Search PubMed⌕ Search

Biomedical subjects

C Lacombe

Publications and source records attributed to C Lacombe.

At least 145 records · Page 8Linked to original sources

A new case of Hb Little Rock [beta 143(H21)His----Gln], a high affinity variant. Study during pregnancy.

A new case of Hb Little Rock [beta 143(H21)His----Gln] is described. This high affinity variant (P50 = 15 mm Hg in whole cells) has a nearly normal cooperativity. The abnormal beta chain is readily detectable using urea-Triton X-100 electrophoresis. The altered beta T-14 peptide was separated by high performance liquid chromatography. The proposita was followed during a pregnancy. Oxygen unloading was reduced, but normal oxygen loading was maintained in the fetus. There were neither placental abnormalities, nor intrauterine growth retardation.

Adult↗

Further characterization of Hb Henri Mondor or alpha 2 beta 2(26)(B8)Glu----Val.

A second case of Hb Henri Mondor is reported. The subject, homozygous for Hb Henri Mondor, is of Algerian origin. The electrophoretical behavior and structural characterization are given and discussed. Hb Henri Mondor, which is characterized by the replacement of the lysine residue in position beta 26, as is the case for Hb E, has normal functional properties and is normally expressed.

Amino Acids↗

Biochemical analyses of murine erythropoietin from plasma and from cloned erythroleukemia cells.

The hydrophobicity, ionic charges, degrees of glycosylation, and Western blot patterns of murine plasma erythropoietin and erythropoietin produced by clones of two murine erythroleukemic cell lines (IW32 and NN10) were compared. Erythropoietins from these different sources exhibited a number of similarities in their biochemical properties: identical retention on DEAE Affigel blue column and similar apparent molecular weights (30-36 kDa) in Western blot analysis. However, differences were also observed: heterogeneous binding to different lectin columns and varied retention on a phenyl Sepharose column. The data thus confirm that these murine plasma erythropoietins have biochemical properties in common with previously studied erythropoietins from humans or sheep. The major difference between erythropoietins secreted by erythroleukemic cell lines and other molecules belonging to the same family appears to be related to their glycosylation.

Animals↗

Expression of the erythropoietin gene.

Injection of cobalt into rats resulted in erythropoietin (EPO) mRNA accumulation in the kidney. The same response was obtained upon bleeding. No EPO mRNA was detected in the spleen, salivary gland, or thymus following cobalt injection or bleeding. In some animals, but not in others, EPO mRNA was also expressed in the liver in response to cobalt injection. Time course studies showed that message appearance begins sometime between 3 and 6 h after cobalt injection. This correlated very well with the EPO concentration in the circulation; EPO levels in the circulation were the same as those of controls at 3 h but increased to six- to sevenfold that of controls by 6 h after cobalt injection. The mature EPO mRNA in the rat and mouse comigrated with the 18S rRNA, indicating that it is about 1,850 nucleotides in length.

Animals↗

Hemoglobin J Iran alpha 2 beta 2 77 (EF1) his----Asp in a Russian-Armenian family.

A third case of Hb J Iran is reported. The propositus is of Russian-Armenian origin and was investigated for hematuria. The electrophoretic behavior and the characterization of primary structure are described. Hb J Iran is stable and has normal functional properties. High resolution Nuclear Magnetic Resonance spectra suggest the presence of structural perturbations in the heme pocket of the variant. Solubility studies of Hb S/Hb J Iran mixture indicated that His beta 77 belongs to a contact region of deoxy Hb S polymers.

Adolescent↗

[Association of autoimmune chronic active hepatitis and acquired erythroblastopenia cured by cyclophosphamide].

To the best of our knowledge this is the reported first case of the successive occurrence in the same patient of chronic active hepatitis and acquired pure red cell aplasia, both probably of autoimmune origin. The diagnosis of autoimmune hepatitis was based on the presence of characteristic lesions at the examination of the liver biopsy specimen, high titer of anti-smooth muscle antibodies in the serum, and remission obtained by steroid therapy. Erythroid aplasia, which appeared during the course of this treatment, was revealed by a regenerative anemia (4.4 g Hb/100 ml) and proved by bone marrow aspiration and biopsy. In vitro bone marrow culture was normal, suggesting the in vivo presence of an inhibitor of erythroblastic differentiation. Red cell aplasia was cured by cyclophosphamide (100 mg/day during 56 days). No recurrence was noted until the death of the patient, which occurred one year later, due to hepatic cholangiocarcinoma. Action of cyclophosphamide on the pure red cell aplasia suggested the immune origin of this disease. The liver and the bone marrow erythroid lineage have probably been the successive targets of immunologic dyscrasia.

Autoimmune Diseases↗

[Treatment of systemic scleroderma with ketanserin. Randomized, double-blind 6-months study of 27 cases].

Twenty-seven patients with systemic scleroderma and Raynaud's phenomenon underwent a randomised double blind therapeutic trial: monotherapy with Ketanserine (80 mg/day for 6 months) against Placebo. The secondary effects were comparable in both groups as were the withdrawals from the trial for aggravation of Raynaud's phenomenon (one in each group). No significant difference was observed between the two groups as regards the evolution of the Raynaud's phenomenon or skin changes. Dysphagia was improved in the Ketanserine group (p less than 0.05) but not in the Placebo group. Some patients in the Ketanserine group experienced an improvement in the Raynaud's phenomenon at the end of the trial period; there were no improvements in the Placebo group. Three haemorrheological parameters (total blood viscosity, plasma viscosity and thixotropism) were abnormal at the beginning of the trial and did not improve by the end in the Ketanserine group. The K infinity coefficient of Quemada's law was normal at the start of the trial and increased after treatment (p less than 0.05).

Clinical Trials as Topic↗

The effect of cholestyramine on apolipoproteins in cholesterol-fed rabbits.

