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ZFIN ID: ZDB-PUB-201130-10
The X-linked acrogigantism-associated gene gpr101 is a regulator of early embryonic development and growth in zebrafish
Trivellin, G., Tirosh, A., Hernández-Ramírez, L.C., Gupta, T., Tsai-Morris, C.H., Faucz, F.R., Burgess, H.A., Feldman, B., Stratakis, C.A.
Date: 2020
Source: Molecular and Cellular Endocrinology   520: 111091 (Journal)
Registered Authors: Burgess, Harold, Feldman, Benjamin
Keywords: GPR101, X-linked acrogigantism, embryonic development, growth, zebrafish
MeSH Terms:
  • Acromegaly/complications
  • Acromegaly/genetics*
  • Animals
  • Embryonic Development/genetics*
  • Female
  • Fertilization/genetics
  • Gastrulation/genetics
  • Gene Expression Regulation, Developmental
  • Genetic Diseases, X-Linked/genetics*
  • Gigantism/complications
  • Gigantism/genetics*
  • Hypothalamus/pathology
  • Mutation/genetics
  • Ovum/metabolism
  • Signal Transduction/genetics
  • Temperature
  • Transcriptome/genetics
  • Up-Regulation/genetics
  • Zebrafish/genetics*
  • Zebrafish/growth & development*
  • Zebrafish Proteins/genetics*
  • Zebrafish Proteins/metabolism
  • Zygote/metabolism
PubMed: 33248229 Full text @ Mol. Cell. Endocrinol.
We recently described X-linked acrogigantism (X-LAG), a condition of early childhood-onset pituitary gigantism associated with microduplications of the GPR101 receptor. The expression of GPR101 in hyperplastic pituitary regions and tumors in X-LAG patients, and GPR101's normally transient pituitary expression during fetal development, suggest a role in the regulation of growth. Nevertheless, little is still known about GPR101's physiological functions, especially during development. By using zebrafish models, we investigated the role of gpr101 during embryonic development and somatic growth. Transient ectopic gpr101 expression perturbed the embryonic body plan but did not affect growth. Loss of gpr101 led to a significant reduction in body size that was even more pronounced in the absence of maternal transcripts, as well as subfertility. These changes were accompanied by gastrulation and hypothalamic defects. In conclusion, both gpr101 loss- and gain-of-function affect, in different ways, fertility, embryonic patterning, growth and brain development.