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Biomedical subjects

M Grønn

Publications and source records attributed to M Grønn.

24 records · Page 2Linked to original sources
PubMed1989

Adrenoleukodystrophy. The chain shortening of erucic acid (22:1(n-9)) and adrenic acid (22:4(n-6)) is deficient in neonatal adrenoleukodystrophy and normal in X-linked adrenoleukodistrophy skin fibroblasts.

The metabolism of long chain unsaturated fatty acids was studied in cultured fibroblasts from patients with X-linked adrenoleukodystrophy (ALD) and with neonatal ALD. By using [14-14C] erucic acid (22:1(n-9)) as substrate it was shown that the peroxisomal beta-oxidation, measured as chain shortening, was impaired in cells from patients with neonatal ALD. The beta-oxidation of adrenic acid (22:4(n-6)), measured as acid-soluble products, was also reduced in the neonatal ALD cells. The peroxisomal beta-oxidation of [14-14C]erucic acid (22:1(n-9)) and [2-14C]adrenic acid (22:4(n-6)) was normal in cells from X-ALD patients. The beta-oxidation, esterification and chain elongation of [1-14C]arachidonic acid (20:4(n-6)) and [1-14C]eicosapentaenoic acid (20:5(n-3)) was normal in both X-linked ALD and in neonatal ALD. Previous studies suggest that the activation of very long chain fatty acids by a lignoceryl (24:0)-CoA ligase is deficient in X-linked ALD, while the peroxisomal beta-oxidation enzymes are deficient in neonatal ALD. The present results suggest that the peroxisomal very long-chain acyl-CoA ligase is not required for activation of unsaturated C20 and C22 fatty acids and that these fatty acids can be efficiently activated by the long chain acyl-(palmityl)-CoA ligase.

Adrenoleukodystrophy↗
PubMed1989

Dietary n-3 fatty acids and children with hyperlipidaemia.

A review of the literature shows that no studies on children with primary genetic lipid disorders have been published. Case reports are presented indicating that hyperlipidaemias presenting in children--both familial hypercholestrolaemia and disorders with secondary hyperlipidaemia (triglycerides) may benefit of the intake of n-3 fatty acids.

Adolescent↗
PubMed1988

Regulation of the metabolism of polyunsaturated fatty acids.

The object of the present work has been to assess some aspects of the control of the metabolism of polyunsaturated fatty acids. In these studies we have used isolated cells; hepatocytes and cardiac myocytes from rat, and lymphocytes and fibroblasts from humans. Attention has focused on the importance of dietary and hormonal factors which can exert an effect shortly after activation of fatty acid, and thus may determine whether the fatty acid will be desaturated, chain-elongated, directly esterified or oxidized. Both the short-term and long-term regulation excerted by dietary fat have been studied in both rat and human. Profound sex-differences exist in fatty acid esterification, oxidation and fatty acid composition in lipoproteins. It is suggested that these differences, at least in part are due to a different content of intracellular fatty acid binding proteins in female and male liver. The peroxisomal retroconversion of C22-unsaturated fatty acids to their C20-homologues is probably involved in the regulation of fatty acid composition in membrane phospholipids, being a sort of "overflow valve". In several somewhat different diseases the patients have no peroxisomes or their peroxisomes are defective. In these peroxisomal diseases the beta-oxidation of very long-chain fatty acids is deficient. In this present work we show that fibroblast from patients with the peroxisomal diseases, Zellweger disease, X-linked adrenoleukodystrophy and neonatal adrenoleukodystrophy differ in their ability to shorten C22-fatty acids.

Animals↗
PubMed1986

C-reactive protein as an indicator of infection in the immunosuppressed child.

C-reactive protein (CRP) concentrations were determined in a prospective fashion in 50 children with malignant disease. In 35 children with active and aggressive disease, but without signs of infection, no significant increase in CRP was detected. Neither did aggressive cytostatic therapy (70 courses) in non-infected children result in an increase. In bacteriologically proven, and in clinical sepsis-suspected cases, CRP values increased in all cases to levels above 100 mg/l (normal values less than 5 mg/l). Effective antibiotic therapy resulted in a prompt decline in CRP. Viral infections resulted in a much smaller increase. We conclude that serial measurements of CRP in these immunosuppressed children are of great help in monitoring infections and defining the group that needs antibiotic therapy. The measurement is also a good indicator of the effectiveness of the antibiotic therapy chosen.

Adolescent↗