Dual mechanism of brain injury and novel treatment strategy in maple syrup urine disease

Brain

Volumn 132, Issue 4, 2009, Pages 903-918

  Zinnanti, William J ^a   Lazovic, Jelena ^b   Griffin, Kathleen ^a   Skvorak, Kristen J ^c   Paul, Harbhajan S ^d   Homanics, Gregg E ^e   Bewley, Maria C ^a   Cheng, Keith C ^f   Lanoue, Kathryn F ^a   Flanagan, John M ^a  

^a PENN STATE COLLEGE OF MEDICINE   (United States)

^b CALIFORNIA INSTITUTE OF TECHNOLOGY   (United States)

^c UNIVERSITY OF PITTSBURGH   (United States)

^d Biomed Research and Technologies Inc   (United States)

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

^f PENN STATE CANCER INSTITUTE   (United States)

Author keywords

Branched chain amino acids; Cerebral oedema; Maple syrup urine disease; Metabolic decompensation; MRI

Indexed keywords

2 OXOISOCAPROIC ACID; BRANCHED CHAIN AMINO ACID; LEUCINE; NORLEUCINE;

ANIMAL EXPERIMENT; ANIMAL MODEL; ANIMAL TISSUE; ARTICLE; BEHAVIOR CHANGE; BLOOD; BRAIN; BRAIN DEVELOPMENT; BRAIN DISEASE; BRAIN INJURY; CATABOLISM; CONTROLLED STUDY; FEMALE; HETEROZYGOTE; HISTOPATHOLOGY; MALE; MAPLE SYRUP URINE DISEASE; MILK; MOUSE; NERVE GROWTH; NEUROPATHOLOGY; NONHUMAN; PERINATAL PERIOD; PRIORITY JOURNAL; PROTEIN DIET; SURVIVAL;

AMINO ACIDS; ANIMALS; BEHAVIOR, ANIMAL; BRAIN; BRAIN EDEMA; BRAIN MAPPING; DIETARY PROTEINS; DISEASE MODELS, ANIMAL; DRUG EVALUATION, PRECLINICAL; GROWTH DISORDERS; KETO ACIDS; MAGNETIC RESONANCE IMAGING; MAPLE SYRUP URINE DISEASE; MICE; MICE, KNOCKOUT; NORLEUCINE; SURVIVAL ANALYSIS;

EID: 64449086258     PISSN: 00068950     EISSN: 14602156     Source Type: Journal    
DOI: 10.1093/brain/awp024     Document Type: Article

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