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Phase I/II Clinical Study to Evaluate the Safety, Tolerability and Efficacy of LY-M003 Injection in Adult Patients With Wilson's Disease

tiistai 30. kesäkuuta 2026 päivittänyt: Lingyi Biotech Co., Ltd.

A Multicenter, Open, Single-arm, Single-dose, Phase I/II Study Evaluating the Safety, Tolerability, and Efficacy of LY-M003 Injection in Adult Patients With Wilson's Disease

This is a multicenter, open-label, single-arm, single-dose Phase I/II clinical study. It aims to evaluate the safety, tolerability, efficacy, immunogenicity, pharmacodynamic (PD) and pharmacokinetic (PK) profiles of LY-M003 Injection in patients with Wilson's Disease (WD).

Tutkimuksen yleiskatsaus

Yksityiskohtainen kuvaus

This is a multicenter, open-label, single-arm, single-dose Phase I/II clinical study. It aims to evaluate the safety, tolerability, efficacy, immunogenicity, pharmacodynamic (PD) and pharmacokinetic (PK) profiles of LY-M003 Injection in patients with Wilson's Disease (WD). The Phase I/II study consists of a main study phase and a long-term follow-up phase. The main study phase includes an 8-week screening period and a 52-week follow-up period after infusion. The long-term follow-up phase starts at Week 53 and lasts until 5 years post-infusion.

The study pre-specified two dose cohorts, consisting of one main dose cohort and one de-escalation dose cohort: Dose Cohort 1 (4.0 × 10¹³ vg/kg) and the de-escalation dose cohort (2.0 × 10¹³ vg/kg). Three subjects will be enrolled in each dose cohort.Dose Cohort 1 serves as the starting dose. After the first subject receives the study drug, a minimum 28-day DLT observation period must be completed to confirm safety before enrolling subsequent subjects.All three subjects in Dose Cohort 1 have completed the 28-day (Day 28) DLT observation period following LY-M003 infusion. The Safety Review Committee (SRC) will make a comprehensive assessment to determine whether to proceed to the dose expansion phase at the dose level of 4.0 × 10¹³ vg/kg.

Opintotyyppi

Interventio

Ilmoittautuminen (Arvioitu)

18

Vaihe

  • Vaihe 2
  • Vaihe 1

Yhteystiedot ja paikat

Tässä osiossa on tutkimuksen suorittajien yhteystiedot ja tiedot siitä, missä tämä tutkimus suoritetaan.

Opiskeluyhteys

Opiskelupaikat

    • Zhejiang
      • Hangzhou, Zhejiang, Kiina, 310003
        • The First Affiliated Hospital, Zhejiang University School of Medicine
        • Päätutkija:
          • Chaohui Yu, PhD
        • Ottaa yhteyttä:
          • Chaohui YU, PhD
          • Puhelinnumero: +86 13957161659
          • Sähköposti: ych623@sina.com

Osallistumiskriteerit

Tutkijat etsivät ihmisiä, jotka sopivat tiettyyn kuvaukseen, jota kutsutaan kelpoisuuskriteereiksi. Joitakin esimerkkejä näistä kriteereistä ovat henkilön yleinen terveydentila tai aiemmat hoidot.

Kelpoisuusvaatimukset

Opintokelpoiset iät

  • Aikuinen

Hyväksyy terveitä vapaaehtoisia

Ei

Kuvaus

Inclusion Criteria:

  1. The subject fully comprehends the purpose, design, methods and possible adverse events of the study, agrees to participate voluntarily and signs the informed consent form (ICF).
  2. Patients with confirmed diagnosis of Wilson's disease (WD).
  3. Subjects with Wilson's disease (WD) confirmed by laboratory testing to have biallelic ATP7B gene mutation or deletion.
  4. The subjects are treated patients with Wilson's disease (WD) who have received standard therapy (e.g., D-penicillamine or zinc acetate) continuously for at least 6 months prior to screening.
  5. Subjects have maintained a low-copper diet for at least 6 consecutive months prior to screening and will continue this dietary restriction throughout the study.
  6. Subjects must agree to refrain from donating blood, organs, tissues or cells at any time after treatment.
  7. Female subjects of childbearing potential (WOCBP) must have a negative pregnancy test.
  8. Subjects and their partners must have no plans for pregnancy from screening through 6 months after study completion, and will voluntarily use effective contraception (e.g., abstinence, condoms). Subjects shall not plan to donate sperm or ova.

