The amino acid l-serine, one of the so-called non-essential amino acids, plays a central role in cellular proliferation. l-Serine is the predominant source of one-carbon groups for the de novo synthesis of purine nucleotides and deoxythymidine monophosphate. It has long been recognized that, in cell cultures, l-serine is a conditional essential amino acid, because it cannot be synthesized in sufficient quantities to meet the cellular demands for its utilization. In recent years, l-serine and the products of its metabolism have been recognized not only to be essential for cell proliferation, but also to be necessary for specific functions in the central nervous system. The findings of altered levels of serine and glycine in patients with psychiatric disorders and the severe neurological abnormalities in patients with defects of l-serine synthesis underscore the importance of l-serine in brain development and function. This paper reviews these recent insights into the role of l-serine and the pathways of l-serine utilization in disease and during development, in particular of the central nervous system.
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Review Article|
May 01 2003
l-Serine in disease and development
Tom J. de KONING;
Tom J. de KONING
1
∗Department of Pediatric Metabolic Diseases, University Medical Centre Utrecht, KC 03.063.0, P.O. Box 85090, 3508 AB Utrecht, The Netherlands
1To whom correspondence should be addressed (e-mail t.dekoning@wkz.azu.nl).
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Keith SNELL;
Keith SNELL
†Institute of Cancer Research, London SW7 3RP, U.K.
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Marinus DURAN;
Marinus DURAN
‡Laboratory for Genetic Metabolic Diseases, Academic Medical Centre, 1105AZ Amsterdam, The Netherlands
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Ruud BERGER;
Ruud BERGER
∗Department of Pediatric Metabolic Diseases, University Medical Centre Utrecht, KC 03.063.0, P.O. Box 85090, 3508 AB Utrecht, The Netherlands
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Bwee-Tien POLL-THE;
Bwee-Tien POLL-THE
§Department of Child Neurology, Emma Children's Hospital, Academic Medical Centre, 1105AZ Amsterdam, The Netherlands
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Robert SURTEES
Robert SURTEES
‖Neurosciences Unit, Institute of Child Health, University College London, London WC1N 1EH, U.K.
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Biochem J (2003) 371 (3): 653–661.
Article history
Received:
November 18 2002
Revision Received:
January 20 2003
Accepted:
January 21 2003
Accepted Manuscript online:
January 21 2003
Citation
Tom J. de KONING, Keith SNELL, Marinus DURAN, Ruud BERGER, Bwee-Tien POLL-THE, Robert SURTEES; l-Serine in disease and development. Biochem J 1 May 2003; 371 (3): 653–661. doi: https://doi.org/10.1042/bj20021785
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