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Maple Syrup Urine Disease Mutation Explanation
This viewer helps interpret the mutation present in Maple Syrup Urine Disease. The correct amino acid for this protein at position 368 is Tyrosine, and in the affected protein it is changed to Cysteine. In the initial view, the mutant protein is shown colored and a second protein that interacts with it appears in gray.

Tyrosine vs. Cysteine

2˚ Structure

1 monomer only

1 monomer (Spacefill)

Tyr 368 Alone

Tyr 368 as Green (Spacefilled)

Neighbors Tyr 368 (light, smaller) & neighbor on the partner protein

Polymer Tyr 368 in White Within Polymer (Spacefill)

Hydrophobic [silver] vs. Hydrophilic [orchid] Residues;(ribbon; 2nd monomer NOT silver/orchid)

Hydrophobic [silver] vs. Hydrophilic Residues* (Spacefill; 2nd monomer NOT silver/orchid)

Reset to initial view

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*To discover atom/amino acid identity, hover the mouse over it. Amino acids will show up using 3-letter code (i.e. [GLY] = glycine); DNA thymine= [DT].

General Instructions: For access to JMOL commands, right click (Mac Users:Hold down control key and click) in the structure display area