The homozygous Snell dwarf mouse is sterile. It has been shown that pituitary hormone levels are low in 3 month old animals except for FSH and LH whose pituitary contents and plasma concentrations are normal. In this study, the pituitary FSH, LH and prolactin (Prl) content, the FSH plasma concentration and the ovarian follicular development of the Snell dwarf mouse were studied at 18, 20, 24, 40 and 80 days of age. Normal mice were also studied at the same age and served as controls. Pituitary FSH was significantly lower in dwarf mice compared with controls during the period days 18 to 30, while plasma FSH was significantly lower during the period days 20 to 80. Pituitary LH was significantly lower in dwarf mice during the period days 18 to 40. In normal mice, pituitary Prl increased with age, but remained consistently low in dwarf mice. The normal number of growing follicles was similar in dwarf mice and controls up to day 30, but thereafter the total number of growing follicles was greater in the controls. In the dwarf mice, the production of antral follicles was low and there were no ovulations. The rates of atresia were similar in the two genotypes. The responsiveness of the dwarf mouse ovary to FSH was then examined. When dwarf and control mice were supplemented with FSH for 5 days starting at 24 days of age, the ovarian and uterine weights increase 6- and 5-fold, respectively, in the dwarf mice, and 2- to 3-fold in the normal mice.(ABSTRACT TRUNCATED AT 250 WORDS)
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http://dx.doi.org/10.1530/acta.0.1060121 | DOI Listing |
Front Endocrinol (Lausanne)
January 2025
Section on Growth and Development, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institute of Health, Bethesda, MD, United States.
Recombinant human IGF-1 is used to treat severe primary IGF-1 deficiency, but this treatment requires twice-daily injection, often does not fully correct the growth deficit, and has important off-target effects. We therefore sought to target IGF-1 to growth plate cartilage by generating fusion proteins combining IGF-1 with single-chain human antibody fragments that target matrilin-3, a cartilage matrix protein. We previously showed that this cartilage-targeting IGF-1 fusion protein (CV1574-1) promoted growth plate function in a GH-deficient (lit) mouse model.
View Article and Find Full Text PDFAchondroplasia, the most prevalent short-stature disorder, is caused by missense variants overactivating the fibroblast growth factor receptor 3 (FGFR3). As current surgical and pharmaceutical treatments only partially improve some disease features, we sought to explore a genetic approach. We show that an enhancer located 29 kb upstream of mouse Fgfr3 (-29E) is sufficient to confer a transgenic mouse reporter with a domain of expression in cartilage matching that of Fgfr3.
View Article and Find Full Text PDFInt J Mol Sci
December 2024
Department of Pediatrics, McGovern Medical School UTHealth, Houston, TX 77030, USA.
Pseudoachondroplasia (PSACH), a severe dwarfing condition characterized by impaired skeletal growth and early joint degeneration, results from mutations in cartilage oligomeric matrix protein (COMP). These mutations disrupt normal protein folding, leading to the accumulation of misfolded COMP in chondrocytes. The MT-COMP mouse is a murine model of PSACH that expresses D469del human COMP in response to doxycycline and replicates the PSACH chondrocyte and clinical pathology.
View Article and Find Full Text PDFMonoclon Antib Immunodiagn Immunother
December 2024
Endocrinology Division, Department of Pediatrics of Saiful Anwar General Hospital, Universitas Brawijaya, Malang, Indonesia.
Congenital hypothyroidism (CH) is a major health issue that can lead to intellectual disability if not detected and treated earlier. The preliminary screening program for neonatal CH in Indonesia gave a provisional incidence of 1:2513. Newborn screening using a dried blood spot sample is the standard method for CH detection, but it has limitations.
View Article and Find Full Text PDFBiochim Biophys Acta Mol Basis Dis
December 2024
Department of Bioactive Molecules, Pharmacology, Gifu Pharmaceutical University, Gifu 501-1196, Japan; United Graduate School of Drug Discovery and Medical Information Sciences, Gifu University, Gifu 501-1196, Japan; Center for One Medicine Innovative Translational Research (COMIT), Division of Innovative Modality Development, Gifu University, Gifu 501-1196, Japan. Electronic address:
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