ERNEST BEUTLER

enamed the Scripps Research
Institute.
At Scripps he established a
hematopoietic stem cell transplantation
program now entering its
fourth decade of service. Beutler
also continued his broad interests,
including the lysosomal storage
diseases, especially Gaucher
disease. He embraced molecular
cloning and together with Joe
Sorge, a postdoctoral fellow in his
laboratory, isolated the glucocerebrosidase
cDNA and the gene
(Sorge et al., 1985). The first effective
enzyme replacement therapy
for Gaucher disease, glucocerebrosidase
(Cerezyme ® ), was made
from the cDNA Beutler provided
the company manufacturing the
drug. He also identified several
new Gaucher disease mutations.
He showed that the dose of
glucocerebroside recommended
by the company was unnecessarily
high and unnecessarily costly.
His therapeutic approach saved
each patient so treated and his or
her insurer as much as $100,000
per year in medical costs (Beutler,
1996). He was a pioneer and an
intellectual force studying the
epidemiology, genetic basis, pathogenesis,
diagnosis, and treatment of
Gaucher and, arguably, the leading
authority on the disease (Beutler,
2006c; Beutler and Grabowski,
2000). He published over 100
scientific papers on some aspect of
the disorder.
When Beutler was at City
of Hope he amassed a large
array of references that he used
in the preparation of papers
and book chapters. He applied
a manual card-sorting device for
reclaiming references he needed,
and had a programmer develop
a technique that he could apply
using a PDP-11/40 computer to
permit him to recover references
by keyword or author. Indeed, he
Ernest Beutler
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