Document Detail

Ornithine transcarbamylase (OTC) in white blood cells.
MedLine Citation:
PMID:  7208155     Owner:  NLM     Status:  MEDLINE    
A radiochemical assay method of ornithine transcarbamylase (OTC) was developed using labeled carbamyl phosphate as a substrate. The enzyme activities determined by this method in peripheral white blood cells from ten normal subjects were 1.32 +/- 0.95 nmoles/mg/hr and the apparent Km's, when assayed at pH 8.5, were 6.4 mM for ornithine and 0.6 mM for carbamyl phosphate. On the contrary, the apparent Km's of human liver OTC were 0.6 mM for ornithine and 0.12 mM for carbamyl phosphate. The average OTC activity of granulocytes was 1.0 nmoles/mg/hr, whereas that of mononuclear cells was 0.4 mmoles/mg/hr. Lymphoid cell lines were established from three normal subjects and an OTC-deficient infant. All these cell lines demonstrated no OTC activity. When arginine was removed from the medium and replaced by ornithine, the lymphoid cells were unable to grow in culture. On autoradiography, the lymphoid cells showed labelling at incubation in the presence of 14C-citrulline, but not with 14C-ornithine.
N Nagata; I Akaboshi; J Yamamoto; I Matsuda; H Ohtsuka; T Katsuki
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Pediatric research     Volume:  14     ISSN:  0031-3998     ISO Abbreviation:  Pediatr. Res.     Publication Date:  1980 Dec 
Date Detail:
Created Date:  1981-05-21     Completed Date:  1981-05-21     Revised Date:  2009-11-19    
Medline Journal Info:
Nlm Unique ID:  0100714     Medline TA:  Pediatr Res     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  1370-3     Citation Subset:  IM    
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MeSH Terms
Amino Acid Metabolism, Inborn Errors / diagnosis
Ammonia / blood
Carbamyl Phosphate / diagnostic use
Carbon Radioisotopes
Cells, Cultured
Leukocytes / enzymology*
Liver / enzymology
Ornithine Carbamoyltransferase / blood*
Ornithine Carbamoyltransferase Deficiency Disease
Radioligand Assay / methods
Reg. No./Substance:
0/Carbon Radioisotopes; 590-55-6/Carbamyl Phosphate; 7006-33-9/Ornithine; 7664-41-7/Ammonia; EC Carbamoyltransferase

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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