Francoise Coustry
Overview
Explore the profile of Francoise Coustry including associated specialties, affiliations and a list of published articles.
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Articles
15
Citations
413
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0
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Recent Articles
1.
Hecht J, Veerisetty A, Hossain M, Patra D, Chiu F, Coustry F, et al.
Int J Mol Sci
. 2021 Sep;
22(17).
PMID: 34502142
Pseudoachondroplasia (PSACH), a short limb skeletal dysplasia associated with premature joint degeneration, is caused by misfolding mutations in cartilage oligomeric matrix protein (COMP). Here, we define mutant-COMP-induced stress mechanisms that...
2.
Hecht J, Veerisetty A, Wu J, Coustry F, Hossain M, Chiu F, et al.
Am J Pathol
. 2021 Jun;
191(9):1624-1637.
PMID: 34116024
Increasing numbers of people are living with osteoarthritis (OA) due to aging and obesity, creating an urgent need for effective treatment and preventions. Two top risk factors for OA, age...
3.
Hecht J, Coustry F, Veerisetty A, Hossain M, Posey K
JBMR Plus
. 2021 Mar;
5(3):e10456.
PMID: 33778324
Misfolding mutations in cartilage oligomeric matrix protein (COMP) cause it to be retained within the endoplasmic reticulum (ER) of chondrocytes, stimulating a multitude of damaging cellular responses including ER stress,...
4.
Posey K, Coustry F, Veerisetty A, Hossain M, Gambello M, Hecht J
Am J Pathol
. 2018 Dec;
189(1):132-146.
PMID: 30553437
Cartilage oligomeric matrix protein (COMP) is a large, multifunctional extracellular protein that, when mutated, is retained in the rough endoplasmic reticulum (ER). This retention elicits ER stress, inflammation, and oxidative...
5.
Posey K, Coustry F, Hecht J
Matrix Biol
. 2018 Mar;
71-72:161-173.
PMID: 29530484
Cartilage oligomeric matrix protein (COMP) is a large pentameric glycoprotein that interacts with multiple extracellular matrix proteins in cartilage and other tissues. While, COMP is known to play a role...
6.
Coustry F, Posey K, Maerz T, Baker K, Abraham A, Ambrose C, et al.
Matrix Biol
. 2018 Jan;
67:75-89.
PMID: 29309831
Mutations in COMP (cartilage oligomeric matrix protein) cause severe long bone shortening in mice and humans. Previously, we showed that massive accumulation of misfolded COMP in the ER of growth...
7.
Posey K, Coustry F, Veerisetty A, Hossain M, Gattis D, Booten S, et al.
Mol Ther
. 2017 Feb;
25(3):705-714.
PMID: 28162960
Mutations in cartilage oligomeric matrix protein cause pseudoachondroplasia, a severe disproportionate short stature disorder. Mutant cartilage oligomeric matrix protein produces massive intracellular retention of cartilage oligomeric matrix protein, stimulating ER...
8.
Antioxidant and anti-inflammatory agents mitigate pathology in a mouse model of pseudoachondroplasia
Posey K, Coustry F, Veerisetty A, Hossain M, Alcorn J, Hecht J
Hum Mol Genet
. 2015 Apr;
24(14):3918-28.
PMID: 25859006
Pseudoachondroplasia (PSACH), a severe short-limb dwarfing condition, results from mutations that cause misfolding of the cartilage oligomeric matrix protein (COMP). Accumulated COMP in growth plate chondrocytes activates endoplasmic reticulum stress,...
9.
Posey K, Coustry F, Veerisetty A, Liu P, Alcorn J, Hecht J
J Bone Miner Res
. 2013 Nov;
29(5):1258-68.
PMID: 24194321
Mutations in the gene encoding cartilage oligomeric matrix protein (COMP) cause pseudoachondroplasia (PSACH), a severe dwarfing condition. Pain, a significant complication, has generally been attributed to joint abnormalities and erosion...
10.
Coustry F, Posey K, Liu P, Alcorn J, Hecht J
Am J Pathol
. 2011 Dec;
180(2):738-48.
PMID: 22154936
Mutations in the cartilage oligomeric matrix protein gene (COMP) cause pseudoachondroplasia (PSACH). This dysplasia results from the intracellular retention of mutant COMP protein and premature death of growth-plate chondrocytes. Toward...