Good day, ladies and gentlemen. And welcome to the Amicus 2Q Results Conference Call and Webcast. At this time, all participants are in a listen-only mode. Later, we will conduct a question-and-answer session, and instructions will follow at that time. (Operator instructions) As a reminder, this conference is being recorded. I would like to introduce your host for today’s conference, Vice President of Finance, Daphne Quimi. Ma’am, you may begin.

Good afternoon. And thank you everyone for joining our conference call to discuss our second quarter 2014 financial results. Speaking on today’s call we have John Crowley, our Chairman and Chief Executive Officer; Chip Baird, our Chief Financial Officer; Jay Barth, our Chief Medical Officer; Bradley Campbell, our Chief Operating Officer; and Hung Do, our Senior Vice President of Discovery Biology. Today’s prepared remarks coincide with the slide presentation that is now available on our corporate website at www.amicusrx.com. The slides are located in the Investor section under Events and Presentations right below the webcast link to today’s call. On slide two, you will find the reference to our Safe Harbor. This conference call contains forward-looking statements within the meaning of the Private Securities Litigation Reform Act of 1995 relating to business, operations and financial conditions of Amicus, including but not limited to, preclinical and clinical development of Amicus’ candidate drug products, cash runway and the timing and reporting of results from clinical trials evaluating Amicus’ candidate drug products. Words such as, but not limited to, look forward to, believe, expect, anticipate, estimate, intend, plan, would, should, could and similar expressions or words identify forward-looking statements. Although, Amicus believes the expectations reflected in such forward-looking statements are based upon reasonable assumptions, there can be no assurance that its expectations will be realized. Actual results could differ materially from those projected in Amicus’ forward-looking statements due to numerous known and unknown risks and uncertainties, including the Risk Factors described in our annual report on Form 10-K for the year ended December 31, 2013. All forward-looking statements are qualified in their entirety by this cautionary statement and Amicus undertakes no obligation to revise or update this presentation to reflect events or circumstances after the date hereof. At this time, it is my pleasure to turn the call over to John Crowley, Chairman and Chief Executive Officer of Amicus.

Great. Thanks, Daphne, and good evening, everyone. This is certainly an exciting time for Amicus and welcome to our review of our second quarter 2014 financial results, and as we've done before we are going to provide several program updates. Let me begin by saying that there are a lot of good things to review and this in almost every respect was a very outstanding quarter for Amicus. We have made some very important progress in all of our programs. I will be speaking a little bit just to address my military leave of absence. But let me begin with just a brief overview before addressing that then turning it over to our teams to take us through a number of these programs. So just to highlight some of what we will go through, our phase 3 Fabry monotherapy program, of course, in the second quarter we released very compelling 12 and 24-month data from our Fabry Study 011 where we clearly showed that migalastat is having an effect in patients for whom it is intended. So very good result from that study, certainly catalyst much of our success in the second quarter. We are now eagerly awaiting the topline results from our second Phase 3 clinical trial, the switch study from ERT our Fabry Study 012. We do expect to have that in the third quarter. The database has been locked. We are at Amicus all still blinded to the data. So none of that is come yet from our contract research organization and team, but we have -- it is some we are very much looking forward to hear in this quarter. Also in these slides and there is some important new data here that we have to share with you on the progress we've made with our…

