Zumutor receives the US patent for its novel biologic ZM008

On February 16, 2023 Zumutor reported that it received a notice of allowance from USPTO on its flagship product : ZM008 (Press release, Zumutor Biologics, FEB 16, 2023, View Source [SID1234627328]).

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ZM008 is a novel monoclonal antibody: designed and developed by scientists at Zumutor Biologics.

This Ab will have far reaching impact on patient outcomes in solid cancers especially those with limited standard therapeutic options.

Clinical trials will commence soon in the US.

Ascendis Pharma Reports Full Year 2022 Results

On February 16, 2023 Ascendis Pharma A/S (Nasdaq: ASND) reported financial results for the full year ended December 31, 2022 and provided a business update (Press release, Ascendis Pharma, FEB 16, 2023, View Source [SID1234627326]).

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"Our unique TransCon technology platform and algorithm for product innovation enables us to address major unmet medical needs with a diverse, growing pipeline of highly differentiated product candidates," said Jan Mikkelsen, Ascendis Pharma’s President and Chief Executive Officer. "With our demonstrated ability to take a product from concept through approval and launch, we will continue to focus on building long term value for patients and other stakeholders, establishing Ascendis Pharma as a leading sustainable, profitable biopharma company."

Select Highlights & Anticipated 2023 Milestones

TransCon hGH:
First European SKYTROFA (lonapegsomatropin) commercial launch in Germany on track for the third quarter of 2023.
In the third quarter of 2023, we anticipate completing enrollment in New InsiGHTS, a four-armed Phase 2 trial designed to investigate the safety, tolerability, and efficacy of different dose levels of TransCon hGH in patients with Turner Syndrome.
We expect Phase 3 topline results from foresiGHt in adult growth hormone deficiency in the fourth quarter of 2023.
Fourth quarter 2022 SKYTROFA (lonapegsomatropin-tcgd) U.S. revenue grew to €17.1 million.
TransCon PTH:
U.S. FDA Priority Review continues for use in adult patients with hypoparathyroidism, with a PDUFA date of April 30, 2023. If approved, U.S. commercial launch expected by the end of the second quarter of 2023.
European Commission decision on MAA anticipated during the fourth quarter of 2023. If approved, first European country launch expected in early 2024.
In anticipation of U.S. and EU approvals, commercial, medical affairs, product supply and other teams continue launch readiness activities.
Phase 3 PaTHway Japan trial achieved its primary objectives; topline results consistent with North American and EU trials.
Enrollment opened in January 2023 for U.S. Expanded Access Program.
Q1-2022 Q2-2022 Q3-2022 Q4-2022 2022
SKYTROFA revenue (millions) € 1.9 € 4.4 € 12.3 € 17.1 € 35.7

TransCon CNP:
Announced positive topline data from the Phase 2 ACcomplisH Trial, with results in children with achondroplasia down to 2 years of age; as of February 14, 2023 all 57 patients currently remain in the trial with treatment duration up to 3 years.
During the second quarter of 2023, we expect to complete enrollment in ApproaCH, a global randomized, double-blind, placebo-controlled Phase 2b trial in children ages 2–11 years with achondroplasia. The trial targets enrollment of ~80 patients.
During the third quarter of 2023, we plan to submit an IND or similar in children under the age of two years with achondroplasia.
TransCon TLR7/8 Agonist:
Reported topline data and recommended Phase 2 dose from the dose escalation portion of the Phase 1/2 transcendIT-101 Trial. Early signs of clinical activity were observed in patients receiving TransCon TLR7/8 Agonist as monotherapy or in combination with pembrolizumab.
Enrollment in the dose expansion phase of transcendIT-101 continues, with a focus on investigating TransCon TLR7/8 Agonist in combination with pembrolizumab in four different cancer types.
TransCon IL-2 β/γ:
The Phase 1/2 IL-βelieγe Trial evaluating TransCon IL-2 β/γ monotherapy in patients with locally advanced or metastatic solid tumors continues to enroll patients. Results from monotherapy dose escalation are expected during the first quarter of 2023. Dose escalation combination therapy results expected during the third quarter of 2023.
Preparing to initiate βelieγe-IT-201, a randomized Phase 2 trial of TransCon IL-2 β/γ and TLR7/8 combination therapies, in the second quarter of 2023.
TransCon RBZ:
Ophthalmology selected as the third therapeutic area; TransCon RBZ (ranibizumab) selected as the first investigational pipeline candidate, designed for higher efficacy with 6-month dosing intervals.
Ended the fourth quarter of 2022 with cash, cash equivalents, and marketable securities totaling €742.9 million.
Full-Year 2022 Financial Results

