Hepatocyte Transplantation Improves Phenotype and Extends Survival in a Murine Model of Intermediate Maple Syrup Urine Disease

Maple syrup urine disease (MSUD; OMIM 248600) is an inborn error of metabolism of the branched chain α-ketoacid dehydrogenase (BCKDH) complex that is treated primarily by dietary manipulation of branched-chain amino acids (BCAA). Dietary restriction is lifelong and compliance is difficult. Liver tra...

Fuld beskrivelse

Na minha lista:
Bibliografiske detaljer
Main Authors: Skvorak, Kristen J, Paul, Harbhajan S, Dorko, Kenneth, Marongiu, Fabio, Ellis, Ewa, Chace, Donald, Ferguson, Carolyn, Gibson, K Michael, Homanics, Gregg E, Strom, Stephen C
Format: Artigo
Sprog:Inglês
Udgivet: Nature Publishing Group 2009
Fag:
Original Articles
Online adgang:https://ncbi.nlm.nih.gov/pmc/articles/PMC2835204/
https://ncbi.nlm.nih.gov/pubmed/19436271
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1038/mt.2009.99
Tags: Tilføj Tag
Ingen Tags, Vær først til at tagge denne postø!

Lignende værker