Exclusion Criteria:

  1. AAV8 neutralizing antibody titer > 1:10 .
  2. History of active gastrointestinal bleeding within the past 3 months.
  3. Decompensated liver cirrhosis or advanced liver disease presenting with portal hypertension, ascites, splenomegaly, esophageal varices, hepatic encephalopathy, etc.
  4. Subjects with other concomitant liver diseases as judged by the investigator, including autoimmune hepatitis, alcoholic liver disease, primary biliary cholangitis, primary sclerosing cholangitis, and/or drug- or toxin-induced liver disease.
  5. Subjects with severe hypersplenism complicated and requiring splenectomy as assessed by the investigator.
  6. Model for End-Stage Liver Disease (MELD) score > 13.
  7. Other disorders of copper metabolism, such as chronic cholestatic liver diseases, disorders of glycosylation, copper metabolism disorders, etc.
  8. A history of non-compliance with copper chelators or zinc agents as assessed by the investigator within 6 months prior to screening.
  9. Previously treated WD subjects with ALT and/or AST levels more than 5 times the upper limit of normal (ULN).
  10. Subjects with severe neurological deficits or impairments that, in the investigator's judgment, compromise their safety and/or ability to participate in the study.
  11. Hemoglobin < 90g/L.
  12. Subjects with positive hepatitis B surface antigen (HBsAg), positive hepatitis C virus (HCV) antibody, positive human immunodeficiency virus (HIV) antibody or positive treponema pallidum antibody.
  13. Subjects with end-stage renal disease on dialysis (Chronic Kidney Disease Stage 3 and above), or creatinine clearance < 60 mL/min.
  14. Severe hyperlipidemia (triglycerides >1000 mg/dL);
  15. Subjects who have received or plan to undergo bone marrow transplantation, hematopoietic stem cell transplantation and/or major organ transplantation, including but not limited to liver transplantation and renal transplantation.
  16. Subjects with clinically diagnosed severe cardiovascular diseases or those deemed by the investigator to have such conditions (e.g., New York Heart Association [NYHA] heart failure classification ≥ Class 3).
  17. Subjects with uncontrolled concomitant diseases or infectious diseases as assessed by the investigator.
  18. Subjects who are allergic to any ingredient of LY-M003 Injection.
  19. Prior receipt of any type of gene therapy or cell therapy.
  20. Use of systemic immunosuppressants or steroids within 3 months prior to administration (except for prophylactic immunosuppressive therapy specified in the protocol).
  21. History of cancer within 5 years prior to screening, excluding completely resected non-melanoma skin cancer, non-metastatic prostate cancer and fully cured ductal carcinoma in situ.
  22. Received live attenuated vaccines within 4 months prior to screening, or planned to receive such vaccines during the clinical trial.
  23. Received treatment or intervention with other investigational drugs or study devices within 28 days or 5 half-lives (for drugs only) prior to screening, whichever is longer.
  24. Pregnant women (or women planning pregnancy) or breastfeeding women.
  25. Other conditions that, in the investigator's opinion, render the subject ineligible for study participation.

Opintosuunnitelma

Tässä osiossa on tietoja tutkimussuunnitelmasta, mukaan lukien kuinka tutkimus on suunniteltu ja mitä tutkimuksella mitataan.

Miten tutkimus on suunniteltu?

