Full Text Journal Articles by
Author Bart O Williams

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Generation and Characterization of Mouse Models for Skeletal Disease.

Gabrielle E Foxa, Ye Liu, Lisa M Turner, Alexander G Robling, Tao Yang, Bart O Williams,

Our laboratories have used genetically engineered mouse models (GEMMs) to assess genetic contributions to skeletal diseases such as osteoporosis and osteoarthritis. Studies on the genetic contributions to OA are often done by assessing how GEMMs respond to surgical methods that induce symptoms modeling OA. Here, we will describe protocols outlining ... Read more >>

Methods Mol Biol (Methods in molecular biology (Clifton, N.J.))
[2021, 2221:165-191]

Cited: 0 times

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Co-deletion of Lrp5 and Lrp6 in the skeleton severely diminishes bone gain from sclerostin antibody administration.

Kyung-Eun Lim, Whitney A Bullock, Daniel J Horan, Bart O Williams, Matthew L Warman, Alexander G Robling,

The cysteine knot protein sclerostin is an osteocyte-derived secreted inhibitor of the Wnt co-receptors LRP5 and LRP6. LRP5 plays a dominant role in bone homeostasis, but we previously reported that Sost/sclerostin suppression significantly increased osteogenesis regardless of Lrp5 presence or absence. Those observations suggested that the bone forming effects of ... Read more >>

Bone (Bone)
[2020, 143:115708]

Cited: 0 times

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LDL receptor-related protein LRP6 senses nutrient levels and regulates Hippo signaling.

Wonyoung Jeong, Soyoung Kim, Ukjin Lee, Zhendong A Zhong, Mikhail Savitsky, Hyeryun Kwon, Jiyoung Kim, Taebok Lee, Jin Won Cho, Bart O Williams, Vladimir L Katanaev, Eek-Hoon Jho,

Controlled cell growth and proliferation are essential for tissue homeostasis and development. Wnt and Hippo signaling are well known as positive and negative regulators of cell proliferation, respectively. The regulation of Hippo signaling by the Wnt pathway has been shown, but how and which components of Wnt signaling are involved ... Read more >>

EMBO Rep (EMBO reports)
[2020, 21(9):e50103]

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Low-Density Lipoprotein Receptor-Related Protein 5-Deficient Rats Have Reduced Bone Mass and Abnormal Development of the Retinal Vasculature.

John L Ubels, Cassandra R Diegel, Gabrielle E Foxa, Nicole J Ethen, Jonathan N Lensing, Zachary B Madaj, Bart O Williams,

Humans carrying homozygous loss-of-function mutations in the Wnt co-receptor, low-density lipoprotein receptor-related protein 5 (LRP5), develop osteoporosis and a defective retinal vasculature known as familial exudative vitreoretinopathy (FEVR) due to disruption of the Wnt signaling pathway. The purpose of this study was to use CRISPR-Cas9-mediated gene editing to create strains ... Read more >>

CRISPR J (The CRISPR journal)
[2020, 3(4):284-298]

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Independent validation of experimental results requires timely and unrestricted access to animal models and reagents.

Cassandra R Diegel, Steven Hann, Ugur M Ayturk, Jennifer C W Hu, Kyung-Eun Lim, Casey J Droscha, Zachary B Madaj, Gabrielle E Foxa, Isaac Izaguirre, , Alexander G Robling, Matthew L Warman, Bart O Williams,

PLoS Genet (PLoS genetics)
[2020, 16(6):e1008940]

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An osteocalcin-deficient mouse strain without endocrine abnormalities.

Cassandra R Diegel, Steven Hann, Ugur M Ayturk, Jennifer C W Hu, Kyung-Eun Lim, Casey J Droscha, Zachary B Madaj, Gabrielle E Foxa, Isaac Izaguirre, Vai Vivarium And Transgenics Core, Noorulain Paracha, Bohdan Pidhaynyy, Terry L Dowd, Alexander G Robling, Matthew L Warman, Bart O Williams,

Osteocalcin (OCN), the most abundant noncollagenous protein in the bone matrix, is reported to be a bone-derived endocrine hormone with wide-ranging effects on many aspects of physiology, including glucose metabolism and male fertility. Many of these observations were made using an OCN-deficient mouse allele (Osc-) in which the 2 OCN-encoding ... Read more >>

PLoS Genet (PLoS genetics)
[2020, 16(5):e1008361]

Cited: 4 times

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Frizzled-4 is required for normal bone acquisition despite compensation by Frizzled-8.

