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Immunolocalization of glutaryl-CoA dehydrogenase (GCDH) in adult and embryonic rat brain and peripheral tissues
- Source :
- Neuroscience. 343
- Publication Year :
- 2016
-
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.
- Subjects :
- 0301 basic medicine
medicine.medical_specialty
Fluorescent Antibody Technique
610 Medicine & health
Glutaryl-CoA dehydrogenase
Biology
Kidney
Muscle Development
Rats, Sprague-Dawley
03 medical and health sciences
Atrophy
Intestinal mucosa
Internal medicine
Peripheral Nervous System
medicine
Animals
Intestinal Mucosa
Lung
Cellular localization
Mice, Knockout
Neurons
Glutaryl-CoA Dehydrogenase
General Neuroscience
Muscles
Embryogenesis
2800 General Neuroscience
Brain
Embryo
Epithelial Cells
medicine.disease
030104 developmental biology
Endocrinology
medicine.anatomical_structure
Biochemistry
Liver
Microscopy, Fluorescence
10036 Medical Clinic
Peripheral nervous system
Female
Subjects
Details
- ISSN :
- 18737544
- Volume :
- 343
- Database :
- OpenAIRE
- Journal :
- Neuroscience
- Accession number :
- edsair.doi.dedup.....3901163a79d2661ac4c1786799365cfd