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

Biochimica et Biophysica Acta - Molecular Basis of Disease

Volumn 1792, Issue 10, 2009, Pages 1004-1010

  Skvorak, Kristen J ^a,b   Hager, Elizabeth J ^a,b   Arning, Erland ^c   Bottiglieri, Teodoro ^c   Paul, Harbhajan S ^d   Strom, Stephen C ^a   Homanics, Gregg E ^a   Sun, Qin ^e   Jansen, Erwin E W ^g   Jakobs, Cornelis ^g   Zinnanti, William J ^h   Gibson, K Michael ^a,b,f  

^a UNIVERSITY OF PITTSBURGH SCHOOL OF MEDICINE   (United States)

^b CHILDREN S HOSPITAL OF PITTSBURGH   (United States)

^c BAYLOR UNIVERSITY MEDICAL CENTER   (United States)

^d Biomed Research and Technologies Inc   (United States)

^e BAYLOR COLLEGE OF MEDICINE   (United States)

^f GRADUATE SCHOOL OF PUBLIC HEALTH   (United States)

^g VU UNIVERSITY AMSTERDAM   (Netherlands)

^h PENN STATE COLLEGE OF MEDICINE   (United States)

Author keywords

GABA; Hepatocyte transplantation (HTx); Intracellular turnover; Large neutral amino acid; Leucine; Monoamine

Indexed keywords

3 O METHYLDOPAMINE; 3,4 DIHYDROXYPHENYLACETIC ACID; 4 AMINOBUTYRIC ACID; ALLOISOLEUCINE; AMINO ACID TRANSPORTER; BRANCHED CHAIN AMINO ACID; DOPAMINE; GLUTAMINE; HOMOCARNOSINE; ISOLEUCINE; LEUCINE; MONOAMINE; NEUROTRANSMITTER; ORNITHINE; SEROTONIN; TRYPTOPHAN; TYROSINE; VALINE;

AMINO ACID ANALYSIS; ANIMAL CELL; ANIMAL EXPERIMENT; ANIMAL MODEL; ANIMAL TISSUE; ARTICLE; BRAIN METABOLISM; CATECHOLAMINE METABOLISM; CONTROLLED STUDY; HEPATOCYTE TRANSPLANTATION; MAPLE SYRUP URINE DISEASE; MOUSE; NONHUMAN; PRIORITY JOURNAL; TREATMENT OUTCOME;

ANIMALS; BRAIN; CARNOSINE; GAMMA-AMINOBUTYRIC ACID; HEPATOCYTES; HUMANS; LIVER; MAPLE SYRUP URINE DISEASE; MICE; MODELS, BIOLOGICAL; NEUROTRANSMITTER AGENTS;

ACER; ANIMALIA; MURINAE; MUS;

EID: 70349276673     PISSN: 09254439     EISSN: None     Source Type: Journal    
DOI: 10.1016/j.bbadis.2009.08.006     Document Type: Article

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