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Estrogen Receptor alpha

Domain

Composed of three domains: a modulating N-terminal domain, a DNA-binding domain and a C-terminal ligand-binding domain. The modulating domain, also known as A/B or AF-1 domain has a ligand-independent transactivation function. The C-terminus contains a ligand-dependent transactivation domain, also known as E/F or AF-2 domain which overlaps with the ligand binding domain. AF-1 and AF-2 activate transcription independently and synergistically and act in a promoter- and cell-specific manner. AF-1 seems to provide the major transactivation function in differentiated cells.

Function

The protein expressed by the gene ESR1 functions as a nuclear hormone receptor involved in regulating eukaryotic gene expression, affecting cellular proliferation and differentiation in target tissues. It mediates ligand-dependent nuclear transactivation either through direct homodimer binding to estrogen response elements (ERE) or by associating with other transcription factors like AP-1/c-Jun, c-Fos, ATF-2, Sp1, and Sp3, allowing ERE-independent signaling. Ligand binding triggers a conformational change, facilitating the interaction with coactivator complexes through LXXLL motifs. This interaction leads to mutual transrepression with NF-kappa-B in a cell-type-specific manner, reducing NF-kappa-B DNA-binding activity and transcription from the IL6 promoter while displacing coregulators like RELA/p65. It can antagonistically or synergistically interact with NF-kappa-B for transcription activation, involving recruitment to adjacent response elements, working with CREBBP. Additionally, it activates the transcription of TFF1 and mediates membrane-initiated signaling through kinase cascades. It's crucial for MTA1-mediated transcriptional regulation of BRCA1 and BCAS3. Isoform 3 specifically activates NOS3 and endothelial nitric oxide production, while isoforms lacking certain functional domains modulate transcriptional activity by competitive ligand or DNA binding and heterodimerization with the full-length receptor, capable of binding to ERE and inhibiting isoform 1. This supplementary information is collated from multiple sources and compiled automatically.

Involvement in disease

Estrogen resistance

ESTRR

A disorder characterized by partial or complete resistance to estrogens, in the presence of elevated estrogen serum levels. Clinical features include absence of the pubertal growth spurt, delayed bone maturation, unfused epiphyses, reduced bone mineral density, osteoporosis, continued growth into adulthood and very tall adult stature. Glucose intolerance, hyperinsulinemia and lipid abnormalities may also be present.

None

The disease is caused by variants affecting the gene represented in this entry.

Post-translational modifications

Phosphorylated by cyclin A/CDK2 and CK1. Phosphorylation probably enhances transcriptional activity. Self-association induces phosphorylation. Dephosphorylation at Ser-118 by PPP5C inhibits its transactivation activity. Phosphorylated by LMTK3 in vitro.

Glycosylated; contains N-acetylglucosamine, probably O-linked.

Ubiquitinated; regulated by LATS1 via DCAF1 it leads to ESR1 proteasomal degradation (PubMed:21602804, PubMed:28068668). Deubiquitinated by OTUB1 (PubMed:19383985). Ubiquitinated by STUB1/CHIP; in the CA1 hippocampal region following loss of endogenous circulating estradiol (17-beta-estradiol/E2) (By similarity). Ubiquitinated by UBR5, leading to its degradation: UBR5 specifically recognizes and binds ligand-bound ESR1 when it is not associated with coactivators (NCOAs) (PubMed:37478846). In presence of NCOAs, the UBR5-degron is not accessible, preventing its ubiquitination and degradation (PubMed:37478846).

Dimethylated by PRMT1 at Arg-260. The methylation may favor cytoplasmic localization (PubMed:18657504, PubMed:24498420). Demethylated by JMJD6 at Arg-260 (PubMed:24498420).

Palmitoylated (isoform 3). Not biotinylated (isoform 3).

Palmitoylated by ZDHHC7 and ZDHHC21. Palmitoylation is required for plasma membrane targeting and for rapid intracellular signaling via ERK and AKT kinases and cAMP generation, but not for signaling mediated by the nuclear hormone receptor.

Sequence similarities

Belongs to the nuclear hormone receptor family. NR3 subfamily.

Tissue specificity

Widely expressed (PubMed:10970861). Not expressed in the pituitary gland (PubMed:10970861).

Isoform 3

Widely expressed, however not expressed in the pituitary gland.

Cellular localization

  • Isoform 1
  • Nucleus
  • Cytoplasm
  • Cell membrane
  • Peripheral membrane protein
  • Cytoplasmic side
  • A minor fraction is associated with the inner membrane.
  • Isoform 3
  • Nucleus
  • Cytoplasm
  • Cell membrane
  • Peripheral membrane protein
  • Cytoplasmic side
  • Cell membrane
  • Single-pass type I membrane protein
  • Associated with the inner membrane via palmitoylation (Probable). At least a subset exists as a transmembrane protein with a N-terminal extracellular domain.
  • Nucleus
  • Golgi apparatus
  • Cell membrane
  • Colocalizes with ZDHHC7 and ZDHHC21 in the Golgi apparatus where most probably palmitoylation occurs. Associated with the plasma membrane when palmitoylated.

Alternative names

ESR, NR3A1, ESR1, Estrogen receptor, ER, ER-alpha, Estradiol receptor, Nuclear receptor subfamily 3 group A member 1

Target type

Proteins

Primary research area

Oncology

Molecular weight

66216Da

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