Truncated VWF Polypeptides for Factor VIII Stabilization

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Solution Overview

Problem

Current treatments for bleeding disorders, such as hemophilia A and von Willebrand disease, face challenges in maintaining the stability and half-life of Factor VIII due to its rapid clearance from plasma, particularly in conditions where von Willebrand Factor (VWF) is deficient or defective.

Innovation Solution

Development of modified truncated von Willebrand Factor polypeptides with enhanced binding affinity to Factor VIII, specifically through modifications in the D' and D3 domains, which form a complex to stabilize and prolong the half-life of Factor VIII in the plasma.

Engineering Contradictions & Design Principles

VSEngineering Contradiction Analysis

1Duration of action of stationary object

If truncated VWF polypeptides are used to bind Factor VIII, then the half-life of Factor VIII is prolonged, but the binding affinity is insufficient compared to full-length VWF

Engineering Contradiction:
Improvehalf-life of Factor VIIIVSAvoidbinding affinity to Factor VIII
Core Design Contradiction:
Duration of action of stationary objectVSReliability

Solution Approach 1:

The invention divides the full-length VWF into truncated polypeptides containing specific domains (D', D3, A1, A2, A3, C1, C2, CK) that retain FVIII binding capability. By segmenting VWF into functional domains, the patent achieves prolonged FVIII half-life while maintaining sufficient binding affinity through optimized domain composition.

Inventive Principle:
Principle #1Segmentation

Solution Approach 2:

The invention modifies the truncated VWF polypeptides through various parameters including amino acid substitutions (e.g., S764A, S764G, S766Y, V1083A), domain combinations, and molecular weight variations to enhance binding affinity to Factor VIII while maintaining the prolonged half-life effect.

Inventive Principle:
Principle #35Parameter changes

2Reliability

If full-length VWF is used to stabilize Factor VIII, then binding affinity is high, but the complexity of the molecule increases production and purification difficulties

Engineering Contradiction:
Improvebinding affinity to Factor VIIIVSAvoidmolecular complexity of VWF
Core Design Contradiction:
ReliabilityVSDevice complexity

Solution Approach 1:

The invention extracts only the essential domains of VWF (truncated versions containing D', D3, A1, A2, A3, C1, C2, CK domains) that are necessary for FVIII binding and stabilization. This extraction removes unnecessary portions of the full-length VWF, reducing molecular complexity while preserving the critical binding function.

Inventive Principle:
Principle #2Taking out (Extraction)

Solution Approach 2:

By segmenting full-length VWF into truncated polypeptides with specific domain compositions, the invention simplifies the molecular structure for easier production and purification while maintaining the essential FVIII binding capability through retained critical domains.

Inventive Principle:
Principle #1Segmentation

3Speed

If conventional FVIII replacement therapy is used, then immediate therapeutic effect is achieved, but frequent administration is required due to rapid clearance

Engineering Contradiction:
Improveonset of therapeutic effectVSAvoidhalf-life of Factor VIII
Core Design Contradiction:
SpeedVSDuration of action of stationary object

Solution Approach 1:

The invention uses truncated VWF polypeptides as intermediary carrier molecules that bind to Factor VIII. This VWF-FVIII complex allows immediate therapeutic effect upon administration while the VWF component protects FVIII from rapid clearance, thereby extending its half-life and reducing administration frequency.

Inventive Principle:
Principle #24Intermediary (Mediator)

Data Source

PatentEP3400002B1Mutated truncated von willebrand factor
Publication Date: 2022.02.02 CSL BEHRING LENGNAU AG
  • EP3400002B1 patent drawingFigure 1
  • EP3400002B1 patent drawingFigure 2a~2d
  • EP3400002B1 patent drawingFigure 3a~3d

AI summary

The present invention provides a modified polypeptide which binds Factor VIII. The polypeptide comprises truncated von Willebrand Factor (VWF) which comprises a sequence as shown in SEQ ID NO:3 or a fragment thereof or a sequence 90% identical thereto, wherein the truncated VWF comprises at least one modification in comparison to SEQ ID NO: 3 in at least one position selected from the group consisting of SI, S3, LI 8, V42, S43, K149, N248, S279, V320, T325, Q395 and K418.