Priyanka Kushwaha, Soohyun Kim, Gabrielle E Foxa, Megan N Michalski, Bart O Williams, Ryan E Tomlinson, Ryan C Riddle,

The activation of the Wnt/β-catenin signaling pathway is critical for skeletal development but surprisingly little is known about the requirements for the specific frizzled (Fzd) receptors that recognize Wnt ligands. To define the contributions of individual Fzd proteins to osteoblast function, we profiled the expression of all 10 mammalian receptors ... Read more >>

J Cell Physiol (Journal of cellular physiology)
[2020, 235(10):6673-6683]

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Loss of Lgals3 Protects Against Gonadectomy-Induced Cortical Bone Loss in Mice.

Kevin A Maupin, Daniel Dick, Johan Lee, Bart O Williams,

Sex hormone deprivation commonly occurs following menopause in women or after androgen-depletion during prostate cancer therapy in men, resulting in rapid bone turnover and loss of bone mass. There is a need to identify novel therapies to improve bone mass in these conditions. Previously, we identified age- and sex-dependent effects ... Read more >>

Calcif Tissue Int (Calcified tissue international)
[2020, 106(3):283-293]

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Perspectives on the role of Wnt biology in cancer.

Christopher K Mirabelli, Roel Nusse, David A Tuveson, Bart O Williams,

Selected members of the Wnt signaling community met during a 4-day period in October 2018 to discuss the current challenges and opportunities associated with targeting the Wnt pathway for therapeutic benefit. A summary of key points of these discussions is presented in this report. ... Read more >>

Sci Signal (Science signaling)
[2019, 12(589):]

Cited: 4 times

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Wnt Signaling Inhibits Osteoclast Differentiation by Activating Canonical and Noncanonical cAMP/PKA Pathways.

Megan M Weivoda, Ming Ruan, Christine M Hachfeld, Larry Pederson, Alan Howe, Rachel A Davey, Jeffrey D Zajac, Yasuhiro Kobayashi, Bart O Williams, Jennifer J Westendorf, Sundeep Khosla, Merry Jo Oursler,

J. Bone Miner. Res. (Journal of bone and mineral research : the official journal of the American Society for Bone and Mineral Research)
[2019, 34(8):1546-1548]

Cited: 1 time

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Regulation of cytoskeleton and adhesion signaling in osteoclasts by tetraspanin CD82.

Alexis Bergsma, Sourik S Ganguly, Mollie E Wiegand, Daniel Dick, Bart O Williams, Cindy K Miranti,

We used a myeloid-specific Cre to conditionally delete CD82 in mouse osteoclasts and their precursors. In contrast to global loss of CD82 (gKO), conditional loss of CD82 (cKO) in osteoclasts does not affect cortical bone, osteoblasts, or adipocytes. CD82 loss results in greater trabecular volume and trabecular number but reduced ... Read more >>

Bone Rep (Bone reports)
[2019, 10:100196]

Cited: 1 time

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Global deletion of tetraspanin CD82 attenuates bone growth and enhances bone marrow adipogenesis.

Alexis Bergsma, Sourik S Ganguly, Daniel Dick, Bart O Williams, Cindy K Miranti,

CD82 is a widely expressed member of the tetraspanin family of transmembrane proteins known to control cell signaling, adhesion, and migration. Tetraspanin CD82 is induced over 9-fold during osteoclast differentiation in vitro; however, its role in bone homeostasis is unknown. A globally deleted CD82 mouse model was used to assess ... Read more >>

Bone (Bone)
[2018, 113:105-113]

Cited: 4 times

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LRP1 Suppresses Bone Resorption in Mice by Inhibiting the RANKL-Stimulated NF-κB and p38 Pathways During Osteoclastogenesis.