Total revenue for 2022 was €51.2 million compared to €7.8 million in 2021. Revenue for 2022 include SKYTROFA U.S. revenue, and license, clinical supply and services provided to third parties, primarily VISEN Pharmaceuticals. Revenue in 2022 benefited from a full-year contribution of SKYTROFA U.S. revenue of €35.7 million compared to €0.9 million in 2021.

Research and development (R&D) costs for 2022 were €379.6 million compared to €295.9 million in 2021. The higher R&D costs in 2022 reflect a one-time reversal of pre-launch inventories in 2021, following the U.S. FDA approval of SKYTROFA in August 2021. In addition, higher R&D costs in 2022 reflect manufacturing of pre-launch inventories for TransCon PTH and an increase in employee and other costs attributable to organizational growth.

Selling, general, and administrative (SG&A) expenses for 2022 were €221.2 million compared to €160.2 million in 2021. Higher SG&A expenses were primarily due to an increase in commercial and administrative personnel following the launch of SKYTROFA in the U.S. and preparation for future product launches.

Our share of net loss of associate was €17.7 million in 2022, compared to a net gain of €12.0 million in 2021. For 2021, the net profit of associate included a non-cash gain of €42.3 million as a result of a financing round in VISEN.

Net finance income was €1.7 million in 2022 compared to a net finance income of €55.8 million in 2021.

For the full year 2022, Ascendis Pharma reported a net loss of €583.2 million, or €10.40 per share (basic and diluted) compared to a net loss of €383.6 million, or €7.00 per share (basic and diluted) for the same period in 2021.

As of December 31, 2022, Ascendis Pharma had cash, cash equivalents, and marketable securities totaling €742.9 million compared to €789.6 million as of December 31, 2021. As of December 31, 2022, Ascendis Pharma had 57,152,295 ordinary shares outstanding.

Conference Call and Webcast Information

Ascendis Pharma will host a conference call and webcast today at 4:30 pm Eastern Time (ET) to discuss its full year 2022 financial results.

Those who would like to participate may access the live webcast here, or register in advance for the teleconference here. The link to the live webcast will also be available on the Investors & News section of the Ascendis Pharma website at View Source A replay of the webcast will be available on this section of our website shortly after conclusion of the event for 30 days.

Xencor to Report Fourth Quarter and Full Year 2022 Financial Results and Host Conference Call on February 23, 2023

On February 16, 2023 Xencor, Inc. (NASDAQ:XNCR), a clinical-stage biopharmaceutical company developing engineered antibodies and cytokines for the treatment of cancer and autoimmune diseases, reported that it will report financial results for the fourth quarter and full year 2022 after the market closes on Thursday, February 23, 2023 (Press release, Xencor, FEB 16, 2023, View Source [SID1234627324]).

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Xencor management will host a conference call the same day at 4:30 p.m. ET (1:30 p.m. PT) to discuss the financial results and provide a corporate update.

The live webcast will be available under "Events & Presentations" in the Investors section of the Company’s website located at investors.xencor.com and will be archived for at least 30 days. Active participants in the conference call may receive credentials for telephone access by registering at the following link: https://register.vevent.com/register/BI70c60751330540e3909534dca3801239.

