2Q19 Galafold® (migalastat) Revenue of $44.1M Represents Highest Quarterly 
Growth Since Launch Driven by Continued Strong Global Uptake 

Reiterating FY19 Revenue Guidance of $160M-$180M

Full Enrollment in Pompe Pivotal Study of AT-GAA on Track by YE19

Significant Progress Across Industry Leading Rare Disease Gene Therapy Portfolio

Strong Balance Sheet with $575M+ Cash

Conference Call and Webcast Today at 8:30 a.m. ET

CRANBURY, N.J., Aug. 08, 2019 (GLOBE NEWSWIRE) --  Amicus Therapeutics (Nasdaq: FOLD), a global biotechnology company focused on discovering, developing and delivering novel medicines for rare metabolic diseases, today announced financial results for the second quarter ended June 30, 2019. The Company also summarized recent program updates and reiterated its full-year 2019 guidance.

John F. Crowley, Chairman and Chief Executive Officer of Amicus Therapeutics, Inc. stated, “2019 has been a great period of growth and achievement at Amicus as we continue to build a leading global rare disease biotechnology company. We are on track to meet or exceed all of our key strategic priorities for the year - continued success for Galafold, tremendous momentum in our Phase 3 Pompe clinical study, positive interim Phase 1/2 clinical data for our first gene therapy for CLN6 Batten disease, and an industry leading rare disease gene therapy portfolio. We are in a very strong position to deliver on our mission for patients and shareholders.”

Corporate Highlights for 2Q19 and Early 3Q19

2Q19 Financial Results

2019 Key Strategic Priorities

2019 Financial Guidance

For the full-year 2019 the Company anticipates total Galafold revenue of $160 million to $180 million. Growth in 2019 is expected to be driven by continued growth in EU markets, further geographic expansion, and successful adoption in the first full year of launch in the U.S. and Japan. For the remainder of the year, consistent with Galafold adoption trends and ordering patterns seen in previous years, Amicus expects flat to moderate revenue growth in the third quarter and higher revenue growth in the fourth quarter of 2019.

Cash, cash equivalents, and marketable securities totaled $575.7 million at June 30, 2019. The Company expects to end 2019 with approximately $400 million in cash on hand. The current cash position is anticipated to fund ongoing operations into 2021.

Anticipated 2019 Milestones by Program

Galafold Oral Precision Medicine for Fabry Disease

AT-GAA for Pompe Disease

Gene Therapy Pipeline:

Conference Call and Webcast
Amicus Therapeutics will host a conference call and audio webcast today, August 8, 2019 at 8:30 a.m. ET to discuss the second quarter 2019 financial results and corporate updates. Interested participants and investors may access the conference call by dialing 877-303-5859 (U.S./Canada) or 678-224-7784 (international), conference ID: 8871329.

A live audio webcast can also be accessed via the Investors section of the Amicus Therapeutics corporate website at http://ir.amicusrx.com/, and will be archived for 30 days. Web participants are encouraged to register on the website 15 minutes prior to the start of the call. A replay of the call will be available for seven days beginning at 11:30 a.m. ET on August 8, 2019. Access numbers for this replay are 855-859-2056 (U.S./Canada) and 404-537-3406 (international); conference ID: 8871329.

About Galafold 
Galafold® (migalastat) 123 mg capsules is an oral pharmacological chaperone of alpha-Galactosidase A (alpha-Gal A) for the treatment of Fabry disease in adults who have amenable GLA variants. In these patients, Galafold works by stabilizing the body’s own dysfunctional enzyme so that it can clear the accumulation of disease substrate. Globally, Amicus Therapeutics estimates that approximately 35 to 50 percent of Fabry patients may have amenable GLA variants, though amenability rates within this range vary by geography. Galafold is approved in Argentina, Australia, Canada, European Union, Israel, Japan, South Korea, Switzerland and the U.S.

U. S. INDICATIONS AND USAGE
Galafold is indicated for the treatment of adults with a confirmed diagnosis of Fabry disease and an amenable galactosidase alpha gene (GLA) variant based on in vitro assay data.

This indication is approved under accelerated approval based on reduction in kidney interstitial capillary cell globotriaosylceramide (KIC GL-3) substrate. Continued approval for this indication may be contingent upon verification and description of clinical benefit in confirmatory trials.

