Classical maple syrup urine disease and brain development: Principles of management and formula design

Molecular Genetics and Metabolism

Volumn 99, Issue 4, 2010, Pages 333-345

  Strauss, Kevin A ^a,b,c   Wardley, Bridget ^d,e   Robinson, Donna ^a   Hendrickson, Christine ^a   Rider, Nicholas L ^a,b   Puffenberger, Erik G ^a,b,c   Shelmer, Diana ^f   Moser, Ann B ^g   Morton, D Holmes ^a,b,c  

^a CLINIC FOR SPECIAL CHILDREN   (United States)

^b LANCASTER GENERAL HOSPITAL   (United States)

^c FRANKLIN AND MARSHALL COLLEGE   (United States)

^d Applied Nutrition Corporation   (United States)

^e NEW YORK UNIVERSITY   (United States)

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

^g KENNEDY KRIEGER INSTITUTE   (United States)

Author keywords

Alpha ketoisocaproic acid; Amino acid transport; Blood brain barrier; Brain development; Branched chain ketoacid dehydrogenase; Leucine; Maple syrup urine disease

Indexed keywords

ALANINE; DOCOSAHEXAENOIC ACID; GLUTAMIC ACID; GLUTAMINE; HISTONE; ISOLEUCINE; LAT 1 PROTEIN; LAT PROTEIN; LEUCINE; LINOLEIC ACID; METHIONINE; OMEGA 3 FATTY ACID; PHENYLALANINE; SELENIUM; THREONINE; TRYPTOPHAN; TYROSINE; UNCLASSIFIED DRUG; VALINE; ZINC;

AMINO ACID TRANSPORT; ARTICLE; BRAIN DEVELOPMENT; CHILD; CONTROLLED STUDY; ENERGY; ERYTHROCYTE MEMBRANE; ESSENTIAL FATTY ACID DEFICIENCY; GASTROENTERITIS; HOSPITALIZATION; HUMAN; INFECTION; MAJOR CLINICAL STUDY; MALE; MAPLE SYRUP URINE DISEASE; NERVOUS SYSTEM; PRESCHOOL CHILD; PRIORITY JOURNAL; PROTEIN DEGRADATION; PROTEIN DEPLETION; SELENIUM DEFICIENCY; TRANSAMINATION; VOMITING; ZINC DEFICIENCY;

AMINO ACIDS; BRAIN; CHILD, PRESCHOOL; FOOD, FORMULATED; HOSPITALIZATION; HUMANS; INFANT; LEUCINE; MAPLE SYRUP URINE DISEASE; NUTRITIONAL REQUIREMENTS;

ACER;

EID: 77649337792     PISSN: 10967192     EISSN: 10967206     Source Type: Journal    
DOI: 10.1016/j.ymgme.2009.12.007     Document Type: Article

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