Good day and welcome to the Amicus Therapeutics First Quarter 2012 Earnings Conference Call. At this time all participants are in a listen-only mode. Later we will conduct a question-and-answer session and instructions will be given at that time. [Operator Instructions]. As a reminder this conference call is being recorded. I would now like to introduce the host for today's conference, Ms. Sara Pellegrino Associate Director, Investor Relations. Please go ahead ma’am.

Good afternoon and thank you for joining our conference call to discuss our first quarter 2012 financial results. Speaking on today's call we have John Crowley, our Chairman and Chief Executive Officer; Bradley Campbell, our Chief Business Officer; and Chip Baird, our Chief Financial Officer. They are joined by Pol Boudes, our Chief Medical Officer; and David Lockhart, our Chief Scientific Officer who are also available to participate in the Q&A session. As a reminder, this conference call will contain forward-looking statements within the meaning of the Private Securities Litigation Reform Act of 1995 relating to the business operations and financial condition of Amicus, including but not limited to preclinical and clinical development of Amicus candidate drug products, the timing and recording of results from preclinical studies and clinical trials evaluating Amicus’ candidate drug products and the projected cash position for the company. Words such as, but not limited to: believe, expect, anticipate, estimate, intend, likely, should and could and similar expressions are words identifying 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 these expectations will be realized. Actual results could differ materially from those projected in Amicus’ forward looking statement 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, 2011 and other public filings with the Securities and Exchange Commission. Amicus does not undertake any obligation to publicly update any forward-looking statements that reflect events or circumstances after the date on which any such statement is made, or to reflect the occurrence of unanticipated events. At this time it is my pleasure to turn the call over to John Crowley, Chairman and Chief Executive Officer of Amicus Therapeutics.

Great. Thanks, Sara. Good evening, everybody, and welcome to our first quarter 2012 conference call. I am happy, as always, to lead the call and let me begin by welcoming Chip Baird, who has been with us for nearly a month as our Chief Financial Officer. Chip comes to us after spending the last decade with PTC therapeutics. So great experience in rare disease area and welcome Chip and we will hear shortly from Chip on some of the financials. After I walk us through some of recent corporate milestones and specifically progress in our February Phase III studies of migalastat as a monotherapy, I will turn it over to Bradley Campbell our Chief Business Officer. Brad will be providing a status update on our Chaperone-ERT combination products. And before I close out the call, Chip again will review the first quarter financials. So a very exciting quarter for Amicus. As you all know, in March of this year we successfully raised $62 million in net proceeds from a public offering. Due to very strong demand and broad participation, we saw several important new shareholders come into Amicus as long-term investors. We hope and also we saw some of our long-term shareholders also continue buying shares in Amicus through this offering. And I would like to welcome any of you to today’s call. We began the year in January at the JPMorgan conference outlining our corporate strategy, our current portfolio and some very important positive preliminary results from the Phase II study of our pharmacological Chaperone migalastat co-administrated with enzyme replacement therapy, or Fabrazyme for Fabry disease known as our Study 013, a very important milestone for the company. And since that time we've made terrific progress executing against that business strategy and our business plan, and we’ve done that…

Thanks, John, and good evening, everybody. Let me just reiterate one thing that while we do believe the enzyme replacement therapies were important first step in the treatment of lysosomal storage disorders, our vision is to use the chaperone-ERT combination platform to create better therapies for patients with LSDs. Our top priority is to improve efficacy and potentially reduce immunogenicity by combining ERTs with our specific pharmacological chaperones. And along our continuum of innovation, this might include combinations with next-generation ERTs or perhaps improved delivery modalities, but we are first and foremost looking for significant improvements with existing standard of care ERTs. Right now we are evaluating several chaperone-ERT combinations in Phase II studies in Fabry and Pompe and a preclinical study negotiating other undisclosed LSD targets where there is a significant opportunity to improve current ERT products. A reminder under our Fabry collaboration with GSK, we are developing migalastat as a monotherapy and in combination with ERT, but we retain exclusive rights to all of our other programs. So most of you are familiar with this by now, but when co-administered with ERT, pharmacological chaperones are designed to bind to and stabilize the infused or common enzyme in the circulation of patients as they receive their ERT. Because of the chaperone is binding to recombinant enzyme rather than the patient’s own enzyme this approach has the potential to improve ERT outcomes regardless of the patient's mutation type. Preclinical chaperone-ERT combination studies and animal models of Fabry, Pompe and Gaucher has shown that a pharmacological chaperone can select and bind to and stabilize the recombinant enzyme, prevent the activation and circulation, increase uptake of active enzymes to the key tissues of disease. And in addition, in Fabry and Pompe animal models we've shown that this increased activity in tissue uptake…