First AML Patient Transplanted with Vor Bio’s Trem-cel Demonstrated Durable Engraftment through Multiple MylotargTM Cycles at Initial Dose Level

On February 16, 2023 Vor Bio (Nasdaq: VOR), a clinical-stage cell and genome engineering company, reported clinical data from VBP101, its Phase 1/2a multicenter, open-label, first-in-human study of trem-cel (previously VOR33) in patients with acute myeloid leukemia (AML) (Press release, Vor BioPharma, FEB 16, 2023, View Source [SID1234627323]). In the first patient, trem-cel maintained hematopoesis through three cycles of Mylotarg (gemtuzumab ozogamicin), which was well-tolerated at the initial dose level of 0.5 mg/m2. A second patient has successfully received a trem-cel transplant and engrafted normally. These data were presented today by Miguel-Angel Perales, MD, Chief, Adult Bone Marrow Transplant Service, Memorial Sloan Kettering Cancer Center in a late-breaking poster at the 2023 Tandem Meetings (Transplantation & Cellular Therapy Meetings of ASTCT and CIBMTR) in Orlando, FL.

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"These data continue to demonstrate the potential of trem-cel as a next-generation hematopoietic stem cell transplant, which could transform treatment for patients with blood cancers such as AML," said Dr. Eyal Attar, Vor Bio’s Chief Medical Officer. "We are also encouraged that a second patient successfully received a trem-cel transplant and look forward to learning more as we treat additional patients and dose escalate Mylotarg. In addition, we plan to file an IND for VCAR33ALLO, a novel allogeneic CAR-T treatment which we believe could be more efficacious than Mylotarg in combination with a trem-cel transplant."

Trem-cel Safety & Durability

Patient 1 maintained neutrophil and platelet counts approximately five months (147 days) after transplantation with trem-cel. Due to detectable measurable residual disease (MRD), Patient 1 was moved to other therapies following administration of the third dose of Mylotarg, subsequently relapsed, and remains on study for long-term follow-up.

Similar to Patient 1, Patient 2 successfully received a trem-cel transplant and showed robust cell recovery with neutrophil engraftment occurring at Day 11 and platelet recovery on Day 17. Trem-cel was well tolerated in both patients, with no related and no unexpected adverse events (AEs) reported.

No Hematological Toxicity Observed Through Repeated Doses of Mylotarg

In Patient 1, neutrophil and platelet cell counts were maintained following three sequential Mylotarg doses at 0.5 mg/m2. This suggests potential protection from Mylotarg-related hematotoxicity. The only AE observed possibly related to Mylotarg through dose 3 was low grade nausea and vomiting, a known side-effect of Mylotarg. Mylotarg first-dose pharmacokinetics revealed 0.5 mg/m2 achieved Cmax and AUC parameters equivalent to 1-2 and 4-5 mg/m2 accordingly, potentially due to the decreased CD33 antigen sink.

Evidence of Mylotarg Causing CD33-negative Donor Cell Enrichment

In Patient 1, CD33-negative donor hematopoiesis was enriched across hematopoietic cell types following Mylotarg administration. In addition, the CD33 deletion was observed in donor cells of myeloid and lymphoid origin which were both enriched following Mylotarg, suggesting that CD33 is expressed in early hematopoietic cells and that Mylotarg treatment enriches for edited donor cells.

Interest in enrollment in VBP101 continues to be strong with a high level of investigator enthusiasm at all nine study sites. The company is moving forward with dose escalation of Mylotarg per the 3+3 dose escalation schema in the protocol. The Company is also on-track to submit an IND in the first half of 2023 for VCAR33ALLO, a CAR-T therapy using allogeneic healthy donor-derived cells, which it intends use in combination with trem-cel as a Treatment System.

About AML

AML is the most common type of acute leukemia in adults and one of the deadliest and most aggressive blood cancers, affecting 20,000 newly diagnosed patients each year in the United States. Approximately half of patients with AML who receive a hematopoietic cell transplant (HCT) suffer a relapse of their leukemia, with two-year survival rates of less than 20%, and relapse rates are higher for patients with certain adverse risk features. The fragility of engrafted hematopoietic stem cells prevents treatment following transplant, giving the cancer a chance to return.

