Straight-chain acyl-CoA oxidase knockout mouse accumulates extremely long chain fatty acids from α-linolenic acid: Evidence for runaway carousel-type enzyme kinetics in peroxisomal β-oxidation diseases

Molecular Genetics and Metabolism

Volumn 75, Issue 2, 2002, Pages 108-119

  Infante, Juan P ^a   Tschanz, Carolyn L ^b   Shaw, Natacha ^b   Michaud, Anthony L ^b   Lawrence, Peter ^b   Brenna, J Thomas ^b  

^a Institute for Theoretical Biochemistry and Molecular Biology   (United States)

^b Department of Obstetrics Gynecology   (United States)

Author keywords

Carnitine; Crypthecodinium cohnii; Desaturase; Mitochondria; mtDHAS; Multifunctional mitochondrial DHA synthase; Peroxisomes; PPAR

Indexed keywords

ACYL COENZYME A OXIDASE; DOCOSAHEXAENOIC ACID; LINOLENIC ACID; LONG CHAIN FATTY ACID; POLYUNSATURATED FATTY ACID; SYNTHETASE;

ADRENOLEUKODYSTROPHY; ANIMAL EXPERIMENT; ANIMAL MODEL; ANIMAL TISSUE; ARTICLE; BRAIN; CARNITINE DEFICIENCY; CATALYSIS; CONTROLLED STUDY; DISORDERS OF PEROXISOMAL FUNCTIONS; ENDOPLASMIC RETICULUM; ENZYME KINETICS; FATTY ACID METABOLISM; FATTY ACID OXIDATION; FATTY ACID SYNTHESIS; KNOCKOUT MOUSE; LIPID LIVER LEVEL; LIVER; NONHUMAN; PRIORITY JOURNAL; ZELLWEGER SYNDROME;

CRYPTHECODINIUM; CRYPTHECODINIUM COHNII;

EID: 0036354354     PISSN: 10967192     EISSN: None     Source Type: Journal    
DOI: 10.1006/mgme.2001.3279     Document Type: Article

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