SMART Teams 2015-2016
Qualification Phase
Brown Deer High School SMART Team
Evan Bruss, T.J. Davis, Jack Hermsen, Justin Johnson, Robert Laughlin, Maurice Lucré,
Chad Marable, Brett Poniewaz, Virginia Tuncel, Gina Wade, Michael Weeden
Teacher: Mr. David Sampe
Arylsulfatase A (ASA): A Sulfatide Accumulator
PDB: 1E1Z
Primary Citation: Von Bülow Rixa, Schmidt Bernhard, Dierks Thomas, Von Figura Kurt, Usón Isabel (2001).
Crystal Structure of an Enzyme-Substrate Complex Provides Insight into the Interaction between Human
Arylsulfatase A and its Substrates During Catalysis. Journal of Molecular Biology 305: 269-277.
Format: Alpha carbon backbone
RP: Zcorp with plaster
Description:
Metachromatic leukodystrophy (MLD) is an autosomal
recessive genetic disorder that affects 1 in 40,000 to
160,000 individuals worldwide. This disorder drastically
shortens the lifespan of people afflicted. Patients
experience symptoms that include motor function
degeneration, cognitive difficulties and behavior
problems. MLD is caused by a mutation in the ARSA
gene. Normally, ARSA codes for arylsulfatase A (ASA),
an enzyme in lysosomes, which hydrolyzes sulfatides
by breaking the ester bond between the sulfate group
and the fatty acids. When ARSA is mutated, regular
sulfatide breakdown cannot occur. The hydrolysis
occurs in cells that produce myelin, (white matter
normally surrounding neurons). Sulfatide accumulation
in myelin producing cells results in cell death.
Ordinarily, myelin provides insulation that allows an
action potential to jump along an axon, making it travel faster. When myelin is absent, the speed of impulse
transmission decreases. Researchers are trying to determine the mechanism by which the amino acids
lys123, lys302, ser150, his229, cys69 bind to sulfatides in the active site. Then, a treatment could be
developed to counteract the deadly effects of this tragic disorder in patients. The Brown Deer High School
SMART (Students Modeling A Research Topic) Team designed a model of ASA using 3D printing technology
to help illustrate the ASA active site.
Specific Model Information:
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Protein Backbone is colored white.
Alpha Helices are colored cornflower blue.
Beta Sheets are colored indian red.
Disulfide Bonds are colored yellow.
Hydrogen Bonds are colored lemon chiffon.
Amino acid side chains involved in the sulfatide binding site displayed in spacefill.
o Serine69 is colored lime.
o Lysine123 is colored lime.
o Serine150 is colored lime.
o Histidine229 is colored lime.
o Lysine302 is colored lime.
Structural supports are colored light cyan.
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