Thanks John. I am very happy to be able to talk to about the Fabry monotherapy program. There are two Phase 3 studies that are the cornerstone of the program, the Facets study and Attract study. So, I will start by reviewing briefly the key elements of those studies. The Facets study, which we also call Study 011 is the study whose result we reported recently, very good result. It -- study begins with a six-month placebo control phase and the 67 patients in total in the study all naïve patients, naïve ERT. And in the six months and 12-month data we look at kidney GL-3, the substrate in the kidney. We also importantly looked at clinical data, which I will get to you briefly on renal function at 12 and 24 months. The Attract study is also called Study 012, the second Phase 3 study and its an ERT switch study, which includes 60 patients, randomized 1.5 to 1 for migalastat versus ERT and these patients are funneled over 18 months. Looking at the clinical endpoints of kidney function and we are looking at both measured GFR and estimated GFR, and these data are due in third quarter of ’14 and we will be able to report them as soon as we are able to provide that information to you. The results from Study 011, the Facets study, which we reported recently, released in April, the 12 and 24 months data showed a clear effect of the drug in Fabry patients with amenable mutations to reduce substrate and to stabilize kidney function. What we saw in analyzing this data is that, patients who switched from placebo to migalastat after being on placebo for six months had statistically significant reduction in kidney IC GL-3 through months 12 and the patients…

Great. Thank you, Jay. That’s a terrific overview of where we are on monotherapy program. But before we leave monotherapy though I do want to share some very that our science team has been doing to better understand and characterize migalastat mechanism of action. As we know we have said for sometime now that this is a unique way to treat Fabry disease and we think we hope it will advancement for patient. Certainly, we think more convenient in its oral form, oral bioavailability of the drug. We also though have understood that patient seem to tolerate the drug very well. So that also plays to the benefit. But when it comes to the core understanding of the mechanism of action, we focused quite a bit on the notion that this is small molecule that specifically targets the patient’s own enzyme providing catalytic confidence and chaperoning the enzyme, elevating enzyme activity in reducing substrate. That’s very clear, 011 study in particular was very clear in demonstrating that. So we’ve also been working to understand what we think could be another potential added benefit of the Migalastat mechanism of action in terms of its chaperoning function and its reduction of cellular stress and the pathways involved within patient’s cells. So with that, I’m going to turn the call for the next slide. Over to Hung Do, who will walk us through the mechanism of action and the potential implications for Fabry patients. So Hung, I’ll turn it to you for slide 8 here.

Great. Thank you, John. So as John alluded to, most of our focus actually has been centered on understanding appropriate patient population and how do those -- this particular smaller molecule to have a significant increase in the patient’s own endogenous enzyme at the right -- sort of, is able to get out of the endosplasmic reticulum model it’s made and then go to lysosomal we are actually need the function. So this actually is shown in slide number 8. What is known and we actually are continuing to understand this better with different diseases is that when a genetic mutation causes change in the amino acid sequence would result in protein. There is significant problem with that protein coding such that the protein causes a certain levels of distress within the cell, which is typically known as the unfolded protein response. This actually can have some pretty significant problems in the cellular pathology such that there is an adaptive response call ER stress which in turn increases the expression of other helper proteins. These are proteins which are often referred to as chaperone protein which help to deal with this unfolded protein. So that these proteins can code better and able to reduce this particular cellular stress. We have lost too much time understanding this but we are starting to do some work to understand whether small molecule pharmacological chaperone cannot only increase the protein traffic gain but also can reduce some of the cellular stress. We’ve actually done some as well as other research labs around the world as we’ve shown in slide number 9 to understand this particular phenomena. In this particular slide what we did was to understand whether small molecular can improve the protein trafficking of the mutant protein out of the endoplastic reticulum which…

Great. Thanks Hung. That was a terrific overview and hopefully everyone gets the sense that we continue to have the effect in patients, we believe by hitting the target, elevating enzyme activity and reducing substrate. We think that was made very clear with the 011 data and numerous preclinical studies that we’ve conducted. We think we can do that as well and in some tissue type, given the targeting of the nature of the small molecule potentially maybe even better than ERT. What we do know though is that we can chaperone the person's own native enzyme through those trafficking pathway that Hung just highlighted. And we think that again that might have a significant benefit for patients but more work to be done there, but it's been some terrific work coming out of our science groups recently and we though important to share as we head into this next level of clinical data. Let me go ahead now and transition on the next slide, slide 11 to Pompe disease. And I’ll do this before I hand it over to Brad to provide an overview of our ERT programs and status. So Pompe is a disease that we’ve known for a long time. I've known it personally since 1998 and professionally since the year 2000. It is a devastating disorder ranging from infants all the way through adulthood. It is one of the more prevalent of the lysosomal storage disorders. We believe the current enzyme therapies, which many of us were involved in from the start are good first step toward treating the disease but we have a long, long way to go. And if you look on the next slide, we just provide some perspective here on slide 12. In many ways, how far we’ve come as a community…

