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6 in total

1. Sequence of the E1 alpha subunit of branched-chain alpha-ketoacid dehydrogenase in two patients with thiamine-responsive maple syrup urine disease.

Authors: B Zhang; R S Wappner; I K Brandt; R A Harris; D W Crabb
Journal: Am J Hum Genet Date: 1990-04 Impact factor: 11.025

2. Direct physical evidence for stabilization of branched-chain alpha-ketoacid dehydrogenase by thiamin pyrophosphate.

Authors: S C Heffelfinger; E T Sewell; L J Elsas; D J Danner
Journal: Am J Hum Genet Date: 1984-07 Impact factor: 11.025

3. N-carbamylglutamate markedly enhances ureagenesis in N-acetylglutamate deficiency and propionic acidemia as measured by isotopic incorporation and blood biomarkers.

Authors: Mendel Tuchman; Ljubica Caldovic; Yevgeny Daikhin; Oksana Horyn; Ilana Nissim; Itzhak Nissim; Mark Korson; Barbara Burton; Marc Yudkoff
Journal: Pediatr Res Date: 2008-08 Impact factor: 3.756

4. Absence of branched chain acyl-transferase as a cause of maple syrup urine disease.

Authors: D J Danner; N Armstrong; S C Heffelfinger; E T Sewell; J H Priest; L J Elsas
Journal: J Clin Invest Date: 1985-03 Impact factor: 14.808

Review 5. Thiamine-responsive inborn errors of metabolism.

Authors: M Duran; S K Wadman
Journal: J Inherit Metab Dis Date: 1985 Impact factor: 4.982

6. A review of the biochemistry, metabolism and clinical benefits of thiamin(e) and its derivatives.

Authors: Derrick Lonsdale
Journal: Evid Based Complement Alternat Med Date: 2006-03 Impact factor: 2.629

6 in total