Great. Thanks, Bradley, and good afternoon, everyone. I am very excited to be here today on my first quarterly call as the CFO of Amicus. Before I turn toward the first quarter of 2012 financial results I would like to comment on our cash position and reiterate the full-year 2012 financial guidance that we first announced during the JPMorgan Conference in January. Cash, cash equivalents and marketable securities at March 31, totaled $108.2 million compared to $60 million at the beginning of 2012. Our current cash position includes the $62 million in net proceeds raised in our public offering of common stock, which closed in early March of this year and significantly strengthened our balance sheet. We continue to expect full-year 2012 operating expenses for a total between $37 million to $43 million net of anticipated cost sharing and milestones related to the GSK collaboration. As a reminder, GSK is responsible for 75% of the development costs for migalastat in 2012 and beyond, subject to annual and aggregate cash. We are also eligible to receive up to $170 million in development, regulatory and commercial milestones as well as tiered double-digit royalties on global sales in migalastat. Turning to the financial results, I will be referring to table 1 in our press release which is available on our corporate website at www.amicustherapeutics.com. Additional details can also be found in our Form 10-Q to be filed later today. Turning to the P&L. Total revenue was $7.8 million for the first quarter of 2012 compared to $6 million in the prior period. Our total revenue consists of research and collaboration revenues recognized under our collaboration with GSK for migalastat. Research revenue for the first quarter of 2012 was $6.1 million up from $4.3 million in the year-ago period. The increase is due to…

Great. Thanks, Chip. So hopefully, everybody can see a lot of momentum at Amicus for all of our ongoing studies. And we certainly have a full calendar of upcoming milestones expected throughout 2012. We continue to move toward our vision of becoming a fully-integrated biopharmaceutical company. As I've discussed with many of you, we do intend to be a company that has strengths from research and development, all the way one day to sales and marketing. We do continue to advance and leverage our core technology platform, to develop chaperone monotherapy and chaperone ERT combination therapies. We have a terrific collaboration with GSK rare diseases, and we continue to strengthen that relationship. And we do continue with GSK to move toward our shared vision to deliver benefits to patients and their families who are affected by these rare and orphan diseases. And so with everything we just covered in the call today and for all our efforts, and for everybody here at Amicus, we sit here today with a market capitalization of just over $200 million. As Chip indicated, we have a cash position at the end of Q1 of just over $108 million. So today, approximately an implied program and technology value of just over $100 million for all of those clinical and preclinical activities here at Amicus. We think that represents a terrific value proposition for investors today, and one that we continue to hope to drive forward in the many months and years to come. With that, Operator, we are happy to take questions.

[Operator Instructions] We have a question from the line of Ritu Baral with Canaccord.

First question, what do you think is driving the much, much faster 012 enrollment, the ATTRACT trial enrollment, versus 011? Is it just the entry criteria difference or is it the shortage as well? What factors should we be thinking of?

