Aura Biosciences Presents Updated Phase 1b/2 Clinical Data for AU-011 at the 43rd Annual Macula Society Meeting

On February 24, 2020 Aura Biosciences, a clinical-stage biopharmaceutical company developing a new class of tumor targeted therapies for initial application in primary tumors such as ocular and bladder cancers, reported the presentation of updated clinical data from its ongoing Phase 1b/2 clinical trial evaluating the safety and efficacy of light-activated AU-011, the Company’s lead product candidate for the first line treatment of primary choroidal melanoma, at the 43rd Annual Macula Society Meeting, which took place February 19-22, 2020 in San Diego, CA (Press release, Aura Biosciences, FEB 24, 2020, View Source [SID1234554632]).

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"The Phase 1b/2 data being recognized at the Macular Society meeting this year show that AU-011 continues to be well tolerated, with a statistically significant reduction in tumor growth rate and vision preservation," said Amy C. Schefler, M.D., Weill Cornell Medical College and Retina Consultants of Houston.

"The high tumor control rates observed to date, together with the high rate of vision preservation, including in patients at high risk for vision loss with sub-macular and peripapillary tumors continue to support AU-011’s significant potential to change the treatment paradigm for patients battling choroidal melanoma," said Cadmus Rich, MD, Chief Medical Officer and Head of Research and Development of Aura Biosciences. "I am proud of the work the company has done to make a significant progress in this ultra-rare disease."

Updated Results from the Phase 1b/2 Study Evaluating AU-011

Dr. Schefler gave an oral presentation titled, "Study Update of an Ongoing Phase 1b/2 Open-label Clinical Trial of AU-011 for the Treatment of Small to Medium Choroidal Melanoma," which highlighted updated data from the ongoing open-label Phase 1b/2 clinical trial, and described the design of the planned Phase 3 trial that Aura expects to initiate during the second half of 2020.

The updated data demonstrate that multiple administrations of light-activated AU-011 were well-tolerated. Among the patients evaluated for safety (n=52), the most common treatment-related adverse events (AEs) were expected and included anterior chamber inflammation, posterior chamber inflammation and increase in intraocular pressure; all were manageable with standard-of-care treatments and the majority resolved without clinical sequelae. Notably, the posterior inflammation appears to originate within and/or around the tumor which is consistent with AU-011’s mechanism of action of acute tumor necrosis. There were two treatment-related serious AEs (vision loss; 3.6%). Tumor control and vision preservation data continue to be supportive of the planned Phase 3 registration trial.

About Choroidal Melanoma

Choroidal melanoma is a rare and aggressive type of eye cancer. Choroidal melanoma is the most common primary intraocular tumor in adults and develops in the uveal tract of the eye. No targeted therapies are available at present, and current radiotherapy treatments can be associated with severe visual loss and other long-term sequelae such as dry eye, glaucoma, cataracts and radiation retinopathy. The most common current treatment is plaque radiotherapy, which involves surgical placement of a radiation device on the exterior of the eye over the tumor. The alternative is enucleation, or total surgical removal of the eye. Choroidal melanoma metastasizes in approximately 50 percent of cases with liver involvement in 80-90% of cases and, unfortunately, metastatic disease is universally fatal (source: OMF). There is a very high unmet need for a new vision sparing targeted therapy that could enable early treatment intervention for this life-threatening rare disease given the lack of approved therapies, and the comorbidities of radioactive treatment options.

About Light-Activated AU-011

AU-011 is a first-in-class targeted therapy in development for the treatment of primary choroidal melanoma. The therapy consists of proprietary viral-like particle bioconjugates (VPB) that are activated with an ophthalmic laser. The VPBs bind selectively to unique receptors on cancer cells in the eye and are derived from technology originally pioneered by Dr. John Schiller of the Center for Cancer Research at the National Cancer Institute (NCI), recipient of the 2017 Lasker-DeBakey Award. Upon activation with an ophthalmic laser, the drug rapidly and specifically disrupts the cell membrane of tumor cells while sparing key eye structures, which may allow for the potential of preserving patients’ vision and reducing other long-term complications of radiation treatment. AU-011 can be delivered using equipment commonly found in an ophthalmologist’s office and does not require a surgical procedure, pointing to a potentially less invasive, more convenient therapy for patients and physicians. AU-011 for the treatment of choroidal melanoma has been granted orphan drug and fast track designations by the U.S. Food and Drug Administration and is currently in clinical development.

