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Wednesday, May 29, 2019

Amicus, Penn expand gene therapy research agreement to five years

Apart from get­ting the FDA to have a change of heart on its Fabry dis­ease ther­apy, Galafold, last year — Am­i­cus Ther­a­peu­tics joined forces with the Uni­ver­sity of Penn­syl­va­nia to work on gene ther­a­pies for four dif­fer­ent dis­or­ders — Pompe dis­ease, Fabry dis­ease, CDKL5 de­fi­ciency and an undis­closed rare meta­bolic dis­or­der. On Wednes­day, the two ex­panded their col­lab­o­ra­tion — to in­clude ad­di­tional lyso­so­mal dis­or­ders and rare dis­eases.

The part­ner­ship was inked to bring to­gether Penn’s AAV vec­tor tech­nol­ogy (AAVs are small, non-​pathogenic viruses with a genome of single-​stranded DNA, which can in­sert ge­netic ma­te­r­ial at a spe­cific site), which is en­gi­neered to en­hance tar­get­ing, safety and gene de­liv­ery — with Am­i­cus’ $FOLD tech­nol­ogy de­signed to op­ti­mize pro­tein ex­pres­sion, se­cre­tion, and the up­take of the tar­get pro­tein.
Un­der the new five-​year agree­ment, the two en­ti­ties will also work to­gether on gene ther­a­pies for Niemann-​Pick Type C (NPC) and two forms of San­fil­ippo Syn­drome. In ad­di­tion, Am­i­cus has the op­por­tu­nity to col­lab­o­rate with Penn’s Gene Ther­apy Pro­gram (GTP) to de­velop pro­grams for the ma­jor­ity of lyso­so­mal dis­or­ders and twelve ad­di­tional rare dis­eases, in­clud­ing Rett Syn­drome, An­gel­man Syn­drome, My­otonic Dy­s­tro­phy and se­lect other mus­cu­lar dy­s­tro­phies. Am­i­cus will in­ject $10 mil­lion an­nu­ally for five years, into GTP’s dis­cov­ery re­search pro­gram —  with the abil­ity to ex­tend.

Am­i­cus’ ex­per­i­men­tal drug for Pompe dis­ease (an in­her­ited lyso­so­mal stor­age dis­or­der), AT-​GAA, did not gen­er­ate much en­thu­si­asm for ac­cel­er­ated re­view by the FDA, de­spite CEO John Crow­ley’s best ef­forts last Sep­tem­ber.
The com­pany’s work with Penn on Pompe dis­ease, mean­while, has gen­er­ated pos­i­tive data.
“We have al­ready seen highly en­cour­ag­ing pre­clin­i­cal re­sults and proof-​of-concept in Pompe dis­ease through our ex­ist­ing col­lab­o­ra­tion…and I be­lieve that we can fur­ther ex­pand and ac­cel­er­ate our ef­forts to rapidly de­velop gene ther­a­pies for many more pa­tients…” said James Wil­son, pro­fes­sor of med­i­cine and pe­di­atrics at Penn’s Perel­man School of Med­i­cine, in a state­ment.

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