Connective Tissue Growth Factor (CTGF)/CCN2 Knockout: A New Murine Model of Craniofacial Development

FACE ◽  
2021 ◽  
pp. 273250162110557
Author(s):  
Joseph T. Tarr ◽  
James P. Bradley
Keyword(s):  
Murine Model ◽  
Craniofacial Development ◽  
Tissue Growth ◽  
Null Mouse ◽  
Molecular Signaling ◽  
Developmental Abnormalities ◽  
Wnt Proteins ◽  
Invaluable Tool ◽  
Human Kind ◽  
Novel Therapeutic

Animal models represent an invaluable tool, which must be judiciously used to provide the greatest benefit to human kind, due to their cost and time effectiveness. The CCN2 null mouse model described in this paper represents a new murine model of craniofacial development. This model is notable for its remarkably consistent phenotype and ease of colony care and propagation. The interaction of CCN2 with the TGF-β, BMP, FGF, EGF, Integrin, and WNT proteins is currently under investigated and signifies a plethora of research opportunities that may help elucidate novel therapeutic options for future patients. This paper presents a descriptive overview of the known craniofacial developmental abnormalities of this model as well as the known molecular signaling aberrances that provide clues to direct future investigative endeavors.

Anti-OX40ligand treatment as a novel therapeutic strategy in a murine model of colitis

2001 ◽  
Vol 120 (5) ◽  
pp. A685-A685
Author(s):  
B SINGH ◽  
V MALMSTROM ◽  
F POWRIE
Keyword(s):  
Murine Model ◽  
Therapeutic Strategy ◽  
Novel Therapeutic

SAT0062 Blockade of connective tissue growth factor (CTGF) ameliorates murine model of rheumatoid arthritis

2013 ◽  
Vol 71 (Suppl 3) ◽  
pp. 491.1-491
Author(s):  
K. Nozawa ◽  
M. Fujishiro ◽  
M. Kawasaki ◽  
A. Yamaguchi ◽  
K. Ikeda ◽  
...  
Keyword(s):  
Rheumatoid Arthritis ◽  
Growth Factor ◽  
Connective Tissue ◽  
Connective Tissue Growth Factor ◽  
Murine Model ◽  
Tissue Growth

scholarly journals Eradicating acute myeloid leukemia in a MllPTD/wt:Flt3ITD/wt murine model: a path to novel therapeutic approaches for human disease

Blood ◽  
2013 ◽  
Vol 122 (23) ◽  
pp. 3778-3783 ◽  
Author(s):  
Kelsie M. Bernot ◽  
John S. Nemer ◽  
Ramasamy Santhanam ◽  
Shujun Liu ◽  
Nicholas A. Zorko ◽  
...  
Keyword(s):  
Acute Myeloid Leukemia ◽  
Murine Model ◽  
Human Disease ◽  
Myeloid Leukemia ◽  
Liposomal Formulation ◽  
Therapeutic Approaches ◽  
Curative Therapy ◽  
Key Points ◽  
Acute Myeloid ◽  
Novel Therapeutic

Key Points The MllPTD/wt:Flt3ITD/wt mouse is a relevant AML model in which the miR-29b–mediated epigenetics-kinome crosstalk is targetable by bortezomib. An original liposomal formulation of bortezomib eradicates AML and yields curative therapy for MllPTD/wt:Flt3ITD/wt AML.

scholarly journals Influence of PAI-1 on Adipose Tissue Growth and Metabolic Parameters in a Murine Model of Diet-Induced Obesity

2000 ◽  
Vol 20 (4) ◽  
pp. 1150-1154 ◽  
Author(s):  
P. E. Morange ◽  
H. R. Lijnen ◽  
M. C. Alessi ◽  
F. Kopp ◽  
D. Collen ◽  
...  
Keyword(s):  
Adipose Tissue ◽  
Murine Model ◽  
Tissue Growth ◽  
Metabolic Parameters ◽  
Diet Induced Obesity ◽  
Pai 1

scholarly journals Development of Normal and Cleft Palate: A Central Role for Connective Tissue Growth Factor (CTGF)/CCN2