Aeglea BioTherapeutics Reports Fourth-Quarter and Full-Year 2019 Financial Results, Highlights Recent Milestones

On February 24, 2020 Aeglea BioTherapeutics, Inc. (NASDAQ:AGLE), a clinical-stage biotechnology company developing next-generation human enzyme therapeutics as solutions for diseases with high unmet medical need, today reported its fourth-quarter and full-year 2019 financial results, and provided recent corporate and program highlights (Press release, Aeglea BioTherapeutics, FEB 24, 2020, View Source [SID1234554631]).

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"Our accomplishments this past year illustrate how we are working to reimagine the potential of human enzymes as transformative solutions for challenging rare genetic disorders," said Anthony Quinn, M.B Ch.B, Ph.D., president and chief executive officer of Aeglea. "In addition to delivering compelling Phase 1/2 data from our lead program for Arginase 1 Deficiency, we’ve made solid progress with our pivotal trial enrollment and started laying the groundwork for the commercial launch. We have also continued to make significant progress in our pipeline, further demonstrating the effectiveness of our platform in advancing programs in multiple therapeutic areas."

"We begin 2020 with a clear vision and heightened conviction of the impact for patients we can create with human enzyme therapeutics. We believe that we are well positioned to achieve a number of important milestones this year, armed with a strong leadership team with deep discovery, development and commercial expertise and driven by our collective goal to address the needs of the communities we serve," concluded Dr Quinn.

Recent Highlights

Pegzilarginase in Arginase 1 Deficiency

The Company expects to complete enrollment of its global, pivotal Phase 3 Pegzilarginase Effect on Arginase 1 Deficiency Clinical Endpoints (PEACE) trial in the third quarter of 2020 and to provide topline data in the first quarter of 2021.

Based on a recent genetic prevalence analysis of Arginase 1 Deficiency (ARG1-D), and with more than 200 patients already identified worldwide, the Company now estimates an addressable patient population of greater than 2,500, up from an estimate of 1,000 patients based solely on initial insights using newborn screening data.

The Company’s patient-identification strategy, informed by critical insights from disease analysis and trial experience, has already identified more than 100 patients in the United States, representing a 40% penetration into the genetic prevalent population.

ACN00177 in Homocystinuria

In January, Aeglea announced the filing of its Clinical Trial Application (CTA) with the United Kingdom’s Medicines and Healthcare Products Regulatory Agency (MHRA) for ACN00177, a novel engineered human enzyme therapy designed to treat homocystinuria, a serious metabolic disorder that results in elevated levels of plasma homocysteine.

The Company expects to initiate a Phase 1/2 trial for ACN00177 in the second quarter of 2020, with initial human proof of concept in the first quarter of 2021.

The Company estimates an addressable homocystinuria patient population of greater than 5,000.

Upcoming Events

Aeglea will be attending the following investor conferences in the coming quarter. Details of the presentations and webcasts will be announced prior to the events.

19th Annual Needham Healthcare Conference, April 14-15, New York City

H.C. Wainwright 2020 Global Life Sciences Conference, April 20-21, London, United Kingdom

Further, Aeglea leadership looks forward to participating in dialogue about our enzyme therapeutics platform during the following industry events, with additional details forthcoming.

