Briquilimab  (Synonyms: JSP-191; AMG-191)

Briquilimab (JSP-191 or AMG-191) is a humanized IgG1 monoclonal antibody that binds human CD117 (c-Kit). Briquilimab blocks the interaction between CD117 receptor and stem cell factor on various CD117 expressing tissues. Briquilimab can lead to inhibition of SCF/c-Kit signaling and MC apoptosis. Briquilimab is a non-toxic approach to target and deplete HSC, enabling blood and immune reconstitution with minimal toxicity with the other agents being used for transient immune suppression to prevent immunologic rejection. Briquilimab can be used in various disease research such as severe combined immunodeficiency (SCID), myelodyplastic syndromes (MDS), acute myeloid leukemia (AML), chronic spontaneous urticarial (CSU), chronic inducible urticarial (CIndU) and asthema^[1][2].

CD117 (c-Kit)^[1]

Briquilimab (75 μg，静脉注射，单次给药) 在移植了低危 MDS 患者造血干细胞的小鼠模型中显著消耗了低危 MDS 造血干细胞^[3]。
Briquilimab (0.3-1 mg/kg，静脉注射，单次给药) 在 2 周后消耗了所有动物体内的人类造血干细胞，但不同 NSG 小鼠模型的消耗程度有所不同^[4]。
Briquilimab (0.1-25 mg/kg，静脉注射，单次给药) 在免疫功能正常的幼年食蟹猴中未观察到不良临床观察结果^[4]。

MCE has not independently confirmed the accuracy of these methods. They are for reference only.

ELISA, FACS, Functional assay

Unconjugated

The product can be reconstituted/diluted with sterile PBS or saline.

145.61 kDa

2574591-89-0

液体

Colorless to light yellow

Shipping with dry ice.

Please store the product under the recommended conditions in the Certificate of Analysis.

• 
  Human IgG1 kappa

• 
  Loaded Briquilimab on AHC2 biosensor, can bind CD117/c-kit Protein, Human (HEK293, His, HY-P72752) with an affinity constant of 5.634E-09 M as determined in BLI assay.
• 
  Immobilized Human c-Kit Protein (ECD, His Tag) can bind Briquilimab. The EC₅₀ for this effect is 1.72 ng/mL.

• [1]. Rajni Agarwal, et al., First Report of Non-Genotoxic Conditioning with JSP191 (anti-CD117) and Hematopoietic Stem Cell Transplantation in a Newly Diagnosed Patient with Severe Combined Immune Deficiency, Blood, Volume 136, Supplement 1, 2020, Page 10, ISSN 0006-4971.

  [2]. Dawoon Jung, et al., Pharmacokinetics of Briquilimab as a Conditioning Agent for Hematopoietic Stem Cell Transplantation in Patients With Severe Combined Immunodeficiency, Myelodysplastic Syndrome, or Acute Myeloid Leukemia, Transplantation and Cellular Therapy, Volume 30, Issue 9, 2024, Pages 923.e1-923.e9, ISSN 2666-6367

  [3]. Pang, W. W., et al., (2019). Anti-CD117 antibody depletes normal and myelodysplastic syndrome human hematopoietic stem cells in xenografted mice. Blood, 133(19), 2069–2078.  [Content Brief]

  [4]. Kwon, H. S., et al., (2019). Anti-human CD117 antibody-mediated bone marrow niche clearance in nonhuman primates and humanized NSG mice. Blood, 133(19), 2104–2108.  [Content Brief]