S. Ushikubo

Purification of human very-long-chain acyl-coenzyme A dehydrogenase and characterization of its deficiency in seven patients.

Toshifumi Aoyama, Masayoshi Souri, S. Ushikubo et al. · Journal of Clinical Investigation · 1995 · 161 citations · Full text

Molecular Biology, Pathology, Vlcad Deficiency +16

Molecular basis of long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency: identification of the major disease-causing mutation in the α-subunit of the mitochondrial trifunctional protein

Lodewijk IJlst, R. J. A. Wanders, S. Ushikubo et al. · Biochimica et Biophysica Acta (BBA) - Lipids and Lipid Metabolism · 1994 · 158 citations

Mitochondrial Biogenesis, Biochemistry, Mitochondrial Function +15

Molecular characterization of mitochondrial trifunctional protein deficiency: formation of the enzyme complex is important for stabilization of both alpha- and beta-subunits.

S. Ushikubo, Toshifumi Aoyama, Takehiko Kamijo et al. · PubMed · 1996 · 104 citations

Peroxisomal Acyl-Coenzyme A Oxidase Is a Rate-Limiting Enzyme in a Very Long-Chain Fatty Acid β-Oxidation System

Toshifumi Aoyama, Masayoshi Souri, Takehiko Kamijo et al. · Biochemical and Biophysical Research Communications · 1994 · 33 citations

Biosynthesis, Biochemistry, Rate-limiting Enzyme +5