IP Library Granted Patent US 12,070,453
Granted Patent B2
US 12,070,453 · App. 16/318,905 · Granted Aug 27, 2024

Treatment of Fabry disease in ERT-naïve and ERT-experienced patients

Inventor: Jeff Castelli (New Hope, PA)
Assignee: Amicus Therapeutics, Inc.
A61K31/445A61K31/45A61K31/7008
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Quick Facts
Patent No.
US 12,070,453
App. No.
16/318,905
Granted
Aug 27, 2024
Kind
B2
Abstract

Provided are dosing regimens for the treatment of Fabry disease in a patient. Certain methods relate to the treatment of ERT-experienced or ERT-nave Fabry patients. Certain methods comprise administering to the patient about 123 mg free base equivalent of migalastat for improving left ventricular mass and/or improving podocyte globotriaosylceramide.

Claims (21)

1. A method of reducing gastrointestinal symptoms in a Fabry patient exhibiting one or more gastrointestinal symptoms, the method comprising administering to the patient a formulation comprising an effective amount of migalastat or salt thereof every other day to thereby reduce one or more of the gastrointestinal symptoms, wherein the effective amount is about 123 mg free base equivalent (FBE), wherein the gastrointestinal symptoms comprise diarrhea, wherein the patient is an enzyme replacement therapy (ERT)-naïve patient and wherein the patient has a mutation in α-Galactosidase A selected from the group consisting of A156T, D33G, G144V, and M187I.

2. The method of claim 1 , wherein administration of migalastat or a salt thereof for at least 18 months to an enzyme replacement therapy (ERT)-naïve patient having diarrhea symptoms at baseline provides a decrease in diarrhea of greater than 1.0 as assessed using the Gastrointestinal-Symptoms-Rating-Scale (GSRS).

3. The method of claim 1 , wherein the patient is administered about 123 mg of migalastat free base every other day.

4. The method of claim 1 , wherein the patient is administered about 150 mg of migalastat hydrochloride every other day.

5. The method of claim 1 , wherein the formulation comprises an oral dosage form.

6. The method of claim 5 , wherein the oral dosage form comprises a tablet, a capsule or a solution.

7. The method of claim 1 , wherein the migalastat or salt thereof is administered for at least 6 months.

8. The method of claim 1 , wherein the migalastat or salt thereof is administered for at least 18 months.

9. A method of reducing gastrointestinal symptoms in a Fabry patient exhibiting one or more gastrointestinal symptoms, the method comprising:

(i) identifying an enzyme replacement therapy (ERT)-naïve patient having Fabry disease and exhibiting diarrhea, reflux, and indigestion, and

(ii) administering to the patient migalastat or salt thereof every other day in an amount effective to reduce the diarrhea, reflux, and indigestion, wherein the effective amount is about 123 mg free base equivalent (FBE),

wherein the patient has a mutation in α-Galactosidase A selected from the group A156T, D33G, G144V, and M187I,

wherein the administration results in (a) a decrease in diarrhea of greater than 1.0 as assessed using the Gastrointestinal-Symptoms-Rating-Scale (GSRS), (b) a decrease in reflux of greater than 0.6 as assessed using the GSRS, and (c) a decrease in indigestion of greater than 0.5 as assessed using the GSRS.

10. The method of claim 9 , wherein the mutation is D33G.

11. The method of claim 9 , wherein the mutation is M187I.

12. The method of claim 1 , wherein the mutation is A156T.

13. The method of claim 1 , wherein the mutation is D33G.

14. The method of claim 1 , wherein the mutation is G144V.

15. The method of claim 1 , wherein the mutation is M187I.

16. The method of claim 9 , wherein the mutation is A156T.

17. The method of claim 9 , wherein the mutation is G144V.

Assignments (9)
SECURITY INTEREST Recorded Apr 27, 2026
From: BIOMARIN PHARMACEUTICAL INC.; AMICUS THERAPEUTICS, INC.
To: CITIBANK, N.A., AS COLLATERAL AGENT
Reel/Frame 075493/0968 →
RELEASE OF SECURITY INTEREST Recorded Apr 27, 2026
From: WILMINGTON TRUST, NATIONAL ASSOCIATION
To: AMICUS THERAPEUTICS, INC.
Reel/Frame 075494/0030 →
ASSIGNMENT OF ASSIGNOR'S INTEREST Recorded Jul 11, 2024
From: CASTELLI, JEFF
To: AMICUS THERAPEUTICS, INC.
Reel/Frame 067959/0418 →
RELEASE OF SECURITY INTEREST Recorded Oct 6, 2023
From: HAYFIN SERVICES LLP
To: AMICUS THERAPEUTICS, INC.
Reel/Frame 065164/0945 →
SECURITY INTEREST Recorded Oct 6, 2023
From: AMICUS THERAPEUTICS, INC.
To: WILMINGTON TRUST, NATIONAL ASSOCIATION
Reel/Frame 065177/0196 →
RELEASE OF SECURITY INTEREST Recorded Jul 30, 2020
From: BPCR LIMITED PARTNERSHIP
To: AMICUS THERAPEUTICS, INC.
Reel/Frame 053360/0659 →
SECURITY INTEREST Recorded Jul 30, 2020
From: AMICUS THERAPEUTICS, INC.
To: HAYFIN SERVICES LLP, AS AGENT
Reel/Frame 053365/0342 →
OMNIBUS CONFIRMATION OF ASSIGNMENT AGREEMENT Recorded May 21, 2020
From: BIOPHARMA CREDIT PLC
To: BPCR LIMITED PARTNERSHIP
Reel/Frame 052741/0173 →
SECURITY INTEREST Recorded May 11, 2020
From: AMICUS THERAPEUTICS, INC.
To: BIOPHARMA CREDIT PLC
Reel/Frame 052625/0916 →
Continuity (2)
Continuation In Part 15213920 · Jul 19, 2016
Related Publication 20190183869A1 · Jun 20, 2019