt thereby to stay the progression of aortic dilatation and to prevent acute dissection of the aorta. The indications for prophylactic treatment were aortic dilatation, with aortic regurgitation in most cases. It was intended to keep the pulse rate below 70 at all times or below 60 at rest. Propranolol given in daily doses of 120-160 mg caused no side effects. The mean observation time for propranolol treatment in the 25 patients was 3.0 +/- 1.8 years ranging from 1-7 years. In spite of treatment, 5 patients (1 female and 4 males) experienced acute aortic dissection of rupture with fatal outcome. This occurred in 2 patients with the asthenic type, in 2 patients with the nonasthenic type, and in 1 patient with the marfanoid hypermobility syndrome. Serial echocardiograms showed that other patients on propranolol developed increasing dilatation of the aortic root. These observations indicate that propranolol does not necessarily protect against aortic dissection nor stop the progression of the aortic dilatation when cystic medial necrosis of the aorta is already present.

Adolescent↗
PubMed1976

Serum beta-lipoprotein subfractions in polyacrylamide gel electrophoresis associated with coronary heart disease.

Subfractions of beta-lipoprotein occurred more frequently in serum from patients with coronary heart disease than in serum from patients with no sign of coronary heart disease. Two beta-lipoprotein subfractions were observed in polyacrylamide gel electrophoresis. The sera containing a beta-lipoprotein subfraction with a position close to the beta-lipoprotein band showed Lp(a) antigenic properties. Triglycerides were raised in the sera with beta-lipoprotein subfractions. The cholesterol level was significantly higher in the coronary heart disease group but was not correlated to the presence of the beta-lipoprotein subfraction. The beta-lipoprotein subfraction may possibly represent an additional risk factor in the genesis of coronary heart disease, even in the absence of other hyperlipoproteinemias.

Adolescent↗
PubMed1976

Neonatal osteomyelitis caused by group B streptococci.

A 3-week-old infant with a group B streptococcal osteomyelitis is described. On admission swelling and fluctuation were combined with local erythema in the right ankle joint region. Group B streptococci, type Ib, were isolated both from blood and from the local focus in the ankle. The diagnosis was further confirmed by X-ray examination showing a lytic lesion in the talus. The child was successfully treated with penicillin, initially in combination with kanamycin.

Ankle Joint↗