No. This was a study, as we have talked about Ritu, we were concerned about enrollment timelines here, because it's a big deal to ask people to come off of an existing therapy, lifesaving therapy for a fatal genetic disease, and to come on to migalastat as their only therapy for Fabry disease. That's a big deal difference in 011. And although, yes, you're correct, we don't have biopsies here. This I think speaks to hopefully the now more than a 150 years of safety data and patient data, the strength of those 17 people who continue now for some of the more than 6 years. I think it's just the totality of the experience and the data and the ongoing momentum in the studies. I think hopefully success builds on success. I think obviously patients and physicians look, just as you guys do, to the patient disposition in the current 011 study. We are very much blinded to that data, so we don't know any of the results. But what we do know is that patients, at least from a tolerability standpoint, seem to be having a good experience. And a 100% conversion into the extension study is pretty remarkable, and I think all those factors come into play. And I can tell you also, too, we've learned a lot from the execution side, and our clinical operations team has done a remarkable job to get with GSK at making sure that we are on top of every item in terms of clinical research and operations to ensure that everything within our control is controlled for. So all those experiences I think bode very well.

Is there a significant trial -- say, overlap between the 2 studies?

There is significant overlap but there are a number of those 25 studies that are new sites, but there is a significant amount of overlap.

And next question, on the Pompe’s program. Can you talk a little bit or give some description as to the doses that you are using in the dose ascension. Are we looking at sort of doubling and tripling or are we talking about more incremental step-ups in dosing?

No, I think it’s the former and without giving specifics, I don't think it’s on clinicaltrial.gov. We just have indicated we are starting at a low dose, moving to a higher dose. That higher dose is still quite a bit lower from what we used years ago as a monotherapy, but we are moving up -- in some cases doubling the dose, so significant. And also very important to remember, very consistent with the animal studies that we did. In many ways what we are doing in the 010 Pompe study, just like we did in the Fabry 013 is hoping to recreate in patients the very positive results that we saw in animals in terms of the safety of the interaction of the small molecule in the ERT, the enhanced tissue uptake. And in this case, we are not looking specifically at substrate reduction, but surrogate market like plasma, increased plasma activity, ERT is very important.

And Ritu remember that as you know, the dose that we are using is much lower than what we used in the monotherapy study with AT2220. And then also the dosing regimen is very different. The drug is only given once every two weeks prior to the infusion as opposed to three times a week as it was with the monotherapy. So that the overall amount of drug used in the combo is far less than what was used previously.

I just want to clarify too, and Bradley went into a great detail, Ritu, on the biopsies. Again, we are very concerned with the safety here, we want to make sure and as excited as we are about the data from the animal studies. We want to make sure as we translate this hopefully into people with Fabry ,that we do it in a very measured fashion. So as Brad indicated, just to reiterate, the first patient who have the biopsies only had the biopsies at base line and then at day seven, where we were looking primarily for the presence of the small molecule. And then when we went to the second cohort, we had some patient -- everybody at base line, some patients at day 3 and some patients at day 7.

Was there a particular observation that triggered the addition of the day three biopsy or was it something in the pharmacokinetics?

I am sorry. Can you repeat that?

Was there something in your observation of the first cohort that triggered the addition of the day 3 or was it something in the sort of pharmacokinetics of the drug?

No. Actually, it was our experience -- a very positive experience in the 013 Study where we saw the day two biopsies significantly enhance tissue uptake of the Fabrazyme. That gave us great encouragement and the proof-of-concept, and that’s when we went back and amended the protocol to include day 3 biopsies. And just to clarify to you -- Pol just passed me a short note here -- they are not baseline biopsies for these patients in day 3 and 7. Go ahead, clarify Pol.

Yes, you compare -- each patient is going to be compared with the same timing, so you compare the patient with the same data; so your day 3 patients or day 7 patient, you’re going to have the first biopsy without the chaperone and second biopsy with the chaperone.

And so that comparison there is ERT alone or ERT plus the small molecule. So each patient gets two muscle biopsies total.

Obviously, it’s just a matter of balancing out how many biopsies can you recently do in a study like this, and so that’s what we are trying to balance the safety of the [unintelligible].

And last question, I’ll hop back in the queue. You mentioned briefly, or you referred to the next study in the Fabry combination therapy; can you give any more detail about what it might look like? How many arms would you have to look at the two components separately in that study or would it be simple two-arms study?

