l-Serine synthesis in the central nervous system : A review on serine deficiency disorders
(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
- Tabatabaie, L. ; Klomp, L. W. ; Berger, R. and de Koning, T. J. LU
- publishing date
- 2010-03-01
- 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}}, }