Cellular transport of <sc>l</sc>-histidine in Hartnup disease

Sverre Halvorsen; Olof Hygstedt; Rudolf Jagenburg; Ottar Sjaastad

doi:10.1172/JCI106121

^{Children's Hospital and Pediatric Research Institute, Rikshospitalet, Oslo, Norway}

^{Department of Medical Biochemistry, University of Gothenburg, Gothenburg, Sweden}

^{Department of Clinical Chemistry, University of Gothenburg, Gothenburg, Sweden}

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^{Children's Hospital and Pediatric Research Institute, Rikshospitalet, Oslo, Norway}

^{Department of Medical Biochemistry, University of Gothenburg, Gothenburg, Sweden}

^{Department of Clinical Chemistry, University of Gothenburg, Gothenburg, Sweden}

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^{Children's Hospital and Pediatric Research Institute, Rikshospitalet, Oslo, Norway}

^{Department of Medical Biochemistry, University of Gothenburg, Gothenburg, Sweden}

^{Department of Clinical Chemistry, University of Gothenburg, Gothenburg, Sweden}

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^{Children's Hospital and Pediatric Research Institute, Rikshospitalet, Oslo, Norway}

^{Department of Medical Biochemistry, University of Gothenburg, Gothenburg, Sweden}

^{Department of Clinical Chemistry, University of Gothenburg, Gothenburg, Sweden}

Find articles by Sjaastad, O. in: PubMed | Google Scholar

Published in Volume 48, Issue 8 on August 1, 1969
J Clin Invest. 1969;48(8):1552–1559. https://doi.org/10.1172/JCI106121.
© 1969 The American Society for Clinical Investigation

Published August 1, 1969 - Version history

The urinary excretion, the intestinal absorption, and the elimination of histidine from blood were studied in two patients with Hartnup disease. On standard diet the patients lost a great proportion of the dietary histidine in the urine, whereas the fecal loss was negligible. A high oral dose of L-histidine gave only a slight increase in plasma histidine and no increase in fecal histidine, but a considerable increase in the urinary histidine output. Intravenously administered L-histidine was eliminated more rapidly than in controls. The lack of increase in plasma histidine after the oral loading may be explained by the rapid elimination from the blood. This was mainly due to a rapid cellular uptake of histidine which is supposed to be a normal reaction of histidine-deprived cells. Thus the only obvious defect in the histidine transport in Hartnup disease is the reabsorption defect in the renal tubules. A generally impaired cellular transport of L-histidine is improbable.