Inherent lipid metabolic dysfunction in glycogen storage disease IIIa

Biochemical and Biophysical Research Communications

Volumn 455, Issue 1-2, 2014, Pages 90-97

  Li, Xin Hua ^a,b   Gong, Qi Ming ^a   Ling, Yun ^a   Huang, Chong ^a   Yu, De Min ^a   Gu, Lei Lei ^a   Liao, Xiang Wei ^a   Zhang, Dong Hua ^a   Hu, Xi Qi ^c   Han, Yue ^a   Kong, Xiao Fei ^a   Zhang, Xin Xin ^a  

^a SHANGHAI JIAO TONG UNIVERSITY   (China)

^b THE THIRD AFFILIATED HOSPITAL OF SUN YAT SEN UNIVERSITY   (China)

^c FUDAN UNIVERSITY   (China)

Author keywords

AGL; Glycogen debranching enzyme; Glycogen storage disease type IIIa; MCAD

Indexed keywords

4ALPHA GLUCANOTRANSFERASE; FATTY ACID; PEROXISOME PROLIFERATOR ACTIVATED RECEPTOR; TRANSCRIPTOME; 4 ALPHA-GLUCANOTRANSFERASE; ACYL COENZYME A DEHYDROGENASE; AMYLO-1,6-GLUCOSIDASE; GLYCOGEN DEBRANCHING ENZYME; UNSATURATED FATTY ACID;

ADOLESCENT; ARTICLE; CASE REPORT; DISORDERS OF LIPID AND LIPOPROTEIN METABOLISM; FATTY ACID OXIDATION; FATTY ACID SYNTHESIS; GENE EXPRESSION; GLYCOGEN STORAGE DISEASE TYPE 3; GLYCOGEN STORAGE DISEASE TYPE 3A; HEPATOMEGALY; HETEROZYGOTE; HUMAN; HYPERLIPIDEMIA; HYPOGLYCEMIA; INTRAABDOMINAL FAT; LIPID METABOLISM; LIPID STORAGE; LIPOGENESIS; LIVER FUNCTION; LIVER FUNCTION TEST; LIVER HISTOLOGY; MALE; MEDIUM CHAIN ACYL COENZYME A DEHYDROGENASE DEFICIENCY; METABOLIC SYNDROME X; MUSCLE WEAKNESS; PHENOTYPE; PHYSICAL EXAMINATION; TANDEM MASS SPECTROMETRY; BIOSYNTHESIS; CELL CULTURE; DEFICIENCY; GENETICS; GLYCOGEN STORAGE DISEASE TYPE III; LIPID METABOLISM, INBORN ERRORS; METABOLISM; MUTATION;

ACYL-COA DEHYDROGENASE; ADOLESCENT; CELLS, CULTURED; FATTY ACIDS; FATTY ACIDS, UNSATURATED; GENE EXPRESSION; GLYCOGEN DEBRANCHING ENZYME SYSTEM; GLYCOGEN STORAGE DISEASE TYPE III; HUMANS; LIPID METABOLISM, INBORN ERRORS; MALE; MUTATION;

EID: 84912114305     PISSN: 0006291X     EISSN: 10902104     Source Type: Journal    
DOI: 10.1016/j.bbrc.2014.10.096     Document Type: Article

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