FRINK Linked Data Fragments server

Matches in SemOpenAlex for { <https://semopenalex.org/work/W3183325901> ?p ?o ?g. }

• W3183325901 abstract "Abstract Albright hereditary osteodystrophy (AHO) is caused by heterozygous inactivation of GNAS , a complex locus that encodes the alpha-stimulatory subunit of GPCRs (Gsα) in addition to NESP55 and XL α s due to alternative first exons. AHO skeletal manifestations include brachydactyly, brachymetacarpia, compromised adult stature, and subcutaneous ossifications. AHO patients with maternally-inherited GNAS mutations develop pseudohypoparathyroidism type 1A (PHP1A) with resistance to multiple hormones that mediate their actions through GPCRs requiring Gsα (eg., PTH, TSH, GHRH, calcitonin) and severe obesity. Paternally-inherited GNAS mutations cause pseudopseudohypoparathyroidism (PPHP), in which patients have AHO skeletal features but do not develop hormonal resistance or marked obesity. These differences between PHP1A and PPHP are caused by tissue-specific reduction of paternal Gsα expression. Previous reports in mice have shown loss of Gsα causes osteopenia due to impaired osteoblast number and function and suggest AHO patients could display evidence of reduced bone mineral density (BMD). However, we previously demonstrated PHP1A patients display normal-increased BMD measurements without any correlation to body mass index or serum PTH. Due to these observed differences between PHP1A and PPHP, we utilized our laboratory’s AHO mouse model to address whether Gsα heterozygous inactivation by the targeted disruption of exon 1 of Gnas differentially affects bone remodeling based on the parental inheritance of the mutation. Mice with paternally-inherited ( GnasE1+/−p ) and maternally-inherited ( GnasE1+/−m ) mutations displayed reductions in osteoblasts along the bone surface compared to wildtype. GnasE1+/−p mice displayed reduced cortical and trabecular bone parameters due to impaired bone formation and excessive bone resorption. GnasE1+/−m mice however displayed enhanced bone parameters due to increased osteoblast activity and normal bone resorption. These distinctions in bone remodeling between GnasE1+/−p and GnasE1+/−m mice appear to be secondary to changes in the bone microenvironment driven by calcitonin-resistance within GnasE1+/−m osteoclasts and therefore warrant further studies into understanding how Gsα influences osteoblast-osteoclast coupling interactions." @default.
• W3183325901 created "2021-08-02" @default.
• W3183325901 creator A5025491098 @default.
• W3183325901 creator A5032184959 @default.
• W3183325901 creator A5037862124 @default.
• W3183325901 creator A5066124486 @default.
• W3183325901 creator A5067453135 @default.
• W3183325901 date "2021-07-27" @default.
• W3183325901 modified "2023-10-12" @default.
• W3183325901 title "Parental origin of Gsα inactivation differentially affects bone remodeling in a mouse model of Albright hereditary osteodystrophy" @default.
• W3183325901 cites W1927572613 @default.
• W3183325901 cites W1963598729 @default.
• W3183325901 cites W1967271150 @default.
• W3183325901 cites W1980492262 @default.
• W3183325901 cites W1980751002 @default.
• W3183325901 cites W1987992389 @default.
• W3183325901 cites W2006146117 @default.
• W3183325901 cites W2006990379 @default.
• W3183325901 cites W2009748729 @default.
• W3183325901 cites W2021127373 @default.
• W3183325901 cites W2040184606 @default.
• W3183325901 cites W2040203747 @default.
• W3183325901 cites W2042512620 @default.
• W3183325901 cites W2051834155 @default.
• W3183325901 cites W2055349132 @default.
