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Isolated growth hormone deficiency type II (IGHD2) is mainly caused by heterozygous splice-site mutations in intron 3 of the GH1 gene. A dominant-negative effect of the mutant GH lacking exon 3 on wild-type GH secretion has been proposed; however, the molecular mechanisms involved are elusive. To uncover the molecular systems underlying GH deficiency in IGHD2, we established IGHD2 model mice, which carry both wild-type and mutant copies of the human GH1 gene, replacing each of the endogenous mouse Gh loci. Our IGHD2 model mice exhibited growth retardation along with intact cellular architecture and mildly activated endoplasmic reticulum stress in the pituitary gland, caused by decreased GH-releasing hormone receptor (Ghrhr) and Gh gene promoter activities. Decreased Ghrhr and Gh promoter activities were likely caused by reduced levels of nuclear CREB3L2, which was demonstrated to stimulate Ghrhr and Gh promoter activity. To our knowledge, this is the first in vivo study to reveal a novel molecular mechanism of GH deficiency in IGHD2, representing a new paradigm that differs from widely accepted models.
Pubmed ID: 31436800 RIS Download
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This polyclonal targets Rabbit
View all literature mentionsThis monoclonal targets GRP78 BiP antibody [EPR4041(2)]
View all literature mentionsThis polyclonal secondary targets Immunoglobulins
View all literature mentionsThis monoclonal targets Human Growth Hormone
View all literature mentionsThis monoclonal targets Caspase-3 (8G10) Rabbit mAb
View all literature mentionsThis monoclonal targets PERK (C33E10) Rabbit mAb
View all literature mentionsThis unknown targets
View all literature mentionsThis unknown targets
View all literature mentionsThis polyclonal targets Human CREB3L1
View all literature mentionsThis polyclonal targets
View all literature mentionsThis polyclonal targets Lamin A/C
View all literature mentionsThis monoclonal targets Pit1
View all literature mentionsThis monoclonal targets GAPDH (6C5)
View all literature mentionsThis polyclonal targets Growth Hormone
View all literature mentionsThis polyclonal targets IgG H&L
View all literature mentionsThis polyclonal targets IgG H&L
View all literature mentionsThis monoclonal targets OASIS/CREB3L1
View all literature mentionsThis monoclonal targets OASIS/CREB3L1
View all literature mentionsThis polyclonal targets Growth Hormone
View all literature mentionsThis monoclonal targets Caspase-3 (8G10) Rabbit mAb
View all literature mentionsThis monoclonal targets GRP78 BiP antibody [EPR4041(2)]
View all literature mentionsThis polyclonal targets Lamin A/C
View all literature mentionsThis monoclonal targets PERK (C33E10) Rabbit mAb
View all literature mentionsThis unknown targets
View all literature mentionsThis polyclonal targets
View all literature mentionsThis unknown targets
View all literature mentionsThis polyclonal targets Human CREB3L1
View all literature mentionsThis monoclonal targets Human Growth Hormone
View all literature mentionsThis monoclonal targets Pit1
View all literature mentionsThis polyclonal secondary targets Immunoglobulins
View all literature mentionsThis polyclonal targets IgG H&L
View all literature mentionsThis polyclonal targets IgG H&L
View all literature mentionsThis polyclonal targets Rabbit
View all literature mentionsThis monoclonal targets GAPDH (6C5)
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