Matches in SemOpenAlex for { <https://semopenalex.org/work/W4385185945> ?p ?o ?g. }
- W4385185945 abstract "SLC7A7 deficiency, or lysinuric protein intolerance (LPI), causes loss of function of the y+LAT1 transporter critical for efflux of arginine, lysine and ornithine in certain cells. LPI is characterized by urea cycle dysfunction, renal disease, immune dysregulation, growth failure, delayed bone age and osteoporosis. We previously reported that Slc7a7 knockout mice (C57BL/6×129/SvEv F2) recapitulate LPI phenotypes, including growth failure. Our main objective in this study was to characterize the skeletal phenotype in these mice. Compared to wild-type littermates, juvenile Slc7a7 knockout mice demonstrated 70% lower body weights, 87% lower plasma IGF-1 concentrations and delayed skeletal development. Because poor survival prevents evaluation of mature knockout mice, we generated a conditional Slc7a7 deletion in mature osteoblasts or mesenchymal cells of the osteo-chondroprogenitor lineage, but no differences in bone architecture were observed. Overall, global Slc7a7 deficiency caused growth failure with low plasma IGF-1 concentrations and delayed skeletal development, but Slc7a7 deficiency in the osteoblastic lineage was not a major contributor to these phenotypes. Future studies utilizing additional tissue-specific Slc7a7 knockout models may help dissect cell-autonomous and non-cell-autonomous mechanisms underlying phenotypes in LPI." @default.
- W4385185945 created "2023-07-25" @default.
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- W4385185945 date "2023-08-01" @default.
- W4385185945 modified "2023-10-14" @default.
- W4385185945 title "Delayed skeletal development and IGF-1 deficiency in a mouse model of lysinuric protein intolerance" @default.
- W4385185945 cites W1540095279 @default.
- W4385185945 cites W1543615399 @default.
- W4385185945 cites W1601032788 @default.
- W4385185945 cites W1882451129 @default.
- W4385185945 cites W1963742875 @default.
- W4385185945 cites W1966108627 @default.
- W4385185945 cites W1968537876 @default.
- W4385185945 cites W1970390200 @default.
- W4385185945 cites W1971975084 @default.
- W4385185945 cites W1975139962 @default.
- W4385185945 cites W1977075685 @default.
- W4385185945 cites W1977344994 @default.
- W4385185945 cites W1977626917 @default.
- W4385185945 cites W1979703244 @default.
- W4385185945 cites W1981057102 @default.
- W4385185945 cites W1985442013 @default.
- W4385185945 cites W1986892225 @default.
- W4385185945 cites W1989343608 @default.
- W4385185945 cites W1989939104 @default.
- W4385185945 cites W1990319596 @default.
- W4385185945 cites W1993672117 @default.
- W4385185945 cites W1999734947 @default.
- W4385185945 cites W2000251770 @default.
- W4385185945 cites W2009810744 @default.
- W4385185945 cites W2014239161 @default.
- W4385185945 cites W2015021045 @default.
- W4385185945 cites W2016623383 @default.
- W4385185945 cites W2017949036 @default.
- W4385185945 cites W2018001278 @default.
- W4385185945 cites W2021020614 @default.
- W4385185945 cites W2021483365 @default.
- W4385185945 cites W2033592813 @default.
- W4385185945 cites W2033599106 @default.
- W4385185945 cites W2038412619 @default.
- W4385185945 cites W2038494534 @default.
- W4385185945 cites W2039825798 @default.
- W4385185945 cites W2040729303 @default.
- W4385185945 cites W2045234728 @default.
- W4385185945 cites W2045359181 @default.
- W4385185945 cites W2050775539 @default.
- W4385185945 cites W2053816440 @default.
- W4385185945 cites W2063806215 @default.
- W4385185945 cites W2067328480 @default.
- W4385185945 cites W2070476795 @default.
- W4385185945 cites W2071555143 @default.
- W4385185945 cites W2072434201 @default.
- W4385185945 cites W2076040081 @default.
- W4385185945 cites W2076775687 @default.
- W4385185945 cites W2077970718 @default.
- W4385185945 cites W2082608938 @default.
- W4385185945 cites W2083899759 @default.
- W4385185945 cites W2084465360 @default.
- W4385185945 cites W2085953036 @default.
- W4385185945 cites W2104135721 @default.
- W4385185945 cites W2107277218 @default.
- W4385185945 cites W2107941531 @default.
- W4385185945 cites W2108382032 @default.
- W4385185945 cites W2111894013 @default.
- W4385185945 cites W2116137012 @default.
- W4385185945 cites W2124522095 @default.
- W4385185945 cites W2132984762 @default.
- W4385185945 cites W2147169548 @default.
- W4385185945 cites W2149979678 @default.
- W4385185945 cites W2155907836 @default.
- W4385185945 cites W2160242253 @default.
- W4385185945 cites W2163820329 @default.
- W4385185945 cites W2168693601 @default.
- W4385185945 cites W2171149103 @default.
- W4385185945 cites W2173755138 @default.
- W4385185945 cites W2192080449 @default.
- W4385185945 cites W2328012396 @default.
- W4385185945 cites W2401224070 @default.
- W4385185945 cites W2514285508 @default.
- W4385185945 cites W2519435241 @default.
- W4385185945 cites W2538509033 @default.
- W4385185945 cites W2569831080 @default.