TABLE 1.
Summary of the abnormalities expressed by primary myelofibrosis (PMF) patients that were first identified in Gata1low mice and that represent potential druggable targets to cure the disease
| Abnormality | Gata1low mice | PMF patients | |
|---|---|---|---|
| HSC | ↑CXCR4 | 39 | 39–42 |
| ↑Mobilization | 39 | 43 | |
| Megakaryocytes | ↑TGF-β | 27, 44 | 44–47 |
| ↑P-selectin | 27 | 48 | |
| ↑LOX2 | 34 | 34 | |
| ↑Neutrophil emperipolesis | 31 | 45 | |
| ↑Parapoptosis | 31 | 49 | |
| Neutrophils | ↑DNA trap | 50 | 51 |
| ↑Emperipolesis | 31 | 45 | |
| Bone marrow microenvironment | ↑TGF-β bioavailability | 44 | 44 |
| ↑Activated fibrocytes | 27 | 52 | |
| ↑Collagen fibers | 33, 35 | 1, 2 | |
| ↓Normal HSC | 27, 44 | 53, 54 | |
| T cells | ↓Activation | 50 | 55, 56 |
| Spleen microenvironment | ↑Malignant HSC | 27, 44 | 53, 54 |
| ↑Megakaryocytes | 33 | 1, 2 | |
| ↑Activated fibrocytes | 27, 44 | 44 | |
| ↑Abnormal endothelial cells | 33 | 57 | |
| ↑TGF-β bioavailability | 44 | 44 | |
| Plasma/serum | ↑TPO | 22 | - |
| ↑SDF-1 | 39 | 41, 58 | |
| ↑Bioactive TGF-β | 44 | 56 | |
| ↑Total TGF-β | 44 | 55, 56 | |
| ↑Antimitochondrial antibodies | - | 50 |