Hepatocyte transplantation (HTx) corrects selected neurometabolic abnormalities in murine intermediate maple syrup urine disease (iMSUD).

Skvorak, Kristen J; Hager, Elizabeth J; Arning, Erland; Bottiglieri, Teodoro; Paul, Harbhajan S; Strom, Stephen C; Homanics, Gregg E; Sun, Qin; Jansen, Erwin E W; Jakobs, Cornelis; Zinnanti, William J; Gibson, K Michael

Skvorak, Kristen J; Hager, Elizabeth J; Arning, Erland; Bottiglieri, Teodoro; Paul, Harbhajan S; Strom, Stephen C; Homanics, Gregg E; Sun, Qin; Jansen, Erwin E W; Jakobs, Cornelis; Zinnanti, William J; Gibson, K Michael.

Affiliation

Skvorak KJ; Department of Pediatrics, University of Pittsburgh School of Medicine, USA; Children's Hospital of Pittsburgh of UPMC, USA.

Biochim Biophys Acta ; 1792(10): 1004-10, 2009 Oct.

Article in En | MEDLINE | ID: mdl-19699299

Subject(s)

Brain/abnormalities; Brain/metabolism; Hepatocytes/transplantation; Maple Syrup Urine Disease/pathology; Maple Syrup Urine Disease/therapy; Animals; Brain/pathology; Carnosine/analogs & derivatives; Carnosine/metabolism; Humans; Liver/pathology; Mice; Models, Biological; Neurotransmitter Agents/metabolism; gamma-Aminobutyric Acid/metabolism

Fulltext

Add to My VHL

XML

PubMed Links

Search on Google

Full text: 1 Collection: 01-internacional Database: MEDLINE Main subject: Brain / Hepatocytes / Maple Syrup Urine Disease Limits: Animals / Humans Language: En Journal: Biochim Biophys Acta Year: 2009 Document type: Article Affiliation country: United States Country of publication: Netherlands

Fulltext

Add to My VHL

XML

PubMed Links

Search on Google