Thiamine pyrophosphate: An essential cofactor in the mammalian metabolism of 3-methyl-branched fatty acids - Implications for thiamine deficiencies?

Advances in Experimental Medicine and Biology

Volumn 544, Issue , 2003, Pages 305-306

  Foulon, Veerle ^a   Casteels, Minne ^a   Mannaerts, Guy P ^a   Gelb, Bruce D ^b   Van Veldhoven, Paul P ^a  

^a UNIVERSITY HOSPITALS LEUVEN   (Belgium)

^b MOUNT SINAI SCHOOL OF MEDICINE   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

COCARBOXYLASE; FATTY ACID DERIVATIVE; OXYTHIAMINE; PHYTANIC ACID; PYRITHIAMINE;

BIOACCUMULATION; CONCENTRATION RESPONSE; CONFERENCE PAPER; CULTURE MEDIUM; FIBROBLAST; GLIA CELL; HUMAN; LIVER CELL; MEGALOBLASTIC ANEMIA; METABOLISM; NONHUMAN; OLIGOMERIZATION; OXIDATION; PRIORITY JOURNAL; PROTEIN ANALYSIS; PROTEIN DEGRADATION; REDUCTION; THIAMINE DEFICIENCY;

MAMMALIA;

EID: 1542269181     PISSN: 00652598     EISSN: None     Source Type: Book Series    
DOI: 10.1007/978-1-4419-9072-3_39     Document Type: Conference Paper

References (2)

1. Foulon, V., Antonenkov, V.D., Croes, K., Waelkens, E., Mannaerts, G.P., Van Veldhoven, P.P. & Casteels, M., 1999, Purification, molecular cloning, and expression of 2-hydroxyphytanoyl-CoA lyase, a peroxisomal thiamine pyrophosphate-dependent enzyme that catalyzes the carbon-carbon bond cleavage during alpha-oxidation of 3-methyl-branched fatty acids. Proc. Natl. Acad. Sci. U.S.A. 96: 10039-10044.
2. Diaz, G.A., Banikazermi, M., Oishi, K., Desnick, R.J., Gelb, B.D., 1999, Mutations in a new gene encoding a thiamine transporter cause thiamine-responsive megaloblastic anaemia syndrome. Nat. Genet. 22(3): 309-312.