| Article ID | Journal | Published Year | Pages | File Type |
|---|---|---|---|---|
| 10832995 | Molecular Genetics and Metabolism | 2013 | 7 Pages |
Abstract
⺠Severe amino acid and neurotransmitter alterations are observed in a mouse model of intermediate Maple Syrup Urine Disease. ⺠Placental stem cells from human amnion were transplanted into the liver of affected mice. ⺠Significant and long-term corrections of amino acid and neurotransmitter levels were observed in the periphery as well as the Central Nervous System. ⺠Human placental stem cells from amnion should be considered for clinical transplant for metabolic liver diseases.
Keywords
Human amnion epithelial cells3-MT5-HIAADOPACBCKDHOLTDOLBCKAα-KGAECOAASerotonin, 5-hydroxytryptamineMSUDAmnion epithelial cells5-HT3-methoxytyramine3,4-dihydroxyphenylacetic acid5-Hydroxyindoleacetic acidbranched-chain α-ketoacid dehydrogenasealpha-ketoglutarateBranched chain amino acidAcetyl-CoAgamma-aminobutyric acidoxalacetateHAECAcoABCAAMaple syrup urine diseaseanalysis of varianceANOVADopaminehomovanillic acidorthotopic liver transplantHVAhigh performance liquid chromatographyHPLCGABA
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Authors
Kristen J. Skvorak, Kenneth Dorko, Fabio Marongiu, Veysel Tahan, Marc C. Hansel, Roberto Gramignoli, Erland Arning, Teodoro Bottiglieri, K. Michael Gibson, Stephen C. Strom,