Osteopetrosis in cathepsin K-deficient mice
نویسندگان
چکیده
منابع مشابه
Osteopetrosis in cathepsin K-deficient mice.
Osteopetrosis is a fascinating, inherited polygenic disorder of bone metabolism characterized by a generalized increase in bone mass due to decreased bone resorption (1). While it is relatively rare, it provides important clues to the understanding of bone cell biology and the pathogenesis of metabolic bone diseases. Since osteopetrosis results from decreased osteoclastic bone resorption, its m...
متن کاملImpaired osteoclastic bone resorption leads to osteopetrosis in cathepsin-K-deficient mice.
Cathepsin K is a recently identified lysosomal cysteine proteinase. It is abundant in osteoclasts, where it is believed to play a vital role in the resorption and remodeling of bone. Pycnodysostosis is a rare inherited osteochondrodysplasia that is caused by mutations of the cathepsin-K gene, characterized by osteosclerosis, short stature, and acroosteolysis of the distal phalanges. With a view...
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Cathepsin G is a neutral serine protease that is highly expressed at the promyelocyte stage of myeloid development. We have developed a homologous recombination strategy to create a loss-of-function mutation for murine cathepsin G. Bone marrow derived from mice homozygous for this mutation had no detectable cathepsin G protein or activity, indicating that no other protease in bone marrow cells ...
متن کاملLinking osteopetrosis and pycnodysostosis: regulation of cathepsin K expression by the microphthalmia transcription factor family.
Various genetic conditions produce dysfunctional osteoclasts resulting in osteopetrosis or osteosclerosis. These include human pycnodysostosis, an autosomal recessive syndrome caused by cathepsin K mutation, cathepsin K-deficient mice, and mitf mutant rodent strains. Cathepsin K is a highly expressed cysteine protease in osteoclasts that plays an essential role in the degradation of protein com...
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Cathepsin K (CatK) is one of the most potent mammalian collagenases. We showed previously the increased expression of CatK in human and animal atherosclerotic lesions. Here, we hypothesized that ablation of CatK mitigates injury-induced neointimal hyperplasia. Male wild-type (CatK(+/+)) and CatK-deficient (CatK(-/-)) mice underwent ligation or a combination of ligation and polyethylene cuff-rep...
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ژورنال
عنوان ژورنال: European Journal of Endocrinology
سال: 1999
ISSN: 0804-4643,1479-683X
DOI: 10.1530/eje.0.1400376