The changes in apolipoproteins on cholestyramine therapy associated with cholesterol feeding were compared to those observed in cholesterol-fed rabbits. Only one molecular species of apo B, identified as apo B-100, was present whatever the dietary or pharmacological treatment indicating the hepatic origin of apo B-containing lipoproteins in these conditions. Cholestyramine has a clear preventive effect on the rise of apo B and apo E which appear following cholesterol feeding. These changes are highly correlated with those of the plasma cholesterol level. In addition, cholestyramine induced a 50% increase in apo AI compared with normal rabbits. These data strongly confirm the beneficial effects of cholestyramine in the treatment of hypercholesterolemia.

Animals↗

Hb Kokura alpha 2 47 (CE5) Asp----Gly beta 2 in a French Jewish family.

An abnormal hemoglobin was found in a young polycythemic man. Case study showed that the polycythemia was not due to hemoglobinopathy as the blood oxygen affinity was normal. Structural study of this variant permitted the characterization of a Hb Kokura alpha 2 47 (CE5) Asp----Gly beta 2 for the first time in France.

Adult↗

Structural and functional studies of hemoglobin Poissy alpha 2 beta 2(56) (D7) Gly----Arg and 86 (F2) Ala----Pro.

Hemoglobin Poissy alpha 2 beta 2(56) (D7) Gly----Arg and 86 (F2) Ala----Pro, is a new variant of the beta chain with two substitutions within the second exon of the corresponding gene. The electrophoretic mobilities are identical to those of Hb Hamadan alpha 2 beta 2(56) (D7) Gly----Arg as is the fingerprint of the tryptic hydrolysate of the two abnormal beta chains. The second substitution beta 86 Ala----Pro was detected by high-pressure liquid chromatography. Hb Poissy has a threefold increase in oxygen affinity with low Hill coefficient and diminished Bohr effect, which are restored to normal upon addition of 2,3-bisphosphoglycerate. Since the functional properties of Hb Hamadan (beta 56 Gly----Arg) have been described as normal, the abnormal function of Hb Poissy may be attributed to the beta 86 (F2) Ala----Pro substitution. Hb Poissy exhibits a mild instability and a greater reactivity of the thiol groups of the beta 93 (F9) Cys residues in the deoxy form than does Hb A. The oxidation rate of Hb Poissy is biphasic indicating a large inequivalence between the alpha and beta hemes. Thereafter NMR studies demonstrated that the beta 86 Ala----Pro substitution produces a displacement of the F helix closer to the heme plane and a large increase in the dynamic fluctuations of the tertiary structure on the proximal side of the beta hemes. These results lead to the conclusion that the beta 86 Ala----Pro substitution produces a destabilization of the F helix extending downwards to the FG corner and altering both the beta hemes and the alpha 1 beta 2 contacts.

Amino Acids↗

Effect of dietary restriction on the plasma apolipoprotein pattern in cholesterol-fed rabbits.

Dietary restriction (half of the control ration) was performed in rabbits given either standard or cholesterol-rich diets. The plasma apolipoproteins were studied on the total, d less than 1.21, lipoprotein fraction using polyacrylamide gel electrophoresis. A marked rise in the amount of both apo-B and apo-E appeared in cholesterol-fed rabbits and was enhanced by dietary restriction. These results reflect the aggravation of hypercholesterolemia when cholesterol feeding is carried out in underfed rabbits. In all groups only one molecular species of apo-B, identified as apo-B-100, was present after overnight fasting. Thus, lipoproteins which accumulate in the plasma following cholesterol feeding, associated with dietary restriction or not, probably are remnants of hepatogenous triglyceride-rich lipoproteins.

Animals↗

Effect of dietary restriction on cholesterol biosynthesis in the liver and the intestine of cholesterol-fed rabbits.

The effect of dietary restriction (half of the control ration) on cholesterol biosynthesis was investigated in rabbits fed either standard or cholesterol-rich diets. Accompanying the amplification of hypercholesterolemia, additional disturbances of cholesterol metabolism were observed when cholesterol feeding was associated with dietary restriction. In the intestine, underfed rabbits showed a more marked inhibition of duodenal cholesterol biosynthesis from [14C]acetate following cholesterol feeding than rabbits on normal caloric ration. In contrast liver cholesterogenesis was equally suppressed in both groups receiving cholesterol-rich diets. Cholesterol biosynthesis from [14C]mevalonate was also inhibited by cholesterol feeding particularly in the duodenum of underfed rabbits. In addition cholesterol feeding induced a marked increase of the labeled esterified: free cholesterol ratio in the liver, demonstrating intensive esterification, this was enhanced by dietary restriction. The additional cholesterol which accumulates in the plasma and in various tissues in underfed rabbits is of dietary origin since the feedback control of cholesterogenesis by exogenous cholesterol was shown to be very effective in these animals.

Animals↗

Hyperlipidemic dementia.

The dementia of a patient with hyperlipidemia improved dramatically on treatment with diet and fenofibrate. Rheologic study showed an abnormality of rouleaux disaggregation, and this disappeared as plasma lipid levels fell and mental state improved.

Clofibrate↗

Hemoglobin kenitra alpha 2 beta 2 69 (E13) Gly----Arg. A new beta variant of elevated expression associated with alpha-thalassemia, found in a Moroccan woman.

Hemoglobin Kenitra is a new variant of the beta chain alpha 2 beta 2 69 (E13) Gly----Arg which does not produce any clinical symptoms. It is a slow-moving hemoglobin with a distinctive pattern of electrophoretic mobilities. The stability test was negative. Oxygen affinity studies were not performed. It was found in association with alpha-thalassemia and microcytosis, but paradoxically a high expression of the variant (55%) was observed.

Amino Acids↗