• W3183325901 cites W2058125623 @default.
• W3183325901 cites W2084283037 @default.
• W3183325901 cites W2087196892 @default.
• W3183325901 cites W2088970884 @default.
• W3183325901 cites W2089513225 @default.
• W3183325901 cites W2097288660 @default.
• W3183325901 cites W2102633546 @default.
• W3183325901 cites W2104924678 @default.
• W3183325901 cites W2108642017 @default.
• W3183325901 cites W2117817280 @default.
• W3183325901 cites W2119368661 @default.
• W3183325901 cites W2125786509 @default.
• W3183325901 cites W2126927420 @default.
• W3183325901 cites W2127145316 @default.
• W3183325901 cites W2133931922 @default.
• W3183325901 cites W2159803524 @default.
• W3183325901 cites W2164155520 @default.
• W3183325901 cites W2167331313 @default.
• W3183325901 cites W2255879554 @default.
• W3183325901 cites W2766135242 @default.
• W3183325901 cites W2811181045 @default.
• W3183325901 cites W2901179793 @default.
• W3183325901 cites W2905490516 @default.
• W3183325901 cites W2908383808 @default.
• W3183325901 cites W2914372526 @default.
• W3183325901 cites W2947932851 @default.
• W3183325901 cites W2977240403 @default.
• W3183325901 cites W3000377151 @default.
• W3183325901 cites W3093143042 @default.
• W3183325901 cites W4211258008 @default.
• W3183325901 cites W4236176029 @default.
• W3183325901 cites W4245936851 @default.
• W3183325901 cites W4249503707 @default.
• W3183325901 doi "https://doi.org/10.1101/2021.07.27.453811" @default.
• W3183325901 hasPublicationYear "2021" @default.
• W3183325901 type Work @default.
• W3183325901 sameAs 3183325901 @default.
• W3183325901 citedByCount "0" @default.
• W3183325901 crossrefType "posted-content" @default.
• W3183325901 hasAuthorship W3183325901A5025491098 @default.
• W3183325901 hasAuthorship W3183325901A5032184959 @default.
• W3183325901 hasAuthorship W3183325901A5037862124 @default.
• W3183325901 hasAuthorship W3183325901A5066124486 @default.
• W3183325901 hasAuthorship W3183325901A5067453135 @default.
• W3183325901 hasBestOaLocation W31833259011 @default.
• W3183325901 hasConcept C104317684 @default.
• W3183325901 hasConcept C126322002 @default.
• W3183325901 hasConcept C134018914 @default.
• W3183325901 hasConcept C170033053 @default.
• W3183325901 hasConcept C202751555 @default.
• W3183325901 hasConcept C2776541429 @default.
• W3183325901 hasConcept C2776886416 @default.
• W3183325901 hasConcept C2777083390 @default.
• W3183325901 hasConcept C2777871287 @default.
• W3183325901 hasConcept C2778260815 @default.
• W3183325901 hasConcept C2780356894 @default.
• W3183325901 hasConcept C2781000923 @default.
• W3183325901 hasConcept C2781133459 @default.
• W3183325901 hasConcept C2781208988 @default.
• W3183325901 hasConcept C36823959 @default.
• W3183325901 hasConcept C519063684 @default.
• W3183325901 hasConcept C54355233 @default.
• W3183325901 hasConcept C71924100 @default.
• W3183325901 hasConcept C74231797 @default.
• W3183325901 hasConcept C86803240 @default.
• W3183325901 hasConceptScore W3183325901C104317684 @default.
• W3183325901 hasConceptScore W3183325901C126322002 @default.
• W3183325901 hasConceptScore W3183325901C134018914 @default.
• W3183325901 hasConceptScore W3183325901C170033053 @default.
• W3183325901 hasConceptScore W3183325901C202751555 @default.
• W3183325901 hasConceptScore W3183325901C2776541429 @default.
• W3183325901 hasConceptScore W3183325901C2776886416 @default.
• W3183325901 hasConceptScore W3183325901C2777083390 @default.
• W3183325901 hasConceptScore W3183325901C2777871287 @default.
• W3183325901 hasConceptScore W3183325901C2778260815 @default.

• next