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TSE: the disease group
The History of TSE (Prion Diseases)
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Table
2. Compounds that have been
studied using test tube, tissue culture, or animal assays.
First
group acts outside the brain; second group acts both inside and outside the
brain.
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Studied
compound
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Effect
on test tube conversion (precursor to PRP)
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Effect on PrP in tissue culture
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Effect on
treated rodents
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Comment
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Polyanions
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Stimulate
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Inhibit
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Prolong incubation
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Pentosan used in
humans (other purposes)
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Sulfonated dyes
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Inhibit
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Inhibit
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Prolong
incubation
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Bind to both
precursor and PrP
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Tetrapyrroles
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Inhibit
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Inhibit
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Prolong incubation
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Used in humans for
other purposes.
|
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Anthracyclines
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Not tested
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Not tested
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Prolong incubation1
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Iododoxyrubicin (IDX)
used successfully in humans with other types of amyloidoses
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Lymphotoxin ß-
receptor-blocker
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Not tested
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Not tested
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Prolongs incubation
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Blocks replication in
spleen cells (follicular dendritic cells)
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Polyene antibiotics
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Inhibit
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Not tested
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Prolong incubation
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Amphotericin B
congener effective when begun long after infection
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Branched polyamines
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Not tested
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Eliminate
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No disease
transmission2
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Disaggregate prion
fibrils, reduce ß-sheet content of
PrP
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Acridine derivatives
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No effect
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Eliminate
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Not tested
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Quinacrine under
study in CJD patients
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Phenothiazines
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Not tested
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Eliminate
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Prolong
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Structurally similar
to acridine drugs
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Cysteine protease
inhibitors
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No effect
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Eliminate
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Not tested
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Bind to PrP
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Anti-PrP antibodies
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Inhibit
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Eliminate
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No disease
transmission2
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Bind to precursor of
PrP on cell surface
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ß-breaker peptides
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Not tested
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Not tested
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Prolong incubation1
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Used in humans for
other purposes
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Synthetic PrP
peptides
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Inhibit
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Inhibit
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Not tested
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Block binding of
PrPsen to PrPres
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1Scrapie-infected
brain homogenate was mixed with drug and then inoculated into animals.
2Cells
were treated and then inoculated into animals.
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