Suunnittelun yksityiskohdat

  • Ensisijainen käyttötarkoitus: Hoito
  • Jako: Ei satunnaistettu
  • Inventiomalli: Peräkkäinen tehtävä
  • Naamiointi: Ei mitään (avoin tarra)

Aseet ja interventiot

Osallistujaryhmä / Arm
Interventio / Hoito
Kokeellinen: Phase 1: LY-M003 Dose group 1
Phase 1: Participants receive a single, peripheral intravenous (IV) infusion of LY-M003 at dose group 1.The dose for Dose Group 1 is 4.0 × 10¹³ vg/kg.All participants are administered the drug once via intravenous injection.
Phase 1: Participants receive a single, peripheral intravenous (IV) infusion of LY-M003 at de-escalation dose.The dose for de-escalation dose is 2.0 × 10¹³ vg/kg.All participants are administered the drug once via intravenous injection.
Phase 2: Participants receive a single, peripheral intravenous (IV) infusion of LY-M003 at dose group 1.The dose for dose group 1 is 4.0 × 10¹³ vg/kg.All participants are administered the drug once via intravenous injection.
Kokeellinen: Phase 1: LY-M003 de-escalation dose
Phase 1: Participants receive a single, peripheral intravenous (IV) infusion of LY-M003 at dose group 1.The dose for Dose Group 1 is 4.0 × 10¹³ vg/kg.All participants are administered the drug once via intravenous injection.
Phase 1: Participants receive a single, peripheral intravenous (IV) infusion of LY-M003 at de-escalation dose.The dose for de-escalation dose is 2.0 × 10¹³ vg/kg.All participants are administered the drug once via intravenous injection.
Phase 2: Participants receive a single, peripheral intravenous (IV) infusion of LY-M003 at dose group 1.The dose for dose group 1 is 4.0 × 10¹³ vg/kg.All participants are administered the drug once via intravenous injection.
Kokeellinen: Phase 2: LY-M003 Dose group 1
Phase 1: Participants receive a single, peripheral intravenous (IV) infusion of LY-M003 at dose group 1.The dose for Dose Group 1 is 4.0 × 10¹³ vg/kg.All participants are administered the drug once via intravenous injection.
Phase 1: Participants receive a single, peripheral intravenous (IV) infusion of LY-M003 at de-escalation dose.The dose for de-escalation dose is 2.0 × 10¹³ vg/kg.All participants are administered the drug once via intravenous injection.
Phase 2: Participants receive a single, peripheral intravenous (IV) infusion of LY-M003 at dose group 1.The dose for dose group 1 is 4.0 × 10¹³ vg/kg.All participants are administered the drug once via intravenous injection.

Mitä tutkimuksessa mitataan?

Ensisijaiset tulostoimenpiteet

Tulosmittaus
Toimenpiteen kuvaus
Aikaikkuna
Phase I:The incidence of dose-limiting toxicity (DLT) events adjudicated by the Safety Review Committee (SRC) within at least 28 days after LY-M003 infusion.
Aikaikkuna: Within 28 days after infusion of LY-M003 Injection
The incidence of DLT will be assessed using CTCAE 6.0.
Within 28 days after infusion of LY-M003 Injection
The incidence of treatment-emergent adverse events (AEs), adverse events of special interest (AESIs) and serious adverse events (SAEs) related to LY-M003 within 52 weeks after infusion.
Aikaikkuna: 5 years
Assessment will be conducted via 12-lead ECG, laboratory tests(blood routine, blood biochemistry, coagulation function, stool routine, urine routine), vital signs (blood pressure, pulse, respiratory rate, body temperature) and physical examination.
5 years