U.S. IMPORTANT SAFETY INFORMATION

ADVERSE REACTIONS
The most common adverse reactions reported with Galafold (≥10%) were headache, nasopharyngitis, urinary tract infection, nausea and pyrexia.

USE IN SPECIFIC POPULATIONS
There is insufficient clinical data on Galafold use in pregnant women to inform a drug-associated risk for major birth defects and miscarriage. Advise women of the potential risk to a fetus.

It is not known if Galafold is present in human milk. Therefore, the developmental and health benefits of breastfeeding should be considered along with the mother’s clinical need for Galafold and any potential adverse effects on the breastfed child from Galafold or from the underlying maternal condition.

Galafold is not recommended for use in patients with severe renal impairment or end-stage renal disease requiring dialysis.

The safety and effectiveness of Galafold have not been established in pediatric patients.

To report Suspected Adverse Reactions, contact Amicus Therapeutics at 1-877-4AMICUS or FDA at 1-800-FDA-1088 or www.fda.gov/medwatch.

For additional information about Galafold, including the full U.S. Prescribing Information, please visit https://www.amicusrx.com/pi/Galafold.pdf.

EU Important Safety Information
Treatment with Galafold should be initiated and supervised by specialists experienced in the diagnosis and treatment of Fabry disease. Galafold is not recommended for use in patients with a nonamenable mutation.

For further important safety information for Galafold, including posology and method of administration, special warnings, drug interactions and adverse drug reactions, please see the European SmPC for Galafold available from the EMA website at www.ema.europa.eu.

About Amicus Therapeutics
Amicus Therapeutics (Nasdaq: FOLD) is a global, patient-dedicated biotechnology company focused on discovering, developing and delivering novel high-quality medicines for people living with rare metabolic diseases. With extraordinary patient focus, Amicus Therapeutics is committed to advancing and expanding a robust pipeline of cutting-edge, first- or best-in-class medicines for rare metabolic diseases. For more information please visit the company’s website at www.amicusrx.com, and follow on Twitter and LinkedIn.

Non-GAAP Financial Measures
Operational revenue growth excluding the impact of foreign currency exchange rates is a non-GAAP financial measure and should not be considered a replacement for, and should be read together with, the most comparable GAAP financial measure. 

Forward-Looking Statements
This press release contains "forward-looking statements" within the meaning of the Private Securities Litigation Reform Act of 1995 relating to preclinical and clinical development of our product candidates, the timing and reporting of results from preclinical studies and clinical trials, the prospects and timing of the potential regulatory approval of our product candidates, commercialization plans, manufacturing and supply plans, financing plans, and the projected revenues and cash position for the Company. The inclusion of forward-looking statements should not be regarded as a representation by us that any of our plans will be achieved. Any or all of the forward-looking statements in this press release may turn out to be wrong and can be affected by inaccurate assumptions we might make or by known or unknown risks and uncertainties. For example, with respect to statements regarding the goals, progress, timing, and outcomes of discussions with regulatory authorities, and in particular the potential goals, progress, timing, and results of preclinical studies and clinical trials, actual results may differ materially from those set forth in this release due to the risks and uncertainties inherent in our business, including, without limitation: the potential that results of clinical or preclinical studies indicate that the product candidates are unsafe or ineffective; the potential that it may be difficult to enroll patients in our clinical trials; the potential that regulatory authorities, including the FDA, EMA, and PMDA, may not grant or may delay approval for our product candidates; the potential that we may not be successful in commercializing Galafold in Europe, Japan, the US and other geographies or our other product candidates if and when approved; the potential that preclinical and clinical studies could be delayed because we identify serious side effects or other safety issues; the potential that we may not be able to manufacture or supply sufficient clinical or commercial products; and the potential that we will need additional funding to complete all of our studies and manufacturing. Further, the results of earlier preclinical studies and/or clinical trials may not be predictive of future results. With respect to statements regarding projections of the Company's revenue and cash position, actual results may differ based on market factors and the Company's ability to execute its operational and budget plans. In addition, all forward-looking statements are subject to other risks detailed in our Annual Report on Form 10-K for the year ended December 31, 2018. You are cautioned not to place undue reliance on these forward-looking statements, which speak only as of the date hereof. All forward-looking statements are qualified in their entirety by this cautionary statement, and we undertake no obligation to revise or update this news release to reflect events or circumstances after the date hereof.

