Complementation in Argininosuccinate Synthetase and Argininosuccinate Lyase Deficiencies in Human Fibroblasts

  • L. Cathelineau
  • D. Pham Dinh
  • P. Briand
  • P. Kamoun
Part of the Advances in Experimental Medicine and Biology book series (AEMB, volume 153)


Gene complementation analysis of genetically defective cells is a useful tool in the knowledge of the heterogeneity of the inherited diseases. We have used the polyethylene glycol fusion on fibroblasts deficient either in argininosuccinate synthetase (ASS) or in argininosuccinate lyase (ASL) to detect a possible heterogeneity of citrullinemia or argininosuccinic aciduria. A slight modification of the method of Tedesco and Mellmanl was used in the search of a positive complementation.


Human Fibroblast Urea Cycle Complementation Test Urea Cycle Disorder Argininosuccinate Synthetase 
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Copyright information

© Springer Science+Business Media New York 1982

Authors and Affiliations

  • L. Cathelineau
    • 1
  • D. Pham Dinh
    • 1
  • P. Briand
    • 1
  • P. Kamoun
    • 1
  1. 1.Laboratoire de Biochimie GénétiqueHôpital Necker- Enfants MaladesParis Cedex 15France

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