That’s exactly what we are thinking through right now. We can think very simply. What we are trying to prove is that the efficiency of the overall administration of the chaperone here with Migalastat has a better outcome for patients than ERT alone. So it might be as simple as a two-arm study; one arm on ERT alone, one arm ERT plus co-administration of the chaperone. The important thing is that unlike 013 Study, it will be repeat dosing. The exact endpoint we’re not clear on; there is a couple of different ones that we can look to. Obviously we would like to default to the one that not only is test positive, but the one that might provide the most insight as to the change that we would expect to see.

And our next question is from the line of Joseph Schwartz with Leerink Swann.

I was wondering, just continuing on the Pompe combination study theme; what types of patients are you enrolling? What kinds of Pompe patients? Are these early or late onset patients? Are naïve or treatment-experienced? Are they patients with antibodies that have formed yet or not?

I’ll let Pol Boudes, our CMO comment on that.

The patients we are enrolling on late-onset patients. They are (inaudible) patients, and they are treated with ERT. So they are not naïve patients.

And remember, Joe, one of the important parts of the study is that we are studying patients who are on ERT and we don’t change their ERT regimen at all. All we do is add in -- on one of their infusions we add in a pre-oral dose of the small molecule. Patients on ERT we don’t pre-select based on anti-body titer. We don’t pre-select based on mutation. They are on ERT and we leave their ERT regimen alone. We just add on, on top of the pre-oral dose of the small molecule.

Okay will you be able see what some of you captured that information what kinds of responses or how the responses vary on whether a patient has a certain level of antibodies or not?

So this is just a single administration study. So we are measuring their titers, and we will be able to tell if the antibody titer affects the circulated - the effect on the PK or the uptake but this is only a single administration study, so we don’t expect to see changes in glycogen or other functional measures in the study?

And then what muscles are you biopsying?

The quad.

And do you have any milestones -- if the 011 study is successful and you hit your primary endpoint, I think you are entitled some milestones from GSK. Do you have that embedded in your financial guidance for 2012. I forget?

Yes, remember again it is $170 million in total milestones. We have guided, Joe, that roughly half of them are clinical and regulatory milestones and the other half is commercial milestones for 011. We have also stated that we don’t expect any significant milestones this year. We could -- we are eligible for some but the larger ones related to some of the end-stage clinical and regulatory triggers like the ones you've just indicated, the ones that would be expected to be in the tens of millions of dollars. We don't expect those in 2012, and likewise in all of our cash guidance we never include any of the milestones.

Can I just sneak one more in -- to chaperones which you are developing for Gaucher, AT2101 and 3375, how do they differ? Is one better or different than the other in any meaningful way?

So 2101 Plicera was the molecule that we developed initially for monotherapy for Gaucher. That one is also a good candidate for a possible use in the combo. AT3375 is a newer molecule that we had developed in part as part of our Parkinson’s work. It has greater brain penetration and it is just as potent. In terms of the molecular interaction, it is more potent in vivo and it is a synthetic derivative of AT2101. So they are related molecules in the same chemical class but 3375 was selected because of some properties that we were looking for, brain penetrants and exit from cells and tissue and the lysosomes.

If it's more potent, would it be tougher or easier to dose it in order to not overdose it and have the inhibitor or does it not work like that?

It may mean that the dose that we can use is lower, but that of course will -- that is in the process of being tested, so it's possible that the dose could be lower and it leaves cells and tissues and lysosomes more quickly. So that could make it so we have less of any inhibition from the molecule, so that we get a greater effect from the enzyme -- from the additional enzyme.

And so, would you maybe then dose it every day, do you think? Is that a possibility unlike the Amigal?

So in the context of the combo, it would still be given just as a pre-oral dose initially when the infusion -- just before the infusion. So as a combo therapy, the dosing is very different. And so, that would just be prior to the start of the infusion so that it can help stabilize the infused enzymes. So it wouldn't be given every other day or multiple times a week. It would only be given once every other week with the infusion.

