GeneID: 482
Names
| Common Name: | PGC-1α | Type: | Gene |
|---|---|---|---|
| Chromosome: | 4 (NC_000004.12) | Locus: | NG_028250.2 (PPARGC1A) |
| HUGO Symbol: | PPARGC1A | Full Name: | PPARG coactivator 1 alpha |
| Exons: | 13 | Introns: | 12 |
Description:
PPARGC1A is a coactivator of nuclear receptors and other transcription factors that regulate metabolic processes. It has been implicated in the regulation of the globin genes. Upregulation of PGC-1α in human primary erythroid progenitor CD34+ cells induces both fetal γ‐globin mRNA and protein expression as well as the percentage of HbF‐positive cell (F cells) without significantly affecting cell proliferation and differentiation. The combination of ZLN005, a PGC‐1α agonist, and hydroxyurea has an additive effect on the expression of γ‐globin and the generation of F cells from cultured CD34+ cells. The use of pharmacological agents such as ZLN005 that induce PGC‐1α offers a candidate therapeutic strategy for the treatment of patients with beta-haemoglobinopathies.
Synonyms: PGC1 , PGC1A , PGC-1alpha
Comments:
N/A
Number of entries/variants: 0
IthaScore
Sequence Viewer
Publications / Origin
- Sun Y, Habara A, Le CQ, Nguyen N, Chen R, Murphy GJ, Chui DHK, Steinberg MH, Cui S, Pharmacologic induction of PGC-1α stimulates fetal haemoglobin gene expression., Br J Haematol, 2022 PubMed
| A/A | Date | Curator(s) | Comments |
|---|---|---|---|
| 1 | 2022-03-23 14:22:50 | The IthaGenes Curation Team | Created |
| 2 | 2022-03-23 14:24:29 | The IthaGenes Curation Team | Reviewed. Reference and Links added. |