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Mental retardation in Down syndrome: Two ways to treat.
- Source :
-
Medical hypotheses [Med Hypotheses] 2019 Oct; Vol. 131, pp. 109289. Date of Electronic Publication: 2019 Jun 26. - Publication Year :
- 2019
-
Abstract
- Mental retardation is a progressive condition in Down syndrome: intelligence starts to decline linearly within the first year. This phenomenon could be related to the overproduction of a toxic compound, hydrogen sulfide. Indeed, a gene located on chromosome 21 controls the production of cystathionine-β-synthase, an enzyme involved in hydrogen sulfide production in the central nervous system. It has recently been demonstrated that excess cystathionine-β-synthase levels are needed and sufficient to induce cognitive phenotypes in mouse models of Down syndrome. Thus, two therapeutic options might be used in Down syndrome patients: the use of a specific cystathionine β-synthase inhibitor and the use of an effective antidote to reduce hydrogen sulfide toxicity. Prenatal treatment of Down syndrome fetuses is also suggested.<br /> (Copyright © 2019 Elsevier Ltd. All rights reserved.)
- Subjects :
- Aminooxyacetic Acid therapeutic use
Animals
Benserazide therapeutic use
Brain metabolism
Chromosomes, Human, Pair 21 genetics
Cobamides therapeutic use
Cystathionine beta-Synthase antagonists & inhibitors
Cystathionine beta-Synthase genetics
Disease Models, Animal
Disease Progression
Disulfiram therapeutic use
Down Syndrome genetics
Enzyme Inhibitors therapeutic use
Gene Dosage
Humans
Hydrogen Sulfide metabolism
Infant, Newborn
Intellectual Disability drug therapy
Intellectual Disability genetics
Mice
Mitochondria metabolism
Rats
Sodium Nitrite therapeutic use
Species Specificity
Thiosulfates metabolism
Cystathionine beta-Synthase physiology
Down Syndrome psychology
Hydrogen Sulfide antagonists & inhibitors
Intellectual Disability therapy
Subjects
Details
- Language :
- English
- ISSN :
- 1532-2777
- Volume :
- 131
- Database :
- MEDLINE
- Journal :
- Medical hypotheses
- Publication Type :
- Academic Journal
- Accession number :
- 31443780
- Full Text :
- https://doi.org/10.1016/j.mehy.2019.109289