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l-Serine synthesis in the central nervous system : A review on serine deficiency disorders

Tabatabaie, L. ; Klomp, L. W. ; Berger, R. and de Koning, T. J. LU (2010) In Molecular Genetics and Metabolism 99(3). p.256-262
Abstract

The de novo synthesis of the amino acid l-serine plays an essential role in the development and functioning of the central nervous system (CNS). l-Serine displays many metabolic functions during different developmental stages; among its functions providing precursors for amino acids, protein synthesis, nucleotide synthesis, neurotransmitter synthesis and l-serine derived lipids. Patients with congenital defects in the l-serine synthesizing enzymes present with severe neurological abnormalities and underscore the importance of this synthetic pathway. In this review, we will discuss the cellular functions of the l-serine pathway, structure and enzymatic properties of the enzymes involved and genetic defects associated with this... (More)

The de novo synthesis of the amino acid l-serine plays an essential role in the development and functioning of the central nervous system (CNS). l-Serine displays many metabolic functions during different developmental stages; among its functions providing precursors for amino acids, protein synthesis, nucleotide synthesis, neurotransmitter synthesis and l-serine derived lipids. Patients with congenital defects in the l-serine synthesizing enzymes present with severe neurological abnormalities and underscore the importance of this synthetic pathway. In this review, we will discuss the cellular functions of the l-serine pathway, structure and enzymatic properties of the enzymes involved and genetic defects associated with this pathway.

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author
; ; and
publishing date
type
Contribution to journal
publication status
published
subject
keywords
3-PGDH deficiency, d-Serine, l-Serine, PSAT deficiency, PSPH deficiency
in
Molecular Genetics and Metabolism
volume
99
issue
3
pages
256 - 262
publisher
Elsevier
external identifiers
  • pmid:19963421
  • scopus:76349118741
ISSN
1096-7192
DOI
10.1016/j.ymgme.2009.10.012
language
English
LU publication?
no
id
f74a51c3-6e6a-47f5-a8ab-c7afc5cceb88
date added to LUP
2020-02-28 13:48:22
date last changed
2024-09-19 19:22:03
@article{f74a51c3-6e6a-47f5-a8ab-c7afc5cceb88,
  abstract     = {{<p>The de novo synthesis of the amino acid l-serine plays an essential role in the development and functioning of the central nervous system (CNS). l-Serine displays many metabolic functions during different developmental stages; among its functions providing precursors for amino acids, protein synthesis, nucleotide synthesis, neurotransmitter synthesis and l-serine derived lipids. Patients with congenital defects in the l-serine synthesizing enzymes present with severe neurological abnormalities and underscore the importance of this synthetic pathway. In this review, we will discuss the cellular functions of the l-serine pathway, structure and enzymatic properties of the enzymes involved and genetic defects associated with this pathway.</p>}},
  author       = {{Tabatabaie, L. and Klomp, L. W. and Berger, R. and de Koning, T. J.}},
  issn         = {{1096-7192}},
  keywords     = {{3-PGDH deficiency; d-Serine; l-Serine; PSAT deficiency; PSPH deficiency}},
  language     = {{eng}},
  month        = {{03}},
  number       = {{3}},
  pages        = {{256--262}},
  publisher    = {{Elsevier}},
  series       = {{Molecular Genetics and Metabolism}},
  title        = {{l-Serine synthesis in the central nervous system : A review on serine deficiency disorders}},
  url          = {{http://dx.doi.org/10.1016/j.ymgme.2009.10.012}},
  doi          = {{10.1016/j.ymgme.2009.10.012}},
  volume       = {{99}},
  year         = {{2010}},
}