CONTACTS:

Investors/Media:
Amicus Therapeutics
Sara Pellegrino, IRC
Vice President, Investor Relations and Corporate Communications
spellegrino@amicusrx.com
(609) 662-5044

Media:
Amicus Therapeutics
Marco Winkler
Director, Corporate Communications
mwinkler@amicusrx.com
(609) 662-2798

FOLD–G

TABLE 1

Amicus Therapeutics, Inc.
Consolidated Statements of Operations
(Unaudited)
(in thousands, except share and per share amounts)

 Three Months Ended June 30, Six Months Ended June 30,
 2019 2018 2019 2018
Revenue:       
Net product sales$44,130  $21,309  $78,176  $38,005 
Cost of goods sold5,367  3,135  9,422  5,750 
Gross profit38,763  18,174  68,754  32,255 
Operating expenses:       
Research and development70,981  34,660  135,574  75,458 
Selling, general, and administrative42,578  29,172  86,881  56,568 
Changes in fair value of contingent consideration payable480  300  1,863  1,400 
Depreciation and amortization1,154  973  2,145  1,942 
Total operating expenses115,193  65,105  226,463  135,368 
Loss from operations(76,430) (46,931) (157,709) (103,113)
Other income (expense):       
Interest income2,599  2,913  5,238  4,650 
Interest expense(4,625) (4,560) (11,079) (9,048)
Loss on exchange of convertible notes(4,501) —  (40,624) — 
Change in fair value of derivatives—  (7,600) —  (2,739)
Other income (expense)(877) (5,316) 209  (2,554)
Loss before income tax(83,834) (61,494) (203,965) (112,804)
Income tax (expense) benefit(717) (339) (885) 1,053 
Net loss attributable to common stockholders$(84,551) $(61,833) $(204,850) $(111,751)
Net loss attributable to common stockholders per common share —
basic and diluted		$(0.36) $(0.33) $(0.91) $(0.61)
Weighted-average common shares outstanding — basic and diluted238,089,824 188,621,423 225,848,013 182,303,128

TABLE 2

Amicus Therapeutics, Inc.
Consolidated Balance Sheets
(Unaudited)
(in thousands, except share and per share amounts)

 June 30,
 2019		 December 31,
 2018
Assets   
Current assets:   
Cash and cash equivalents$220,578  $79,749 
Investments in marketable securities355,078  424,403 
Accounts receivable28,709  21,962 
Inventories10,395  8,390 
Prepaid expenses and other current assets20,116  16,592 
Total current assets634,876  551,096 
Operating lease right-of-use assets, less accumulated amortization of $2,641 and $0 at June 30,
2019 and December 31, 2018, respectively		35,052  — 
Property and equipment, less accumulated depreciation of $16,890 and $15,671 at June 30,
2019 and December 31, 2018, respectively		15,273  11,375 
In-process research & development23,000  23,000 
Goodwill197,797  197,797 
Other non-current assets12,035  6,683 
Total Assets$918,033  $789,951 
Liabilities and Stockholders’ Equity   
Current liabilities:   
Accounts payable, accrued expenses, and other current liabilities$84,119  $80,625 
Deferred reimbursements2,750  5,500 
Operating lease liabilities2,678  — 
Total current liabilities89,547  86,125 
Deferred reimbursements11,406  10,156
Convertible notes2,070  175,006
Senior secured term loan146,994  146,734
Contingent consideration payable21,247  19,700
Deferred income taxes6,465  6,465
Operating lease liabilities36,259  — 
Other non-current liabilities3,987  2,853
Total liabilities317,975  447,039 
Commitments and contingencies   
Stockholders’ equity:   
Common stock, $0.01 par value, 500,000,000 shares authorized, 254,513,522 and
189,383,924 shares issued and outstanding at June 30, 2019 and December 31, 2018,
respectively		2,589  1,942 
Additional paid-in capital2,201,447  1,740,061 
Accumulated other comprehensive loss:   
Foreign currency translation adjustment352  495 
Unrealized gain (loss) on available-for-sale securities355  (427)
Warrants12,387  13,063 
Accumulated deficit(1,617,072) (1,412,222)
Total stockholders’ equity600,058  342,912 
Total Liabilities and Stockholders’ Equity$918,033  $789,951