共 50 条
[22]
THIAMIN-RESPONSIVE MAPLE-SYRUP-URINE DISEASE - DECREASED AFFINITY OF THE MUTANT BRANCHED-CHAIN ALPHA-KETO ACID DEHYDROGENASE FOR ALPHA-KETOISOVALERATE AND THIAMIN PYROPHOSPHATE
[J].
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA-BIOLOGICAL SCIENCES,
1982, 79 (10)
:3300-3304
[24]
Molecular basis of intermittent maple syrup urine disease: novel mutations in the E2 gene of the branched-chain α-keto acid dehydrogenase complex
[J].
Journal of Human Genetics,
1998, 43
:91-100
[29]
EFFECT OF POLYAMINES ON PHOSPHORYLATION OF BRANCHED-CHAIN ALPHA-KETO ACID DEHYDROGENASE
[J].
ALPHA-KETO ACID DEHYDROGENASE COMPLEXES : ORGANIZATION, REGULATION, AND BIOMEDICAL RAMIFICATIONS: A TRIBUTE TO LESTER J REED,
1989, 573
:397-399