The main shareholders of Orphazyme A/S are currently:

• MH Investment ApS (10.00% to 14.99%)
• Scandinavian Investment Group A/S (10.00% to 14.99%)
• Nordic Compound Invest A/S (5.00% to 9.99%)
• LSP V Cooperatieve U.A. A.(5.00% to 9.99%)

Based on the latest major shareholder notification received by Orphazyme.