CRISPR-Cas9 Editing of DMD Gene Exons for Muscular Dystrophy

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Solution Overview

Problem

Current gene therapy approaches for Duchenne muscular dystrophy (DMD), Becker muscular dystrophy (BMD), and dilated cardiomyopathy type 3B face challenges due to the large size of the DMD gene, with delivery and long-term efficacy issues, requiring frequent intramuscular injections and compromising therapy effectiveness.

Innovation Solution

The use of CRISPR-Cas9 mediated genome-editing systems to introduce breaks in the DMD gene, allowing for break-induced deletions or indels, thereby altering the dystrophin sequence to restore a correct reading frame, using gRNAs to target specific positions and Cas9 nucleases to create single or double strand breaks, avoiding unwanted chromosome elements.

Engineering Contradictions & Design Principles

VSEngineering Contradiction Analysis

1Reliability

If current gene therapy approaches are used to treat DMD, then the DMD gene can be targeted, but the large size of the DMD gene causes delivery issues and requires frequent intramuscular injections

Engineering Contradiction:
Improvetherapy effectivenessVSAvoidfrequency of administration
Core Design Contradiction:
ReliabilityVSEase of operation

Solution Approach 1:

The patent extracts and targets only the specific mutated exon region of the DMD gene using CRISPR-Cas9, rather than attempting to deliver or edit the entire 2.2 megabase gene. This extraction of the critical target region enables effective treatment without the delivery limitations imposed by the gene's full size, reducing the need for frequent administrations.

Inventive Principle:
Principle #2Taking out (Extraction)

2Reliability

If current gene therapy approaches are used for DMD, then treatment can be provided, but delivery issues arise due to the large size of the DMD gene

Engineering Contradiction:
Improvetreatment efficacyVSAvoiddelivery system complexity
Core Design Contradiction:
ReliabilityVSDevice complexity

Solution Approach 1:

The invention extracts the essential therapeutic function to targeting and editing only the specific mutated exon sequence within the DMD gene. This reduces the complexity of the delivery system by eliminating the need to deliver the entire large gene, focusing instead on delivering only the CRISPR-Cas9 components needed to address the specific mutation.

Inventive Principle:
Principle #2Taking out (Extraction)

Solution Approach 2:

The patent segments the DMD gene editing task into targeting only the specific mutated exon region rather than the entire gene. This segmentation allows the use of smaller, more manageable CRISPR-Cas9 delivery systems that can effectively reach and edit the specific target without the complexity of delivering the full 2.2 megabase gene.

Inventive Principle:
Principle #1Segmentation

3Duration of action of stationary object

If frequent intramuscular injections are administered, then therapy can be maintained, but long-term efficacy is compromised

Engineering Contradiction:
Improvetherapy durationVSAvoidlong-term efficacy
Core Design Contradiction:
Duration of action of stationary objectVSReliability

Solution Approach 1:

The CRISPR-Cas9 system performs preliminary genetic editing to correct the mutated exon sequence, establishing a permanent fix that eliminates the need for repeated treatments. This preliminary correction action addresses the root cause of the disease at the genetic level, providing long-lasting efficacy without requiring frequent maintenance injections.

Inventive Principle:
Principle #10Preliminary action

Solution Approach 2:

The genetic correction achieved through CRISPR-Cas9 editing enables the cell's own machinery to produce functional dystrophin protein from the corrected gene sequence. This self-service mechanism allows the body to continuously produce the necessary protein without external intervention, maintaining long-term efficacy without frequent administrations.

Inventive Principle:
Principle #25Self-service

Applied Scientific Principles

This section explains which scientific principles are used to turn an abstract innovation direction into a practical engineering solution.

Function Achieved in This Case

This approach potentially delays the onset or progression of these diseases by altering the DMD gene, providing a more effective and sustainable treatment option compared to existing therapies, with the potential for reduced frequency of administration and improved long-term efficacy.

Implementation Method 1

The use of CRISPR-Cas9 mediated genome-editing systems to introduce breaks in the DMD gene, allowing for break-induced deletions or indels, thereby altering the dystrophin sequence to restore a correct reading frame

Methodology Applied
Scientific EffectCRISPR-Cas9 mediated genome editing:

Data Source

PatentEP3748004A1Crispr/CAS-related methods and compositions for treating duchenne muscular dystrophy and becker muscular dystrophy
Publication Date: 2020.12.09 EDITAS MEDICINE INC
  • EP3748004A1 patent drawingFigure 1A
  • EP3748004A1 patent drawingFigure 1B
  • EP3748004A1 patent drawingFigure 1C

AI summary

CRISPR/CAS-related compositions and methods for treatment of DMD, BMD, or DCM type 3B are described.