Major Shareholder Requirements

According to the EU Transparency Directive implemented in Denmark as of 1 June 2007 shareholders are obliged to notify both Orphazyme and the Danish Financial Supervisory Authority when the voting rights conferred on the shares or the nominal value of the shares held by the shareholder exceeds or falls below the thresholds of 5%, 10%, 15%, 20%, 25%, 1/3, 50%, 2/3, 90% or 100%. The voting rights shall be calculated on the basis of all the shares to which voting rights are attached even if the exercise thereof is suspended.

The notification shall as a minimum include the following information:

(a) the number of voting rights conferred and/or share capital nominal value held and direct or indirect ownership;

(b) the date on which the threshold was reached, exceeded or no longer reached;

(c) the identity of the shareholder; and

(d) the chain of controlled undertakings through which voting rights and/or share capital are effectively held, if applicable.

The notification shall be effected as soon as possible, to be understood as the same day on which the threshold was reached, exceeded or no longer reached.

The notification to Orphazyme shall be made by post and email to the responsible of Investor Relations. At the same time as the notification to Orphazyme, a notification to the Danish Financial Supervisory Authority shall be made electronically and using a special form which is available on the Danish Financial Supervisory Authority’s webpage.

We encourage major shareholders to notify us about major shareholder transactions via email to
majorshareholder@orphazyme.com.