Sustaining hypercitrullinemia, hypercholesterolemia and augmented oxidative stress in Japanese children with aspartate/glutamate carrier isoform 2-citrin-deficiency even during the silent period

Molecular Genetics and Metabolism

Volumn 97, Issue 1, 2009, Pages 21-26

  Nagasaka, Hironori ^a   Okano, Yoshiyuki ^b   Tsukahara, Hirokazu ^c   Shigematsu, Yosuke ^c   Momoi, Toru ^d   Yorifuji, Junko ^d   Miida, Takashi ^e   Ohura, Toshihiro ^f   Kobayashi, Keiko ^g   Saheki, Takeyori ^h   Hirano, Kenichi ⁱ   Takayanagi, Masaki ^a   Yorifuji, Tohru ^d  

^a CHIBA CHILDREN'S HOSPITAL   (Japan)

^b OSAKA CITY UNIVERSITY GRADUATE SCHOOL OF MEDICINE   (Japan)

^c UNIVERSITY OF FUKUI HOSPITAL   (Japan)

^d KYOTO UNIVERSITY GRADUATE SCHOOL OF MEDICINE   (Japan)

^e JUNTENDO UNIVERSITY   (Japan)

^f TOHOKU UNIVERSITY HOSPITAL   (Japan)

^g KAGOSHIMA UNIVERSITY   (Japan)

^h TOKUSHIMA BUNRI UNIVERSITY   (Japan)

ⁱ OSAKA UNIVERSITY GRADUATE SCHOOL OF MEDICINE   (Japan)

Author keywords

Adult onset type II citrullinemia; Amino acids; Aspartate glutamate carrier isoform 2 citrin; Carbohydrates; Hypercholesterolemia; Neonatal intrahepatic cholestasis caused by citrin deficiency; Oxidative stress; Silent period

Indexed keywords

8 HYDROXYDEOXYGUANOSINE; ACROLEIN; ACROLEIN DERIVATIVE; APOLIPOPROTEIN A1; APOLIPOPROTEIN B; ASPARTIC ACID; CATALASE; CITRULLINE; GALACTOSE; GLUCOSE; GLUTAMIC ACID; HIGH DENSITY LIPOPROTEIN; LACTIC ACID; LOW DENSITY LIPOPROTEIN; LYSINE; NITRATE; NITRIC OXIDE; NITRITE; ORNITHINE; PYRUVIC ACID; SUPEROXIDE DISMUTASE; UREA;

AMINO ACID METABOLISM; ARTICLE; BLOOD ANALYSIS; CARBOHYDRATE METABOLISM; CHILD; CHOLESTEROL METABOLISM; CITRIN DEFICIENCY; CITRULLINEMIA; CLINICAL ARTICLE; CONTROLLED STUDY; FEMALE; HUMAN; HUMAN TISSUE; HYPERCHOLESTEROLEMIA; HYPERCITRULLINEMIA; INTRAHEPATIC CHOLESTASIS; LIPID METABOLISM; LIVER FUNCTION TEST; MALE; OXIDATIVE STRESS; PRESCHOOL CHILD; PRIORITY JOURNAL; PROTEIN DEFICIENCY; SCHOOL CHILD;

AMINO ACIDS; APOLIPOPROTEINS; ASIAN CONTINENTAL ANCESTRY GROUP; BIOLOGICAL MARKERS; CARBOHYDRATES; CHILD; CHILD, PRESCHOOL; CITRULLINEMIA; FASTING; FEMALE; HUMANS; HYPERCHOLESTEROLEMIA; INFANT; JAPAN; LIPID METABOLISM; LIVER; LIVER FUNCTION TESTS; MALE; MEMBRANE TRANSPORT PROTEINS; MITOCHONDRIAL PROTEINS; NITRIC OXIDE; OXIDATIVE STRESS; UREA; VITAMIN E;

EID: 63449091161     PISSN: 10967192     EISSN: 10967206     Source Type: Journal    
DOI: 10.1016/j.ymgme.2009.01.009     Document Type: Article

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