Sickle cell anemia
GtoPdb Disease Summaries
This section gives an overview of the disease, and where available shows the following:
- Synonyms: Shows known synonyms for the disease.
- Description: Gives a basic description/definition of the disease.
- Database Link: External links to the same disease at the Disease Ontology, OMIM or Orphanet sites.
- Immunopharmacology comments: General comments about the target's role in immunopharmacology, provided by GtoImmuPdb curators.
- Associated with: Counts are displayed for the total targets the disease is associated with in GtoPdb. The counts of targets and ligands of immunological relevance associated to the disease are also shown.
More information can be found in the help pages.
✖| Disease ID: | 862 | |
| Name: | Sickle cell anemia | |
| Associated with: | 2 targets | |
| Database Links |
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Disease Ontology:
DOID:10923 |
Targets
GtoPdb Disease Summaries - Targets
Click on the target name to link to its detailed view page
Where available, information is display on the role of the target in the disease; drugs which target the disease and their therapeutic use and side-effects.
If there is mutation data curated in GtoPdb this is indicated, with a link back to the appropriate section on the target detailed view page
Immuno ligand interactions - If available, a table of immuno-relevant ligands is shown. These ligands have been curated as having an association to the disease and possess interaction data with the target in GtoPdb. The approval status of the ligand is shown, along with curator comments and an indication of whether the target is considered the primary target of the ligand.
More information can be found in the help pages.
✖| KCa3.1 | |
| Role: | KCa3.1 is involved in erythrocyte dehydration during sickling |
| Drugs: | Clotrimazole, senicapoc |
| Therapeutic use: | Phase 3 clinical trials for ICA-17043 (senicapoc) were stopped in 2007 apparently due to lack of efficacy in reducing the incidence of sickling crisis |
| References: | 1-3 |
| selectin P (CD62) | |
| Role: | The P-selectin/P-selectin glycoprotein ligand 1 (PSGL-1) interaction is involved in cell adhesion, largely between leukocytes, platelets and vascular endothelial cells. In Sickle cell disease P-selectin/PSGL-1 complexes promote abnormal adhesion of sickled erythrocytes to activated endothelial cells which precipitates vascular occlusion and pain crises. |
| Drugs: | Crizanlizumab |
| Comments: | Crizanlizumab binds to P-selectin and blocks interaction with PSGL-1, as a mechanism to reduce the frequency of vaso-occlusive crises in patients with sickle cell disease. |
| References: | 4 |
Ligands
GtoPdb Disease Summaries - Ligands
Click ligand name to view ligand summary page
- Approved: If the ligand is an approved drug this is indicated, along with approval bodies.
- Immuno: Immuno icon indicates the ligand is immuno-relevant
Click the arrow in the final column to expand comments
- Immuno Disease Comments: Curatorial comments specifically added as part of GtoImmuPdb. They give more information on the association between the ligand and disease in the context of immunopharmacology.
- Clinical Use: General clinical comments relating to the ligand and may not necessarily be specific to the disease in question. With hyperlink to more details on the ligand summary pages.
- Bioactivty Comments: Curatorial comments specifically about the compounds biological activity - with hyperlink to more details on the ligand summary pages.
More information can be found in the help pages.
✖No ligand related data available for Sickle cell anemia
References
1. Ataga KI, Orringer EP, Styles L, Vichinsky EP, Swerdlow P, Davis GA, Desimone PA, Stocker JW. (2006) Dose-escalation study of ICA-17043 in patients with sickle cell disease. Pharmacotherapy, 26 (11): 1557-64. [PMID:17064199]
2. Brugnara C, de Franceschi L, Alper SL. (1993) Inhibition of Ca(2+)-dependent K+ transport and cell dehydration in sickle erythrocytes by clotrimazole and other imidazole derivatives. J Clin Invest, 92 (1): 520-6. [PMID:8326017]
3. Brugnara C, Gee B, Armsby CC, Kurth S, Sakamoto M, Rifai N, Alper SL, Platt OS. (1996) Therapy with oral clotrimazole induces inhibition of the Gardos channel and reduction of erythrocyte dehydration in patients with sickle cell disease. J Clin Invest, 97 (5): 1227-34. [PMID:8636434]
4. Kaur K, Kennedy K, Liles D. (2023) Crizanlizumab in sickle cell disease. Pain Manag, [Epub ahead of print]. [PMID:37850353]