Thank you, John. And so I think that for us to, through our lot of experience as John alluded to over the past 15 or 16 years with Pompe disease, we actually have come to understand so much more about this enzyme and what actually is required for this particular ERT to be an effective approach for treating Pompe disease. And so on slide number 13, this actually is a way for us to better highlight the major problems with this particular enzyme. Let me show this particular slide is that we use a receptor column, let just say this is (indiscernible) receptor which is known to be utilized for internalizing the ERT that is able to get into both and be delivered to lysosomes. What is interesting is, these ERTs require specialized carbohydrate structure called mannose-6 phosphate. And so what we’ve done in this particular experiment is that we utilized the intend receptor, mannose-6 phosphate receptor which can be put on to a column and just measured how much of the ERT is able to bind the receptor. So top panel, actually is the standard-of-care. What is shown here is that when the enzyme is put onto this column, the fraction of enzyme that does not contain mannose-6 phosphate cannot find the receptor and therefore flows through the column. (Indiscernible) actually does contain mannose-6 phosphate by the column which then we elude with excess amounts of the free carbohydrate mannose-6 phosphate therefore out computing the binding. And so what you’re seeing in the top panel is that the standard-of-care only contains a small fraction of the enzyme which actually -- which contains mannose-6 phosphate. This is one of the major reasons why this enzyme needs to be dosed at such high dosages where they have some clinical benefit. With…

Great. Thanks Hung for that overview and for all the terrific work that you and the team have put into this certainly in your time back now at Amicus. This has been another part of our progress this year but certainly building on the many years of experience and a lot of passion going into making a better medicine in Pompe. Lots of work has been done into all of our ERT next generation program. And on slide 16, Brad will take us through some of the highlights. Go ahead.

Great. Thanks, John. So as John mentioned on slide 16, let me just first provide an update on our Fabry next-generation ERT program, which is of course, co-formulated with migalastat. We’ve continued to make great progress towards beginning a Phase I, II study in Fabry patients and to that end we’ve successfully completed two key operational milestones, including the completion of migalastat IV PK study and also final manufacturing of drug product of that co-formulated product and we continue to work closely with investigators on a protocol design. In parallel as we’ve mentioned before, we’ve also been evaluating sources of long-term supply of Fabry enzyme. And as you saw in the press release, at this point we have several ongoing promising business development discussions underway. And so we have made the strategic decision to wait to begin the next Phase I, II pending the outcome of those discussions. As soon as we have further clarity there, we’ll provide an update to all of you on the next steps for the program. On a related note, I’m pleased to announce that Dipal Doshi has recently joined Amicus as Senior Vice President of Business Development and Business Planning. Dipal brings over 15 years of operating, investing and business development experience in the biotech and healthcare industry. And he is a great addition to the management team and to our efforts along these lines. And then finally just a quick comment on our Parkinson's program. As you know, we've had an ongoing effort to develop pharmacological chaperones that target GCase for the treatment of Parkinson's. We previously had initiated a research collaboration with Biogen Idec to discover and develop a class of novel small molecules that target the same pathway. At this time, we’ve agreed with Biogen to conclude that collaboration in September. However, importantly, Amicus does retain all global worldwide rights to our most advanced Parkinson's compound AT3375. And with that, I will turn it over to Chip to walk us through our financials.

