PUBLICATION
The X-linked acrogigantism-associated gene gpr101 is a regulator of early embryonic development and growth in zebrafish
- Authors
- 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.
- ID
- ZDB-PUB-201130-10
- 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.
Citation
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. (2020) The X-linked acrogigantism-associated gene gpr101 is a regulator of early embryonic development and growth in zebrafish. Molecular and Cellular Endocrinology. 520:111091.
Abstract
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.
Genes / Markers
Expression
Phenotype
Mutations / Transgenics
Human Disease / Model
Sequence Targeting Reagents
Fish
Orthology
Engineered Foreign Genes
Mapping