[Operator Instructions] And our next question is from the line of Anupam Rama with JPMorgan.

Coming out of the world meeting, one of the questions we get a lot of about the amenable mutations for migalastat. If I remember correctly, if you look at the baseline data, the percentage of a minimal mutation for migalastat was a little bit higher than sort of the 40%, 50% that you've quoted. Can you comment a little bit about how we should think about that, and if the baseline data are reflective of what you might see outside a clinical trial setting?

So from the pharmacogenetics work, we've determined that it's around -- 60% of the missense mutations can show an effect with this small molecule when tested in cells outside the body. And then in Fabry disease, around 60% to 65% of the mutations are missense. And so, that leaves us to say that about 35% to 40% of the mutations in the world are likely to be amenable to the small molecule in vivo. I think you're referring to the Phase III baseline numbers. In that case, remember that physicians already knew that this was a monotherapy study for pharmacological chaperone. So they already knew to be looking for missense mutations when the mutation was known. So there was already some selection bias based on what they already knew. But they were not selecting patients for screening based strictly on our pharmacogenetics table. So in fact, there were patients that came in with mutations that were not amenable. There were patients who came in with mutations that had never been seen before. We actually found 15 mutations that had never been reported in the literature for a Fabry patient. So it was a larger percentage than you would get if you just randomly selected Fabry patients from the population. That's why the number in the Phase III screening was larger. But our estimate of saying that if you took the entire Fabry population, we still estimate, based on everything, we know that the small molecule should be appropriate for -- as a monotherapy for between 40% and 50% of the Fabry population. And that percentage, we believe, is actually growing with time because with the time it's clear that Fabry disease is probably more common than was previously thought, maybe as large in some studies as 1 in 2,000 or so in the population and these additional patients that are being found with much higher frequency have missense mutations, and those mutations that are being found are more likely to be a minimal missense mutations. So we take the Fabry population as it exists now, probably around 35% or so and with time as more of the later onset patients are being found there with high probability missense and they are higher probability minimal missense and that’s how the percentage grows to upwards at around 50%.

And we have follow-up from the line of Ritu Baral with Canaccord.

Just in reference to the immunogenicity study that you are doing with 2220, ultimately what you do, I guess, think at this point that clinical benefit for 2220 will be based on? Reduction in immunogenicity or increased potency? What do you think will ultimately be suspect -- ultimately to be more important?

I don’t know about more important Ritu. I think that both are very important overall. It’s important to remember that both very related, they go with each other. The immunogenicity of these proteins, especially Lumizyme, isn’t necessarily that it’s not tolerated by patients, even though Lumizyme does, I believe, have a Black Box warning and there is some anaphylaxis associated with it. Typically, that can be managed. The greater concerned around the immune response to these proteins are the antibodies that are triggered can attach to the ERT and take them to non-target tissue, non-target organs. So in case of Pompe, the anti bodies are really rendering much of the ERT not effective in dealing with the course of the disease; it’s taking at the places other than muscle. That’s why it’s so important in these studies that we are doing right now. And again, that we’ve announced just here tonight that we’re going to have the data in Q3, the advantage to open other studies that we’ll be able to categorize; First, what is the immunogenicity and the immune profile of the Lumizyme-ERT? And then secondly, at varying dose concentrations with our chaperone, can we mitigate that immune response? And that’s going to be a very, very important series of findings that will help guide what that next series of clinical studies should be. And let me just add, again we don’t think this is limited just to Lumizyme. We think this is going to be a problem with any Pompe ERT that we see in development today, either in the clinic or the ones that know are in preclinical developments. And again, it’s the same phenomenon, although to maybe to a different degree than we’ve seen with any of the ERTs that we tested in any of the lysosomal storage disorders.

Thank you. This concludes the question-and-answer portion of today’s conference call. Thank you for your participation in Amicus Therapeutics First Quarter 2012 Earnings Conference Call. This does conclude the program and you may now disconnect. Thank you and have a wonderful day.