GPR101

GPR101 is a human gene encoding for a G protein-coupled receptor 101. It is classified as an orphan receptor, meaning its endogenous ligand or activating molecule is currently unknown. GPR101 is primarily expressed in the brain, specifically within the hypothalamus and pituitary gland, suggesting a role in neuroendocrine function.

While the exact function of GPR101 remains under investigation, genetic studies have implicated it in growth regulation and pituitary adenoma formation. Gain-of-function mutations and duplications in the GPR101 gene have been identified in patients with X-linked acrogigantism (XLAG), a rare condition characterized by excessive growth starting in early childhood, often caused by pituitary gigantism. This implies that increased signaling through GPR101 can lead to enhanced growth hormone secretion from the pituitary.

Further research is needed to identify the natural ligand for GPR101 and to fully elucidate its role in normal physiology and disease pathogenesis. Understanding the GPR101 signaling pathway may provide potential therapeutic targets for XLAG and other growth disorders. Its potential involvement in other neurological and endocrine functions is also an area of ongoing investigation.