GPR101 encodes an orphan class A/rhodopsin-like G protein-coupled receptor. The protein is a multi-pass plasma-membrane receptor with no firmly established endogenous ligand, but evolutionary and structural features support a GPCR signaling role. Increased GPR101 dosage is associated with X-linked acrogigantism and growth hormone-secreting pituitary adenoma predisposition, consistent with altered pituitary signaling rather than a defined enzymatic activity.
Relationship is the entry's genetic[].relationship_type. untyped means the entry has a genetic record for the gene but does not type it; its free-text association is shown instead.
| Disorder | Relationship | Where the gene appears |
|---|---|---|
| GPR101-related pituitary adenoma 2 | causative germline and somatic gain of functionunknown |
mechanism: GPR101 copy-number gain; GPR101-driven adenylate cyclase-activating GPCR signaling; Reported GPR101 p.E308D variant association
diagnosis: Xq26.3/GPR101 copy-number testing
|
| Pituitary Tumor | no genetic record |
mechanism: Clonal Expansion of an Adenohypophyseal Cell Lineage
subtype: Somatotroph
|
Identity from HGNC (snapshot 2026-10-01). Page layers are described in the gene pages documentation.