2018 ◽  
Vol 6 (3) ◽  
pp. 18 ◽  
Author(s):  
Joseph Tarr ◽  
Alex Lambi ◽  
James Bradley ◽  
Mary Barbe ◽  
Steven Popoff
Keyword(s):  
Growth Factors ◽  
Growth Factor ◽  
Connective Tissue ◽  
Cleft Palate ◽  
Signaling Pathways ◽  
Connective Tissue Growth Factor ◽  
Tissue Growth ◽  
Altered Expression ◽  
Palate Development ◽  
Wnt Proteins

Development of the palate is the result of an organized series of events that require exquisite spatial and temporal regulation at the cellular level. There are a myriad of growth factors, receptors and signaling pathways that have been shown to play an important role in growth, elevation and/or fusion of the palatal shelves. Altered expression or activation of a number of these factors, receptors and signaling pathways have been shown to cause cleft palate in humans or mice with varying degrees of penetrance. This review will focus on connective tissue growth factor (CTGF) or CCN2, which was recently shown to play an essential role in formation of the secondary palate. Specifically, the absence of CCN2 in KO mice results in defective cellular processes that contribute to failure of palatal shelf growth, elevation and/or fusion. CCN2 is unique in that it has been shown to interact with a number of other factors important for palate development, including bone morphogenetic proteins (BMPs), fibroblast growth factors (FGFs), epidermal growth factor (EGF), Wnt proteins and transforming growth factor-βs (TGF-βs), thereby influencing their ability to bind to their receptors and mediate intracellular signaling. The role that these factors play in palate development and their specific interactions with CCN2 will also be reviewed. Future studies to elucidate the precise mechanisms of action for CCN2 and its interactions with other regulatory proteins during palatogenesis are expected to provide novel information with the potential for development of new pharmacologic or genetic treatment strategies for clinical intervention of cleft palate during development.

scholarly journals A novel therapeutic trial of homogentisic aciduria in a murine model of alkaptonuria

1999 ◽  
Vol 44 (2) ◽  
pp. 79-84 ◽  
Author(s):  
Yasuyo Suzuki ◽  
Kazumi Oda ◽  
Yasuji Yoshikawa ◽  
Toyoki Maeda ◽  
T. Suzuki
Keyword(s):  
Murine Model ◽  
Therapeutic Trial ◽  
Novel Therapeutic

The Cranial Base in Craniofacial Development: a Gene Therapy Study

2007 ◽  
Vol 86 (10) ◽  
pp. 956-961 ◽  
Author(s):  
S. Kyrkanides ◽  
P. Kambylafkas ◽  
J.H. Miller ◽  
R.H. Tallents ◽  
J.E. Puzas
Keyword(s):  
Gene Therapy ◽  
Cranial Base ◽  
Craniofacial Development ◽  
Craniofacial Morphology ◽  
Developmental Abnormalities ◽  
Oxygenase Activity ◽  
Skeletal Defects ◽  
Cox 2 ◽  
Development Analysis

The etiology of midface retrusion remains largely unclear. We hypothesized that the cranial base synchondroses play a key role in the development of the craniofacial skeleton in the Sandhoff mouse model. We observed that developmental abnormalities of the cranial base synchondroses involving proliferative chondrocytes are important in craniofacial growth and development. Neonatal restitution of β-hexosaminidase in mutant mice by gene therapy successfully ameliorated the attendant skeletal defects and restored craniofacial morphology in vivo, suggesting this as a critical temporal window in craniofacial development. Analysis of our data implicates parathyroid-related peptide (PTHrP) and cyclo-oxygenase-2 (COX-2) as possible factors underlying the development of the aforementioned skeletal defects. Hence, timely restitution of a genetic deficiency or, alternatively, the restoration of PTHrP or cyclo-oxygenase activity by the administration of PTH and/or non-steroidal anti-inflammatory drugs or COX-2 selective inhibitors to affected individuals may prove beneficial in the management of midface retrusion.

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