کد مقاله | کد نشریه | سال انتشار | مقاله انگلیسی | نسخه تمام متن |
---|---|---|---|---|
9943346 | 1573501 | 2005 | 10 صفحه PDF | دانلود رایگان |
عنوان انگلیسی مقاله ISI
Sustained Osteomalacia of Long Bones Despite Major Improvement in Other Hypophosphatasia-Related Mineral Deficits in Tissue Nonspecific Alkaline Phosphatase/Nucleotide Pyrophosphatase Phosphodiesterase 1 Double-Deficient Mice
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موضوعات مرتبط
علوم پزشکی و سلامت
پزشکی و دندانپزشکی
کاردیولوژی و پزشکی قلب و عروق
پیش نمایش صفحه اول مقاله
چکیده انگلیسی
We have shown previously that the hypomineralization defects of the calvarium and vertebrae of tissue nonspecific alkaline phosphatase (TNAP)-deficient (Akp2â/â) hypophosphatasia mice are rescued by simultaneous deletion of the Enpp1 gene, which encodes nucleotide pyrophosphatase phosphodiesterase 1 (NPP1). Conversely, the hyperossification in the vertebral apophyses typical of Enpp1â/â mice is corrected in [Akp2â/â; Enpp1â/â] double-knockout mice. Here we have examined the appendicular skeletons of Akp2â/â, Enpp1â/â, and [Akp2â/â; Enpp1â/â] mice to ascertain the degree of rescue afforded at these skeletal sites. Alizarin red and Alcian blue whole mount analysis of the skeletons from wild-type, Akp2â/â, and [Akp2â/â; Enpp1â/â] mice revealed that although calvarium and vertebrae of double-knockout mice were normalized with respect to mineral deposition, the femur and tibia were not. Using several different methodologies, we found reduced mineralization not only in Akp2â/â but also in Enpp1â/â and [Akp2â/â; Enpp1â/â] femurs and tibias. Analysis of calvarial- and bone marrow-derived osteoblasts for mineralized nodule formation in vitro showed increased mineral deposition by Enpp1â/â calvarial osteoblasts but decreased mineral deposition by Enpp1â/â long bone marrow-derived osteoblasts in comparison to wild-type cells. Thus, the osteomalacia of Akp2â/â mice and the hypomineralized phenotype of the long bones of Enpp1â/â mice are not rescued by simultaneous deletion of TNAP and NPP1 functions.
ناشر
Database: Elsevier - ScienceDirect (ساینس دایرکت)
Journal: The American Journal of Pathology - Volume 166, Issue 6, June 2005, Pages 1711-1720
Journal: The American Journal of Pathology - Volume 166, Issue 6, June 2005, Pages 1711-1720
نویسندگان
H. Clarke Anderson, Dympna Harmey, Nancy P. Camacho, Rama Garimella, Joseph B. Sipe, Sarah Tague, Xiaohong Bi, Kristen Johnson, Robert Terkeltaub, José Luis Millán,