Toissijaiset tulostoimenpiteet

Tulosmittaus
Toimenpiteen kuvaus
Aikaikkuna
Percentage reduction in the dosage of standard of care (SoC) medications within 52 weeks after administration.
Aikaikkuna: Within 52 weeks after administration
Assessment will be based on the percentage of participants who underwent standard dose reduction after infusion.
Within 52 weeks after administration
Number and proportion of subjects who discontinued standard of care (SoC) medications within 52 weeks after administration.
Aikaikkuna: Within 52 weeks after administration
Assessment will be based on the number and percentage of participants who discontinued standard treatment medications.
Within 52 weeks after administration
Duration (in treatment weeks) of consecutive discontinuation of SoC medications among the above subjects.
Aikaikkuna: Within 52 weeks after administration
Assessment will be based on the number of consecutive cycles during which participants discontinued standard medications.
Within 52 weeks after administration
Change in serum non-caeruloplasmin-bound copper (NCC) from baseline within 52 weeks after administration
Aikaikkuna: Within 52 weeks after administration
Assessment will be based on changes in serum non-ceruloplasmin-bound copper from baseline.
Within 52 weeks after administration
Change in serum ceruloplasmin level from baseline within 52 weeks after administration
Aikaikkuna: Within 52 weeks after administration
Assessment will be based on changes in serum ceruloplasmin level from baseline.
Within 52 weeks after administration
Change in serum ceruloplasmin activity level from baseline within 52 weeks after administration
Aikaikkuna: Within 52 weeks after administration
Assessment will be based on changes in erum ceruloplasmin activity level from baseline.
Within 52 weeks after administration
Change in 24-hour urinary copper content from baseline at each follow-up time point within 52 weeks after administration
Aikaikkuna: Within 52 weeks after administration
Assessment will be based on changes in 24-hour urinary copper content from baseline.
Within 52 weeks after administration
Change in serum total copper from baseline within 52 weeks after administration
Aikaikkuna: Within 52 weeks after administration
Assessment will be based on changes in serum total copper from baseline
Within 52 weeks after administration
Change from baseline in total score of the neurological subscale of the Unified Wilson's Disease Rating Scale (UWDRS) within 52 weeks after administration
Aikaikkuna: Within 52 weeks after administration
Assess the change from baseline in the total score of Part I of the Unified Wilson's Disease Rating Scale (UWDRS). Part I of the UWDRS is the neurological subscale, with a total score ranging from 0 to 208. Higher scores indicate more severe disease and poorer functional status.
Within 52 weeks after administration
Change from baseline in hepatic subscale score of the Unified Wilson's Disease Rating Scale (UWDRS) within 52 weeks after administration
Aikaikkuna: Within 52 weeks after administration
Assess the change from baseline in the total score of Part II of the Unified Wilson's Disease Rating Scale (UWDRS). Part II is the hepatic subscale, with a total score ranging from 0 to 36. Higher scores indicate more severe disease and poorer functional status.
Within 52 weeks after administration
Change from baseline in psychiatric subscale score of the Unified Wilson's Disease Rating Scale (UWDRS) within 52 weeks after administration
Aikaikkuna: Within 52 weeks after administration
Assess the change from baseline in the total score of Part III of the Unified Wilson's Disease Rating Scale (UWDRS). Part III is the psychiatric subscale, with a total score ranging from 0 to 76. Higher scores indicate more severe disease and poorer functional status.
Within 52 weeks after administration
Change from baseline in individual item/subscale scores (speech, handwriting, standing and gait) of the Unified Wilson's Disease Rating Scale (UWDRS) within 52 weeks after administration
Aikaikkuna: Within 52 weeks after administration
Assessment will be based on changes from baseline in individual item/subscale scores (speech, handwriting, standing and gait) of the Unified Wilson's Disease Rating Scale (UWDRS).The total score for speech, writing, standing and gait each ranges from 0 to 4. Higher scores indicate more severe conditions.
Within 52 weeks after administration
Change in ALT and AST from baseline within 52 weeks after administration
Aikaikkuna: Within 52 weeks after administration
Assessment will be based on changes in ALT and AST from baseline
Within 52 weeks after administration
Change in liver stiffness from baseline within 52 weeks after administration
Aikaikkuna: Within 52 weeks after administration

Assessment will be based on changes in liver stiffness from baseline.Liver stiffness is measured by liver and gallbladder ultrasonography combined with ultrasound elastography, with the unit of kPa.

