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Biomedical subjects

K Rasmussen

Publications and source records attributed to K Rasmussen.

At least 487 records · Page 27Linked to original sources
PubMed1972

Tiglicaciduria in propionicacidaemia.

Tiglic acid, which has not previously been found in human body fluids, was recently detected in the urine of two patients with propionicacidaemia. These patients had a documented defect in the oxidation of propionate. A competition between acrylyl-CoA and tiglyl-CoA for crotonase could explain the accumulation of tiglic acid.

Acrylates↗
PubMed1972

3-hydroxypropionate: significance of -oxidation of propionate in patients with propionic acidemia and methylmalonic acidemia.

[l-(14)C]Propionate administered intravenously was metabolized to methylmalonate, to 3-hydroxypropionate, and to methylcitrate in the urine of a patient with methylmalonic acidemia. L-[U-(14)C]Isoleucine and L-[U-(14)C]valine were also converted to urinary methylmalonate and to 3-hydroxypropionate in the patient. Two patients with propionic acidemia due to a defect in propionyl-CoA carboxylase metabolized [l-(14)C]propionate to uninary methylcitrate and 3-hydroxypropionate. The appearance of radioactive 3-hydroxypropionate in the urine after the administration of these compounds indicates that beta-oxidation of propionyl-CoA through acryloyl-CoA was functioning in these patients. The conversion of valine to 3-hydroxypropionate suggests that valine is oxidized by way of propionate and propionyl-CoA in man.

Acidosis↗