Immunolocalization of glutaryl-CoA dehydrogenase (GCDH) in adult and embryonic rat brain and peripheral tissues.

Details

Serval ID
serval:BIB_31DB58187F90
Type
Article: article from journal or magazin.
Collection
Publications
Institution
Title
Immunolocalization of glutaryl-CoA dehydrogenase (GCDH) in adult and embryonic rat brain and peripheral tissues.
Journal
Neuroscience
Author(s)
Braissant O., Jafari P., Remacle N., Cudré-Cung H.P., Do Vale Pereira S., Ballhausen D.
ISSN
1873-7544 (Electronic)
ISSN-L
0306-4522
Publication state
Published
Issued date
20/02/2017
Peer-reviewed
Oui
Volume
343
Pages
355-363
Language
english
Notes
Publication types: Journal Article
Publication Status: ppublish
Abstract
Glutaryl-CoA dehydrogenase (GCDH) is a mitochondrial enzyme that is involved in the degradation of tryptophan, lysine and hydroxylysine. Deficient enzyme activity leads to glutaric aciduria type-I (GA-I). This neurometabolic disease usually manifests with acute encephalopathic crises and striatal neuronal death in early childhood leading to an irreversible dystonic-dyskinetic movement disorder. Fronto-temporal atrophy and white matter changes are already present in the pre-symptomatic period. No detailed information on GCDH expression during embryonic development and in adulthood was available so far. Using immunofluorescence microscopy and cell-type-specific markers to localize GCDH in different tissues, we describe the differential cellular localization of GCDH in adult rat brain and peripheral organs as well as its spatiotemporal expression pattern. During embryonic development GCDH was predominantly expressed in neurons of the central and peripheral nervous system. Significant expression levels were found in epithelial cells (skin, intestinal and nasal mucosa) of rat embryos at different developmental stages. Besides the expected strong expression in liver, GCDH was found to be significantly expressed in neurons of different brain regions, renal proximal tubules, intestinal mucosa and peripheral nerves of adult rats. GCDH was found widely expressed in embryonic and adult rat tissues. In rat embryos GCDH is predominantly expressed in brain implying an important role for brain development. Interestingly, GCDH was found to be significantly expressed in different other organs (e.g. kidney, gut) in adult rats probably explaining the evolving phenotype in GA-I patients.

Keywords
Animals, Brain/cytology, Brain/enzymology, Brain/growth & development, Epithelial Cells/cytology, Epithelial Cells/enzymology, Female, Fluorescent Antibody Technique, Glutaryl-CoA Dehydrogenase/genetics, Glutaryl-CoA Dehydrogenase/metabolism, Intestinal Mucosa/cytology, Intestinal Mucosa/enzymology, Intestinal Mucosa/growth & development, Kidney/cytology, Kidney/enzymology, Kidney/growth & development, Liver/cytology, Liver/enzymology, Liver/growth & development, Lung/cytology, Lung/enzymology, Lung/growth & development, Mice, Knockout, Microscopy, Fluorescence, Muscle Development/physiology, Muscles/cytology, Muscles/enzymology, Neurons/cytology, Neurons/metabolism, Peripheral Nervous System/cytology, Peripheral Nervous System/enzymology, Peripheral Nervous System/growth & development, Rats, Sprague-Dawley, central and peripheral nervous system, embryonic development, expression, glutaric aciduria type-I, glutaryl-CoA-dehydrogenase, rat tissues
Pubmed
Web of science
Create date
29/12/2016 10:57
Last modification date
20/08/2019 14:17
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