About the VBP101 Clinical Trial

VBP101 is a Phase 1/2a, multicenter, open-label, first-in-human study of trem-cel in participants with AML who are undergoing human leukocyte antigen (HLA)-matched allogeneic HCT. Trem-cel is an allogeneic CRISPR/Cas9 genome-edited hematopoietic stem and progenitor cell (HSPC) therapy product, lacking the CD33 protein. It is being investigated for participants with CD33+ AML at high risk for relapse after HCT to allow post-HCT targeting of residual CD33+ acute AML cells using Mylotarg without toxicity to engrafted cells. Participants undergo a myeloablative HCT with matched related or unrelated donor CD34-selected HSPCs engineered to remove CD33 expression (trem-cel drug product). Mylotarg is given after engraftment for up to four cycles. The primary endpoint is the incidence of successful engraftment, defined as the first day of 3 consecutive days of absolute neutrophil count (ANC) ≥500 cells/mm2 by day 28. Part 1 of this study is evaluating the safety of escalating Mylotarg dose levels to determine the maximum tolerated dose (MTD) and recommended Phase 2 dose. Part 2 will expand the number of participants to evaluate the Mylotarg recommended Phase 2 dose. For more information, visit: View Source

About Trem-cel

Tremtelectogene empogeditemcel (trem-cel), formerly VOR33, is a genome-edited hematopoietic stem and progenitor allogeneic donor product candidate where CD33 has been deleted using genome engineering. Transplant with trem-cel is designed to replace standard of care transplants for patients suffering from AML and potentially other blood cancers. Trem-cel has the potential to enable powerful targeted therapies in the post-transplant setting including CD33-targeted CAR-T cells.

Ultragenyx Reports Fourth Quarter and Full Year 2022 Financial Results and Corporate Update

On February 16, 2023 Ultragenyx Pharmaceutical Inc. (NASDAQ: RARE), a biopharmaceutical company focused on the development and commercialization of novel products for serious rare and ultrarare genetic diseases, reported its financial results for the quarter and full year ended December 31, 2022 and reaffirmed its financial guidance for 2023 (Press release, Ultragenyx Pharmaceutical, FEB 16, 2023, View Source [SID1234627322]).

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"In 2022, we continued to reach more patients around the world as we successfully commercialized multiple products to treat rare diseases," said Emil D. Kakkis, M.D., Ph.D., chief executive officer and president of Ultragenyx. "2023 will be a year of meaningful development progress for us as we focus on data generation from two of our key programs, UX143 in osteogenesis imperfecta and GTX-102 in Angelman syndrome, and the continued advancement of our late-stage gene therapy programs."

Fourth Quarter and Full Year 2022 Selected Financial Data Tables and Financial Results

Revenues (dollars in thousands) (unaudited)
Three Months Ended December 31, Year Ended December 31,
2022 2021 2022 2021

Crysvita Collaboration and Product Revenues:
North America Collaboration $ 66,903 $ 50,211 $ 215,024 $ 171,198
ROW Product Sales 7,698 5,272 42,678 21,422
Crysvita in Ultragenyx Territories1 74,601 55,483 257,702 192,620
EU Royalty Revenue 6,058 4,741 21,692 18,195
Total Crysvita Revenue 80,659 60,224 279,394 210,815
Dojolvi 16,412 11,825 55,612 39,560
Mepsevii 4,798 3,111 20,637 16,035
Total Collaboration and Product Revenues 101,869 75,160 355,643 266,410
Daiichi Sankyo 1,479 8,229 7,686 84,996
Total Revenue $ 103,348 $ 83,389 $ 363,329 $ 351,406

Net Revenues
For the fourth quarter of 2022, Ultragenyx reported $103.3 million in total revenue. Ultragenyx recognized $74.6 million in Crysvita (burosumab) revenue in the Ultragenyx territories1, which includes $66.9 million in collaboration revenue in the North American profit-share territory and net product sales in other regions of $7.7 million. Total royalty revenue related to European Crysvita sales was $6.1 million. Dojolvi (triheptanoin) revenue in the fourth quarter of 2022 was $16.4 million. Mepsevii (vestronidase alfa) revenue for the fourth quarter of 2022 was $4.8 million. Total revenue for the fourth quarter also includes $1.5 million of revenue related to the collaboration and license agreement with Daiichi Sankyo that was executed in March 2020.

