GeneID: 482


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

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


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Publications / Origin

  1. 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
Created on 2022-03-23 14:22:50, Last reviewed on 2022-03-23 14:24:29 (Show full history)

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