Di Lu, Jianshuang Li, Huadie Liu, Gabrielle E Foxa, Kevin Weaver, Jie Li, Bart O Williams, Tao Yang,

Single-nucleotide polymorphisms in the LRP1 gene coding sequence are associated with low bone mass, and cell culture studies suggest that LRP1 plays a role in osteoblast proliferation and osteoblast-mediated osteoclastogenesis. However, the in vivo function of LRP1 in bone homeostasis has not been explored. In this work, we studied the ... Read more >>

J Bone Miner Res (Journal of bone and mineral research : the official journal of the American Society for Bone and Mineral Research)
[2018, 33(10):1773-1784]

Cited: 3 times

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Bone loss from Wnt inhibition mitigated by concurrent alendronate therapy.

Babita Madan, Mitchell J McDonald, Gabrielle E Foxa, Cassandra R Diegel, Bart O Williams, David M Virshup,

Dysregulated Wnt signaling is associated with the pathogenesis of cancers, fibrosis, and vascular diseases. Inhibition of Wnt signaling has shown efficacy in various pre-clinical models of these disorders. One of the key challenges in developing targeted anti-cancer drugs is to balance efficacy with on-target toxicity. Given the crucial role Wnts ... Read more >>

(Bone research)
[2018, 6:17]

Cited: 7 times

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A quest for clarity in bone erosion: The role of sequestosome 1 in Paget's disease of bone.

Megan N Michalski, Bart O Williams,

Alterations in the SQSTM1 gene are a putative cause of Paget's disease of bone, yet results are conflicting about how these mutations impact osteoclasts, the cell type believed to be the main pathological contributor. In this issue of JBC, Zach et al. provide important new evidence that the protein encoded ... Read more >>

J Biol Chem (The Journal of biological chemistry)
[2018, 293(24):9542-9543]

Cited: 0 times

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Enhanced cortical bone expansion in Lgals3-deficient mice during aging.

Kevin A Maupin, Kevin Weaver, Alexis Bergsma, Cheryl Christie, Zhendong A Zhong, Tao Yang, Bart O Williams,

Imbalances between bone formation and bone resorption, which can occur due to aging or sex hormone deprivation, result in decreased bone mass and an increased risk of fracture. Previous studies have suggested that the β-galactoside binding lectin, galectin-3, is involved in bone remodeling. We compared bone parameters of mice having ... Read more >>

Bone Res (Bone research)
[2018, 6:7]

Cited: 0 times

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Ginkgolic acid, a sumoylation inhibitor, promotes adipocyte commitment but suppresses adipocyte terminal differentiation of mouse bone marrow stromal cells.

Huadie Liu, Jianshuang Li, Di Lu, Jie Li, Minmin Liu, Yuanzheng He, Bart O Williams, Jiada Li, Tao Yang,

Sumoylation is a post-translational modification process having an important influence in mesenchymal stem cell (MSC) differentiation. Thus, sumoylation-modulating chemicals might be used to control MSC differentiation for skeletal tissue engineering. In this work, we studied how the differentiation of mouse bone marrow stromal cells (mBMSCs) is affected by ginkgolic acid ... Read more >>

Sci Rep (Scientific reports)
[2018, 8(1):2545]

Cited: 5 times

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Publisher Correction: Desumoylase SENP6 maintains osteochondroprogenitor homeostasis by suppressing the p53 pathway.

Jianshuang Li, Di Lu, Hong Dou, Huadie Liu, Kevin Weaver, Wenjun Wang, Jiada Li, Edward T H Yeh, Bart O Williams, Ling Zheng, Tao Yang,

In the originally published version of this Article, the positions of the final two authors in the author list were inadvertently inverted during the production process. This error has now been corrected in both the PDF and HTML versions of the Article. ... Read more >>

Nat Commun (Nature communications)
[2018, 9(1):646]

Cited: 2 times

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Wnt Signaling in Ewing Sarcoma, Osteosarcoma, and Malignant Peripheral Nerve Sheath Tumors.

Matthew G Pridgeon, Patrick J Grohar, Matthew R Steensma, Bart O Williams,

PURPOSE OF REVIEW:Wnt signaling plays a central role in development and homeostasis, and its dysregulation is a common event in many types of human cancer. Here we explore in detail the contributions of Wnt signaling to the initiation and maintenance of three types of saroma: Ewing sarcoma, osteosarcoma, and malignant ... Read more >>

Curr Osteoporos Rep (Current osteoporosis reports)
[2017, 15(4):239-246]

Cited: 10 times

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Sclt1 deficiency causes cystic kidney by activating ERK and STAT3 signaling.