Great. Thanks Bradley, and good afternoon everyone. As John mentioned at start of the call, we significantly strengthened our balance sheet in second quarter to successful execution to a $40 billion at-the-market equity financing. Within sales of primary shares under the ATM in May and sold the final block of shares on July 1. As a result, we've elected to show two columns on slide 17, the first being the official June 30 balance sheet numbers, as well as the July 2 balance sheet numbers, which show the effect of the final proceeds received from the ATM financing. Now as you can see the June 30 cash balance was $78 million, which is compared to $82 million at December 31st of last year. So with the final proceeds from the ATM, we had $98.4 million in cash and cash equivalent as of July 2nd. In terms of additional sources of funding, we still have $10 million remaining available under the debt facility we entered into in the fourth quarter of 2013. As previously guided, we expect full year 2014 net cash spend to total between $54 million and $59 million. With the proceeds from the ATM in hand, we believe that our current cash position is sufficient to fund our current operating plan into 2016. Turning to our second quarter 2014 financial results on slide 18, I will be referencing Tables 1 and 2 in the press release which we issued earlier today and additional details can be found in our quarterly report on Form 10-Q, which will be filed later this evening. We recorded modest revenues of $475,000 in the second quarter of 2014, in conjunction with the Biogen collaboration. Total operating expenses for the second quarter decreased to $14.7 million, compared to $16.0 million for the second quarter of 2013. The year-over-year decrease was primarily due to decreases in personnel costs as well as decreases in contract research costs. The net loss attributable to common shareholders in the second quarter of 2014 was $14.6 million compared to a net loss of $15.3 million in the second quarter of 2013. Net loss per share of $0.22 in the second quarter of 2014 was narrower than the net loss per share of $0.31 in the second quarter of 2013. The narrower net loss and net loss per share versus the year ago period is primarily attributable to the lower overall loss and an increased shares account as compared to the year ago period. As of June30, 2014, we had approximately 72.9 million shares outstanding compared to 49.6 million shares outstanding in June of 2013. This summarizes our key financials results for the second quarter of 2014 as well as our full year 2014 guidance. Happy to answer any questions during the Q&A session. But for now, we’ll turn the call back to John.

Great. Thank you, Chip. And just on the last slide here before we open it up for Q&A, just regarding the continuity of leadership at Amicus, driven by a military deployment, again just to summarize I will remain Chairman and CEO. I expect that leave of absence to be about 32 weeks. I will deploy, we expect by the end of September, again deploying to Afghanistan. And I think for these deployments, they are always toughest on those you leave behind. I will be perfectly fine and safe. And in fact in the last 90 minutes or so since the press release, I have gotten some wonderfully kind e-mails from people. And I assure you my sacrifice is very, very small compared to everyone else who served in those theaters. In many ways, I continue to serve in the company of heroes. You’ve always of course, are concerned about those who leave behind and just as I'm absolutely confident that Aileen will take terrific care of our three kids. While I’m deployed, I'm also absolutely confident that Brad and Chip and Jay and the senior team and everybody in Amicus will continue to perform all of their duties and continue to do an excellent job. Managing Amicus, I still expect to be available for again some of the major strategic decisions in business issues. This is a very exciting time and for very selfish reasons. I'm sorry, I’m not going to be there for this physical absence because I think this is going to be yet another extraordinary couple of quarters for Amicus, which mean I'm all the more eager to come back in the first half of the year and resume my full-time duty as a Chairman and CEO. So, again, it was a terrific quarter, we hope we have just as exciting and positive quarter in 2014 and inn the quarters ahead of us. With that, Operator, we’re happy to open it up to questions.

Thank you. (Operator Instructions) And our first question comes from Mike Ulz with JPMorgan. Sir your line is open.

Hi, guys. Thanks for taking the question. Just with Phase 2 data from the 012 study coming up here in the near future. Can you comment on where you guys see a sort of the biggest risk to a positive outcome for that study?

Yeah. Sure. We’ve considered lots of potential outcomes, of course, Mike, we’re hopeful and confident that the data will be where we needed to be and again, we’re still blinded to all of that. The patient disposition numbers have been very, very positive. Jay, I’ll let you comment specifically to any risks with the study and how we’ve mitigated those risks.

