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Literature summary for 1.2.4.4 extracted from

  • Aevarsson, A.; Chuang, J.L.; Wynn, R.M.; Turley, S.; Chuang, D.T.; Hol, W.G.J.
    Crystal structure of human branched-chain alpha-ketoacid dehydrogenase and the molecular basis of multienzyme complex deficiency in maple syrup urine disease (2000), Structure, 8, 277-291.
    View publication on PubMed

Activating Compound

Activating Compound Comment Organism Structure
thiamine diphosphate
-
Homo sapiens

Cloned(Commentary)

Cloned (Comment) Organism
-
Homo sapiens

Crystallization (Commentary)

Crystallization (Comment) Organism
-
Homo sapiens

Protein Variants

Protein Variants Comment Organism
additional information missense mutation in E1b subunits that cause maple syrup urine disease: R114W-alpha, T166M-alpha, R220W-alpha. N222S-alpha. M64T-alpha. G204S-alpha. A208T-alpha. T265R-alpha, I281T-alpha, N126Y-beta, Q145K-alpha, H156R-beta, A209D-alpha, A240P-alpha. G245R-alpha, R252H-alpha, F364C-alpha, Y368C-alpha and Y393N-alpha Homo sapiens

Organism

Organism UniProt Comment Textmining
Homo sapiens
-
-
-

Source Tissue

Source Tissue Comment Organism Textmining

Cofactor

Cofactor Comment Organism Structure
CoA
-
Homo sapiens
NAD+
-
Homo sapiens