Jianshuang Li, Di Lu, Huadie Liu, Bart O Williams, Paul A Overbeek, Brendan Lee, Ling Zheng, Tao Yang,

Ciliopathies form a group of inherited disorders sharing several clinical manifestations because of abnormal cilia formation or function, and few treatments have been successful against these disorders. Here, we report a mouse model with mutated Sclt1 gene, which encodes a centriole distal appendage protein important for ciliogenesis. Sodium channel and ... Read more >>

Hum. Mol. Genet. (Human molecular genetics)
[2017, 26(15):2949-2960]

Cited: 10 times

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Deletion of tumor suppressors adenomatous polyposis coli and Smad4 in murine luminal epithelial cells causes invasive prostate cancer and loss of androgen receptor expression.

Kenneth C Valkenburg, Angelo M De Marzo, Bart O Williams,

Prostate cancer is the most diagnosed non-skin cancer in the US and kills approximately 27,000 men per year in the US. Additional genetic mouse models are needed that recapitulate the heterogeneous nature of human prostate cancer. The Wnt/beta-catenin signaling pathway is important for human prostate tumorigenesis and metastasis, and also ... Read more >>

Oncotarget (Oncotarget)
[2017, 8(46):80265-80277]

Cited: 5 times

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Low-Density Lipoprotein Receptor-Related Proteins in Skeletal Development and Disease.

Tao Yang, Bart O Williams,

The identification of the low-density lipoprotein receptor (LDLR) provided a foundation for subsequent studies in lipoprotein metabolism, receptor-mediated endocytosis, and many other fundamental biological functions. The importance of the LDLR led to numerous studies that identified homologous molecules and ultimately resulted in the description of the LDL-receptor superfamily, a group ... Read more >>

Physiol. Rev. (Physiological reviews)
[2017, 97(3):1211-1228]

Cited: 5 times

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Wnt5a promotes Frizzled-4 signalosome assembly by stabilizing cysteine-rich domain dimerization.

Zachary J DeBruine, Jiyuan Ke, Kaleeckal G Harikumar, Xin Gu, Peter Borowsky, Bart O Williams, Wenqing Xu, Laurence J Miller, H Eric Xu, Karsten Melcher,

Wnt/β-catenin signaling is activated when extracellular Wnt ligands bind Frizzled (FZD) receptors at the cell membrane. Wnts bind FZD cysteine-rich domains (CRDs) with high affinity through a palmitoylated N-terminal "thumb" and a disulfide-stabilized C-terminal "index finger," yet how these binding events trigger receptor activation and intracellular signaling remains unclear. Here ... Read more >>

Genes Dev (Genes & development)
[2017, 31(9):916-926]

Cited: 19 times

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Osteoblast-specific deletion of Hrpt2/Cdc73 results in high bone mass and increased bone turnover.

Casey J Droscha, Cassandra R Diegel, Nicole J Ethen, Travis A Burgers, Mitchell J McDonald, Kevin A Maupin, Agni S Naidu, PengFei Wang, Bin T Teh, Bart O Williams,

Inactivating mutations that lead to loss of heterozygosity within the HRPT2/Cdc73 gene are directly linked to the development of primary hyperparathyroidism, parathyroid adenomas, and ossifying fibromas of the jaw (HPT-JT). The protein product of the Cdc73 gene, parafibromin, is a core member of the polymerase-associated factors (PAF) complex, which coordinates ... Read more >>

Bone (Bone)
[2017, 98:68-78]

Cited: 3 times

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LRP5: From bedside to bench to bone.

Bart O Williams,

A role for low-density lipoprotein-related receptor 5 (LRP5) in human bone was first established by the identification of genetic alterations that led to dramatic changes in bone mass. Shortly thereafter, mutations that altered the function of the sclerostin (SOST) gene were also associated with altered human bone mass. Subsequent studies ... Read more >>

Bone (Bone)
[2017, 102:26-30]

Cited: 8 times

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