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Hypothetical maple Syrup Urine Mutation
Maple Syrup Urine Disease: Mutation Explanation This viewer helps interpret the mutation present in Maple Syrup Urine Disease. Consider a change in which the correct amino acid, phenylalanine, at position 207 is changed to serine. In the initial view, the mutant protein is shown colored and a second, different protein that interacts with it appears in gray.

Phenylalanine vs. Serine



2˚ Structure

One monomer only

1 monomer (Spacefill)

Phe 207 Alone

Phe 207, golden (Spacefilled)

Neighbors Phe 207 (golden) with near amino acid neighbors on neighbor protein (sidechain carbons light geen; labels begin with 'B:'

Neighbors Phe 207 (golden) & near amino acid neighbors on neighbor protein (sidechain carbons light green; labels begin with 'B:')

Neighbors Center Phe 207 (golden, medium sized); show near amino acid neighbors on neighbor protein (sidechain carbons light green; labels begin with 'B:')

Hydrophobic [silver] vs. Hydrophilic [orchid] Residues (Ribbon)

Hydrophobic [silver] vs. Hydrophilic Residues+ (Spacefill w/Tyr 368 selected)

Reset      Slice it!

*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