  • <5.8 kPa: No liver fibrosis (F0)
  • 5.8 ~ 6.8 kPa: Mild liver fibrosis (F1)
  • 6.8 ~ 9.1 kPa: Moderate liver fibrosis (F2)
  • 9.1 ~ 10.3 kPa: Severe liver fibrosis (F3)
  • >10.3 kPa: Liver cirrhosis (F4) Higher values indicate more advanced liver fibrosis or cirrhosis.
Within 52 weeks after administration
Change in Kayser-Fleischer (K-F) rings from baseline within 52 weeks after administration
Aikaikkuna: within 52 weeks after administration
Assessment will be based on changes in Kayser-Fleischer (K-F) rings from baseline
within 52 weeks after administration
Long-term follow-up study: Percentage reduction in standard of care (SoC) medication dosage after 52 weeks of administration
Aikaikkuna: Weeks 53 to 5 years after LY-M003 infusion
Assessment will be based on the percentage of participants who underwent standard dose reduction after infusion
Weeks 53 to 5 years after LY-M003 infusion
Long-term follow-up study: Number and proportion of subjects who discontinued standard of care (SoC) medications after 52 weeks of treatment
Aikaikkuna: Weeks 53 to 5 years after LY-M003 infusion
Assessment will be based on the number and percentage of participants who discontinued standard treatment medications.
Weeks 53 to 5 years after LY-M003 infusion
Long-term follow-up study: Number of consecutive treatment weeks during which the above-mentioned subjects remained off SoC medications
Aikaikkuna: Weeks 53 to 5 years after LY-M003 infusion
Assessment will be based on the number of consecutive cycles during which participants discontinued standard medications.
Weeks 53 to 5 years after LY-M003 infusion
Long-term follow-up study: Changes in serum ceruloplasmin activity levels from baseline after 52 weeks of treatment
Aikaikkuna: Weeks 53 to 5 years after LY-M003 infusion
Assessment will be based on changes in erum ceruloplasmin activity level from baseline.
Weeks 53 to 5 years after LY-M003 infusion
Long-term follow-up study: Changes in serum ceruloplasmin concentration levels from baseline following 52 weeks of treatment
Aikaikkuna: Weeks 53 to 5 years after LY-M003 infusion
Assessment will be based on changes in serum ceruloplasmin level from baseline.
Weeks 53 to 5 years after LY-M003 infusion
Long-term follow-up study: Changes in non-ceruloplasmin bound copper (NCC) in serum from baseline after 52 weeks of treatment
Aikaikkuna: Weeks 53 to 5 years after LY-M003 infusion
Assessment will be based on changes in serum non-ceruloplasmin-bound copper from baseline
Weeks 53 to 5 years after LY-M003 infusion
Long-term follow-up study: Changes in total serum copper from baseline after 52 weeks of treatment
Aikaikkuna: Weeks 53 to 5 years after LY-M003 infusion
Assessment will be based on changes in serum total copper from baseline
Weeks 53 to 5 years after LY-M003 infusion
Long-term follow-up study: Changes of 24-hour urinary copper levels from baseline at all follow-up visits after 52 weeks of treatment
Aikaikkuna: Weeks 53 to 5 years after LY-M003 infusion
Assessment will be based on changes in 24-hour urinary copper content from baseline.
Weeks 53 to 5 years after LY-M003 infusion
Long-term follow-up study: Changes in quantitative neurological function scores of the Unified Wilson's Disease Rating Scale (UWDRS) from baseline after 52 weeks of treatment
Aikaikkuna: Weeks 53 to 5 years after LY-M003 infusion
Assess the change from baseline in the total score of Part I of the Unified Wilson's Disease Rating Scale (UWDRS). Part I of the UWDRS is the neurological subscale, with a total score ranging from 0 to 208. Higher scores indicate more severe disease and poorer functional status.
Weeks 53 to 5 years after LY-M003 infusion
Long-term follow-up study: Changes in hepatic subscale quantitative scores of the UWDRS from baseline after 52 weeks of treatment
Aikaikkuna: Weeks 53 to 5 years after LY-M003 infusion
Assess the change from baseline in the total score of Part II of the Unified Wilson's Disease Rating Scale (UWDRS). Part II is the hepatic subscale, with a total score ranging from 0 to 36. Higher scores indicate more severe disease and poorer functional status.
Weeks 53 to 5 years after LY-M003 infusion
Long-term follow-up study: Changes in psychiatric symptom subscale quantitative scores of the UWDRS from baseline after 52 weeks of treatment
Aikaikkuna: Weeks 53 to 5 years after LY-M003 infusion
Assess the change from baseline in the total score of Part III of the Unified Wilson's Disease Rating Scale (UWDRS). Part III is the psychiatric subscale, with a total score ranging from 0 to 76. Higher scores indicate more severe disease and poorer functional status.
Weeks 53 to 5 years after LY-M003 infusion
Long-term follow-up study: Changes from baseline in individual item and subscale scores (Speech, Handwriting, Standing and Gait) of the quantitative UWDRS after 52 weeks of treatment
Aikaikkuna: Weeks 53 to 5 years after LY-M003 infusion
Assessment will be based on changes from baseline in individual item/subscale scores (speech, handwriting, standing and gait) of the Unified Wilson's Disease Rating Scale (UWDRS).The total score for speech, writing, standing and gait each ranges from 0 to 4. Higher scores indicate more severe conditions.
Weeks 53 to 5 years after LY-M003 infusion
Long-term follow-up study: Changes in ALT and AST levels from baseline after 52 weeks of treatment
Aikaikkuna: Weeks 53 to 5 years after LY-M003 infusion
Assessment will be based on changes in ALT and AST from baseline
Weeks 53 to 5 years after LY-M003 infusion
Long-term follow-up study: Changes in liver stiffness measurements from baseline after 52 weeks of treatment
Aikaikkuna: Weeks 53 to 5 years after LY-M003 infusion