Revenue for the year ended December 31, 2022 was $363.3 million, including $257.7 million in Crysvita revenue in the Ultragenyx territories1. Crysvita collaboration revenue in the North American profit-share territory was $215.0 million and net Crysvita product sales in other regions were $42.7 million. Total royalty revenue related to European Crysvita royalties was $21.7 million. Dojolvi product revenue for the year ended December 31, 2022 was $55.6 million. Mepsevii product revenue was $20.6 million. For the year ended December 31, 2022, revenue related to our strategic manufacturing partnership with Daiichi Sankyo was $7.7 million.

Selected Financial Data (dollars in thousands, except per share amounts) (unaudited)
Three Months Ended December 31, Year Ended December 31,
2022 2021 2022 2021
Total revenue $ 103,348 $ 83,389 $ 363,329 $ 351,406
Operating expense:
Cost of sales 5,319 3,509 28,320 16,008
Research and development 170,808 123,013 705,789 497,153
Selling, general and administrative 72,849 59,431 278,139 219,982
Total operating expense 248,976 185,953 1,012,248 733,143
Net loss $ (151,833 ) $ (122,458 ) $ (707,421 ) $ (454,025 )
Net loss per share, basic and diluted $ (2.16 ) $ (1.79 ) $ (10.12 ) $ (6.70 )

Operating Expenses
Total operating expenses for the fourth quarter of 2022 were $249.0 million, including non-cash stock-based compensation of $29.4 million. Total operating expenses for the year ended December 31, 2022 were $1,012.2 million, which includes $130.4 million of non-cash stock-based compensation and $75.0 million of in-process research and development expense associated with the acquisition of GeneTx in July 2022. In 2023, annual operating expenses are expected to decrease as the company manages headcount and increases operational leverage while executing on high value programs.

Net Loss
For the fourth quarter of 2022, Ultragenyx reported net loss of $151.8 million, or $2.16 per share basic and diluted, compared with a net loss for the fourth quarter of 2021 of $122.5 million, or $1.79 per share, basic and diluted. For the year ended December 31, 2022, net loss was $707.4 million, or $10.12 per share, basic and diluted, compared with a net loss for the same period in 2021 of $454.0 million, or $6.70 per share, basic and diluted.

Cash, Cash Equivalents and Marketable Debt Securities
Cash, cash equivalents, and marketable debt securities were approximately $896.7 million as of December 31, 2022.

2023 Financial Guidance
For the full year 2023, the company reaffirms:

Total revenue in the range of $425 million to $450 million
Crysvita revenue in the range of $325 million to $340 million. This includes all regions where Ultragenyx will recognize revenue, including the royalties in Europe, which have been ongoing, and the royalties in North America, which will begin in April 2023.
Dojolvi revenue in the range of $65 million to $75 million
Net Cash Used in Operations is expected to be less than $400 million
Recent Updates and 2023 Clinical Milestones

UX143 (setrusumab) monoclonal antibody for Osteogenesis Imperfecta (OI): Phase 2/3 study dosing patients; Phase 2 enrollment complete, data expected in mid-2023
Ultragenyx is currently dosing patients in the Phase 2/3 Orbit study of UX143 in pediatric and adult patients with OI aged five to <26 years. Enrollment in the Phase 2 portion of the study is complete and data are expected in mid-2023. The Phase 2 data are expected to include changes in bone biomarkers response and some data on bone mineral density that will be used to establish the dosing algorithm for the Phase 3 portion of the study. Optimizing the dosing across the age range of the study is intended to support meaningful benefit in the reduction of clinically evident fractures in all age groups studied.

In addition, in the first half of 2023, Ultragenyx intends to initiate a randomized study in OI in children under age five with serious bone disease, comparing bisphosphonates to UX143. Younger pediatric patients with OI often have a much higher fracture rate than other age groups and a greater medical need, driving clinical urgency for better treatment options. Total fractures is expected to be the primary endpoint in the study.

GTX-102 antisense oligonucleotide for Angelman syndrome: Phase 1/2 continues dose exploration
As of January 2023, 23 patients had received loading doses ranging from 2 mg to 10 mg, with maintenance dosing ranging up to 10 mg to 14 mg. Ten patients have had between six and twelve months of exposure to GTX-102 and five patients have been on continuous therapy for more than one year. Based on the current protocols, the dose-finding cohorts in the U.K. and Canada will be followed by two, larger expansion cohorts with approximately 20 patients in each age range. Discussions are currently ongoing with the FDA to harmonize the three regions.