Annual Meeting of the Society for Inherited Metabolic Disorders (SIMD), April 26-29, Austin, Texas

World Orphan Drug Congress USA 2020, April 29 to May 1, Oxon Hill, Maryland

Fourth Quarter and Full Year 2019 Financial Results

As of December 31, 2019, Aeglea had available cash, cash equivalents, marketable securities and restricted cash of $73.4 million. Based on Aeglea’s current operating plan, management believes it has sufficient capital resources to fund anticipated operations through the first quarter of 2021.

Research and development expenses totaled $17.6 million for the fourth quarter of 2019 and $11.8 million for the fourth quarter of 2018. The increase was primarily associated with investing in manufacturing and pre-commercial activities for Aeglea’s lead product candidate, pegzilarginase; ramp-up in toxicology, investigational new drug (IND)-enabling studies, and manufacturing activities for ACN00177 in Homocystinuria; and personnel-related expenses.

Research and development expenses totaled $64.6 million for the year ended December 31, 2019, compared with $36.7 million for the year ended December 31, 2018. The increase was primarily due to investing in manufacturing and pre-commercial activities for Aeglea’s lead product candidate, pegzilarginase; a ramp-up in toxicology, IND-enabling studies, and manufacturing activities for ACN00177 in Homocystinuria; and expanded clinical development activity and personnel-related expenses.

General and administrative expenses totaled $4.3 million for the fourth quarter of 2019 and $3.5 million for the fourth quarter of 2018. This increase was primarily due to additional employee headcount, commercial readiness support, and facilities to support company growth.

General and administrative expenses totaled $15.7 million for the year ended December 31, 2019, compared with $12.6 million for the year ended December 31, 2018. This increase was primarily due to additional employee headcount, compensation, and facilities to support company growth.

Net loss totaled $21.5 million and $14.9 million for the fourth quarter of 2019 and 2018, respectively, with non-cash stock compensation expense of $1.2 million and $1.4 million for the fourth quarter of 2019 and 2018, respectively. Net loss totaled $78.3 million and $44.3 million for the years ended December 31, 2019 and 2018, respectively, with non-cash stock compensation expense of $4.9 million and $4.3 million for the years ended December 31, 2019 and 2018, respectively.

About Pegzilarginase in Arginase 1 Deficiency

Pegzilarginase is an enhanced human arginase that enzymatically lowers levels of the amino acid arginine. Aeglea is developing pegzilarginase for the treatment of patients with Arginase 1 Deficiency (ARG1-D), a rare debilitating disease presenting in childhood with persistent hyperargininemia, severe progressive neurological abnormalities and early mortality. Pegzilarginase is intended for use as an enzyme therapy to reduce elevated blood arginine levels in patients with ARG1-D. Aeglea’s Phase 1/2 and Phase 2 open-label extension (OLE) data for pegzilarginase in patients with ARG1-D demonstrated clinical improvements and sustained lowering of plasma arginine. The Company’s single, global pivotal Phase 3 PEACE trial is designed to assess the effects of treatment with pegzilarginase versus placebo over 24 weeks with a primary endpoint of plasma arginine reduction.

About ACN00177 in Homocystinuria

Aeglea is developing ACN00177 for the treatment of patients with cystathionine beta synthase (CBS) deficiency, also known as Classical Homocystinuria. Homocysteine accumulation plays a key role in multiple progressive and serious disease-related complications, including thromboembolic vascular events, skeletal abnormalities including severe osteoporosis, developmental delay, intellectual disability, lens dislocation and severe myopia. ACN00177 has been designed as a novel recombinant human enzyme, which degrades the amino acid homocysteine and its related homocystine dimer. With this mechanism, ACN00177 is intended to lower the abnormally high blood levels of homocysteine in patients with homocystinuria. Preclinical data demonstrated that ACN00177 improved important disease-related abnormalities and survival in a mouse model of homocystinuria. The Company has submitted a Clinical Trial Application (CTA) with the United Kingdom’s Medicines and Healthcare Products Regulatory Agency (MHRA) and expects to initiate a Phase 1/2 trial in the second quarter of 2020.