Assessment will be based on changes in liver stiffness from baseline.Liver stiffness is measured by liver and gallbladder ultrasonography combined with ultrasound elastography, with the unit of kPa.

  • <5.8 kPa: No liver fibrosis (F0)
  • 5.8 ~ 6.8 kPa: Mild liver fibrosis (F1)
  • 6.8 ~ 9.1 kPa: Moderate liver fibrosis (F2)
  • 9.1 ~ 10.3 kPa: Severe liver fibrosis (F3)
  • >10.3 kPa: Liver cirrhosis (F4) Higher values indicate more advanced liver fibrosis or cirrhosis.
Weeks 53 to 5 years after LY-M003 infusion
Long-term follow-up study: Changes in Kayser-Fleischer (K-F) ring grading from baseline after 52 weeks of treatment
Aikaikkuna: Weeks 53 to 5 years after LY-M003 infusion
Assessment will be based on changes in Kayser-Fleischer (K-F) rings from baseline
Weeks 53 to 5 years after LY-M003 infusion

Yhteistyökumppanit ja tutkijat

Täältä löydät tähän tutkimukseen osallistuvat ihmiset ja organisaatiot.

Tutkijat

  • Päätutkija: Chaohui Yu, PhD, Zhejiang University

Opintojen ennätyspäivät

Nämä päivämäärät seuraavat ClinicalTrials.gov-sivustolle lähetettyjen tutkimustietueiden ja yhteenvetojen edistymistä. National Library of Medicine (NLM) tarkistaa tutkimustiedot ja raportoidut tulokset varmistaakseen, että ne täyttävät tietyt laadunvalvontastandardit, ennen kuin ne julkaistaan ​​julkisella verkkosivustolla.

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