Based on the ongoing observations of encouraging clinical activity in the dose escalation cohorts, screening began for patients in expansion Cohort A (ages 4 to <8 years) and expansion Cohort B (ages 8 to <18 years) in February 2023. Each expansion cohort will enroll approximately 20 patients and will evaluate the same safety, pharmacokinetic, and efficacy measures as the dose escalating cohorts.

There continues to be dose and time dependent clinical activity seen across the study population, including three of the original U.S. patients who have been re-dosed, under the amended or expanded access protocols. The next data update, based on a larger number of patients in the program, is expected later this year.

DTX401 AAV gene therapy for Glycogen Storage Disease Type Ia (GSDIa): Enrollment in Phase 3 study complete
The Phase 3 study has a 48-week primary efficacy analysis period, and the company has enrolled approximately 50 patients eight years of age and older, randomized 1:1 to DTX401 or placebo. The primary endpoint is the reduction in oral glucose replacement with cornstarch while maintaining glucose control. The last patient has entered the baseline screening phase of the Phase 3 study of DTX401, with Phase 3 data expected in the first half of 2024.

DTX301 AAV gene therapy for Ornithine Transcarbamylase (OTC) Deficiency: Phase 3 study enrolling patients
Ultragenyx has randomized and dosed the first patient in the Phase 3 study with additional patients in the approximate 4- to 8-week baseline screening period. The 64-week study will include approximately 50 patients, randomized 1:1 to DTX301 or placebo. The primary endpoints are response as measured by removal of ammonia-scavenger medications and protein-restricted diet and change in 24-hour ammonia levels.

UX701 AAV gene therapy for Wilson Disease: Stage 1 of pivotal clinical study dosing patients; expect interim Stage 1 enrollment completion in mid-2023
The company is dosing patients in the first stage of the pivotal UX701 study under a recently amended protocol that removes placebo from the dose finding stage and enrolls five patients per cohort. During this stage of the study, safety and efficacy of up to three dose levels of UX701 will be evaluated and a dose will be selected for further evaluation in Stage 2. Completion of Stage 1 enrollment is expected in mid-2023 with data expected in early 2024 that would include safety and potentially initial signs of clinical activity.

UX111 AAV gene therapy for UX111 for the treatment of Sanfilippo syndrome type (MPS IIIA): Discussions ongoing with FDA to determine a plan to file for accelerated approval
In May 2022, Ultragenyx announced an exclusive license agreement with Abeona Therapeutics for UX111, formerly ABO-102. Under the terms of the agreement, Ultragenyx assumed responsibility for the UX111 program in exchange for Abeona’s right to receive tiered royalties of up to 10% on net sales, and milestone payments upon the attainment of certain commercial revenue milestones.

A meeting with the FDA to discuss a plan to file for accelerated approval is expected to occur in the first half of 2023.

UX053 mRNA for glycogen storage disease type III (GSDIII): Phase 1/2 single ascending dose cohort enrolled; data in 1H23
Dosing in the single ascending dose stage of the Phase 1/2 study of UX053 for the treatment of GSDIII has been completed. Data from this cohort are expected in the first half of 2023. Based on these analyses and other work, we will then review our plans for the next steps in the program.

1: Ultragenyx territories include the collaboration revenue from the North American profit-share territory (U.S. and Canada) and other regions where revenue from product sales is recognized by Ultragenyx (Latin America, Turkey). This excludes the European territory revenue, which is recognized as non-cash royalty revenue since the rights were sold to Royalty Pharma in December 2019.

Conference Call and Webcast Information

Ultragenyx will host a conference call today, Thursday, February 16, 2023, at 2 p.m. PT/ 5 p.m. ET to discuss the fourth quarter and full year 2022 financial results and provide a corporate update. The live and replayed webcast of the call will be available through the company’s website at View Source To participate in the live call, please register by clicking on the following link (https://register.vevent.com/register/BI4de832f7ef8946e486c3198ed80e759f), and you will be provided with dial in details. The replay of the call will be available for one year.