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First In class oliGo THerapy for Myotonic Dystrophy type 1 (DM1)

Project description

A novel molecular therapy against myotonic dystrophy

Myotonic dystrophy type 1 is an inherited muscular dystrophy that is characterised by muscle weakness and wasting as well as prolonged muscle tensing. Recent evidence indicates altered microRNA expression such as upregulation of the microRNA-23b which plays a key role in the translation of muscle-related proteins. Funded by the European Innovation Council, the fight-dm1 project will bring forward a novel molecular therapy based on synthetic single-stranded oligonucleotides called antimiRs. These molecules have been designed to be complementary to that of microRNA-23b so that they can block its function or degrade it. Following promising preclinical efficacy, the patented technology will be validated in a patient study.

Objective

Myotonic dystrophy type 1 (DM1) is a clinically and genetically heterogeneous disorder with more than 1 million diagnosed patients worldwide, making it the most common adult muscular dystrophy. Available treatments only relief symptoms. Given this need, we offer ATX-01, a first in class miRNA therapy that inhibits miR-23b without changing the DNA, which rescues DM1 pathogenic mis-splicing. We have demonstrated improved target engagement effects in skeletal and respiratory muscle in in-vitro and in-vivo studies, granting us an FDA pre-IND approval and orphan drug designation. Our approach, antimiRs conjugated to fatty acids, constitutes the ENTRY platform, which guarantees efficient drug delivery to target muscular and extra-muscular tissue at low doses. This patented technology will be validated through a Phase I/IIa clinical trial and chronic preclinical studies, to then be extended to applications as other myotonic dystrophies, Fuchs dystrophy, cachexia, among other opportunities.

Fields of science (EuroSciVoc)

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Keywords

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Programme(s)

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Topic(s)

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Funding Scheme

Funding scheme (or “Type of Action”) inside a programme with common features. It specifies: the scope of what is funded; the reimbursement rate; specific evaluation criteria to qualify for funding; and the use of simplified forms of costs like lump sums.

HORIZON-EIC-ACC-BF - HORIZON EIC Accelerator Blended Finance

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Call for proposal

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(opens in new window) HORIZON-EIC-2022-ACCELERATOR-01

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Coordinator

ARTHEX BIOTECH SL
Net EU contribution

Net EU financial contribution. The sum of money that the participant receives, deducted by the EU contribution to its linked third party. It considers the distribution of the EU financial contribution between direct beneficiaries of the project and other types of participants, like third-party participants.

€ 2 500 000,00
Address
CALLE CATEDRATICO AGUSTIN ESCARDINO
46980 VALENCIA
Spain

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SME

The organization defined itself as SME (small and medium-sized enterprise) at the time the Grant Agreement was signed.

Yes
Region
Este Comunitat Valenciana Valencia/València
Activity type
Private for-profit entities (excluding Higher or Secondary Education Establishments)
Links
Total cost

The total costs incurred by this organisation to participate in the project, including direct and indirect costs. This amount is a subset of the overall project budget.

€ 20 074 282,53
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