Okay. I think, we’ve learned so much from the 011 study, that we've been able to minimize any risk in the 012 study. We know how to analyze the data. This probably the most important thing that we’ve learned from 011 and we know from the experience of the investigators, customer we involved in both studies, how the data collected, I am referring to the renal function data. And because of the similarities of the studies in terms of the, what the type of patients, the measuring thing done, the duration of the studies, we feel very good based on the result we saw on 011 about what’s upcoming in 012, and we could everything that we learned in the conduct of the first study and the analysis of the 011 to apply to the 012 study. So, I really think we're in quite good shape and that’s why I am very optimistic about the data that’s coming up in 012.

Just add to that, Mike, we early on in the planning of this study, we had learned from some of our Phase 2 studies as well. So, for instance, patients were carefully randomized by ERT, by (indiscernible), even by levels of pertain area at baseline.

Okay. Thanks, guys.

Very welcome.

Thank you. And our next question comes from Mr. Joseph Schwartz of Leerink Partners. Your line is open.

Great. Thanks very much. I was wondering since I see that you finished the Phase 1 trial for IV migalastat, you could characterize those data, because I remember with -- when the oral migalastat was studied in a co-administration strategy? You needed to give the drug ahead of time in order to get the drug on Board approximate to the time that the enzyme would be available for meeting with the chaperone? So what did you learned from this study and how are you going to use this information when you go into Phase 1, 2?

Hey. Thank you, Joe. Jay, maybe if you’ll take the first part of that, just outlining the work that was done and what was the significance of the study. And then, Brad, maybe you and Jay, can together answer what it means for future development of the next-generation Fabry ERT.

The study had really helped us define the dose going forward. We understand the pharmacokinetics of the IV dosing of migalastat and that’s really information that we needed in order to make sure we’re going into the next study with the data that we need. So we understand the PK of the IV dose. We understand how the dose fit and what the regimen should look like.

Yeah. Joe, I mean, as you articulate with the oral form of migalastat, it limits you in some ways and how you can deliver it and what time you have to deliver it and from a long-term perspective, both from -- you getting the exact right dose of the IV migalastat plus the dose of the ERT. But then also from a clinical and commercial perspective, not having to stay 30 minutes ahead of your infusion take the oral dose migalastat. There is a whole host the reasons why we believe having that IV form was a prudent way to go and so this study was, as Jay mentioned, was successful and we got the information we needed to design the next Phase 1, 2 study.

Okay. Great. I'll look forward to seeing more as that advances. Thank you.

Great. Thank you, Joe.

Thank you. (Operator Instructions) Our next question comes from Kim Lee of Janney Capital. Your line is open.

Good afternoon and thanks for taking the question.

Sure, Kim.

And I have -- yeah, I have a question on Parkinson's disease and since the -- since you’ll be concluding your collaboration with Biogen? Can you tell us what next steps are for this indication and what your plans are? Thanks.

Yeah. Kim, this is an early stage research program that builds to upon a couple of years of work that we had been doing at Amicus. I think it was good collaboration. We enjoyed working for the one-year period with Biogen from our perspective and we had about six or seven full-time Amicus employees who are engaged in this project, while paid for completely by Biogen. As we're advancing and our next-generation ERT programs are advancing, we really needed the bandwidth within the company. In addition, since not renewing this partnership for another year gives us the rights to take back these molecules. They, of course, preserve the right to use 3375 molecule and others in a next-generation cache ERT that was we build up our pipeline. We think that could be a very important tool set for us. So in terms of Parkinson's going forward, we’re in the process of evaluating that. What we’ll do with that program specifically, but we don't yet have any further comments on we will take Parkinson.

Okay. Great. Thank you.

At this time, I see no further question in queue. I’d like to turn the call back over to you Mr. Crowley for any closing remarks.