Acorda Therapeutics to Present at Cowen Annual Health Care Conference

On February 24, 2020 Acorda Therapeutics, Inc. (NASDAQ: ACOR) reported that Ron Cohen, M.D., Acorda’s President and Chief Executive Officer, will present at the Cowen and Company Annual Health Care Conference on Monday, March 2 at 2:50PM EST (Press release, Acorda Therapeutics, FEB 24, 2020, View Source [SID1234554630]). A live audio webcast of the presentation can be accessed under "Investor Events" in the Investor section of the Acorda website at www.acorda.com, or you may use the link:

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http://wsw.com/webcast/cowen57/acor/

Spherix Announces a Special Dividend Distribution

On February 23, 2020 Spherix Incorporated (Nasdaq: SPEX) reported that on February 21, 2020, a special committee of the Board of Directors of Spherix Incorporated ("Spherix") approved a distribution to Spherix stockholders of 70,000 shares of Hoth Therapeutics, Inc. ("Hoth") held by Spherix (Press release, Spherix, FEB 23, 2020, View Source [SID1234554625]).

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Each Spherix stockholder will be entitled to receive one (1) share of Hoth common stock for every seventy (70) shares of Spherix common stock held as of 5 p.m. Eastern Time on March 4, 2020, the record date. Spherix will not distribute fractional shares of Hoth common stock, and any fractional shares will be rounded down to the nearest whole share.

Spherix stockholders do not need to take any action to receive the shares of Hoth common stock, other than be a shareholder of record on March 4, 2020. Spherix stockholders do not need to pay any consideration for, surrender or exchange shares of Spherix common stock.

Mr. Anthony Hayes, CEO of Spherix stated, "Returning capital to shareholders is an important part of our strategy, and the distribution of a portion of our holdings in Hoth is the first step in this endeavor. We continue to work towards becoming a diversified biopharmaceutical company with a compelling portfolio of potential compounds to develop and commercialize."

WuXi Biologics Congratulates Immutep on Positive Interim Data for IMP-321

On February 23, 2020 WuXi Biologics ("WuXi Bio") (2269.HK), a leading global open-access biologics technology platform company offering end-to-end solutions for biologics discovery, development and manufacturing, reported that its strategic partner Immutep, a biotechnology company developing novel immunotherapy treatments for cancer and autoimmune diseases, on the positive interim data from the company’s ongoing Phase II TACTI-002 study (Press release, WuXi Biologics, FEB 23, 2020, View Source [SID1234554624]).

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The data relates to use of Immutep’s lead product candidate eftilagimod alpha ("efti" or "IMP321"), a soluble LAG-3 protein, as part of a combination treatment with pembrolizumab. The study shows encouraging results that the Overall Response Rate (ORR) of the combination treatment reaches 47% among first line non-small cell lung cancer patients, while that of pembrolizumab monotherapy is only about 20%. The activation of antigen presenting cells (APC) and subsequent T cell recruitment with efti may lead to stronger anti-tumor responses than observed with pembrolizumab alone.

"The results we are seeing are highly encouraging. WuXi Biologics is a key partner for this novel immune oncology approach. As our trials continue to advance, the expertise and quality the WuXi Biologics team delivers will become even more important to Immutep as they provide a robust and reliable manufacturing platform for efti’s global clinical development," commented Mr. Marc Voigt, CEO of Immutep.

"Congratulations to Immutep on making this exciting progress," said Dr. Chris Chen, CEO of WuXi Biologics, "We are honored to enable global innovative biotechnology companies such as Immutep to advance molecules from idea to commercialization, and will continue to deliver highest quality biologics through our robust and high quality global supply chain network to expedite clinical development of this program to benefit patients worldwide."

Empowered by WuXi Biologics’ proprietary technology platforms, Immutep, which had only four employees initially, has been expediting clinical trials of the innovative IMP321 in Europe and the United States since 2010. In November 2016, the two companies signed an MOU to form a strategic biologics development and manufacturing partnership for IMP321.