Defining the extracellular matrix using proteomics
A Byron, JD Humphries… - International journal of …, 2013 - Wiley Online Library
The cell microenvironment has a profound influence on the behaviour, growth and survival
of cells. The extracellular matrix (ECM) provides not only mechanical and structural support …
of cells. The extracellular matrix (ECM) provides not only mechanical and structural support …
[HTML][HTML] The unfolded protein response and its relevance to connective tissue diseases
RP Boot-Handford, MD Briggs - Cell and tissue research, 2010 - Springer
The unfolded protein response (UPR) has evolved to counter the stresses that occur in the
endoplasmic reticulum (ER) as a result of misfolded proteins. This sophisticated quality …
endoplasmic reticulum (ER) as a result of misfolded proteins. This sophisticated quality …
The matrilins: modulators of extracellular matrix assembly
AR Klatt, AKA Becker, CD Neacsu, M Paulsson… - The international journal …, 2011 - Elsevier
The matrilins form a family of oligomeric extracellular adaptor proteins that are most strongly
expressed in cartilage but also present in many other extracellular matrices. Matrilins bind to …
expressed in cartilage but also present in many other extracellular matrices. Matrilins bind to …
Armet/Manf and Creld2 are components of a specialized ER stress response provoked by inappropriate formation of disulphide bonds: implications for genetic skeletal …
CL Hartley, S Edwards, L Mullan, PA Bell… - Human molecular …, 2013 - academic.oup.com
Abstract Mutant matrilin-3 (V194D) forms non-native disulphide bonded aggregates in the
rER of chondrocytes from cell and mouse models of multiple epiphyseal dysplasia (MED) …
rER of chondrocytes from cell and mouse models of multiple epiphyseal dysplasia (MED) …
[HTML][HTML] An unfolded protein response is the initial cellular response to the expression of mutant matrilin-3 in a mouse model of multiple epiphyseal dysplasia
S Nundlall, MH Rajpar, PA Bell, C Clowes… - Cell Stress and …, 2010 - Elsevier
Multiple epiphyseal dysplasia (MED) can result from mutations in matrilin-3, a structural
protein of the cartilage extracellular matrix. We have previously shown that in a mouse …
protein of the cartilage extracellular matrix. We have previously shown that in a mouse …
Multiple epiphyseal dysplasia and related disorders: molecular genetics, disease mechanisms, and therapeutic avenues
EP Dennis, PL Greenhalgh‐Maychell… - Developmental …, 2021 - Wiley Online Library
For the vast majority of the 6000 known rare disease the pathogenic mechanisms are poorly
defined and there is little treatment, leading to poor quality of life and high healthcare costs …
defined and there is little treatment, leading to poor quality of life and high healthcare costs …
Diagnosis of mitochondrial DNA depletion syndromes
Mitochondrial DNA (mtDNA) depletion syndromes (MDDS) are a group of clinically
heterogeneous autosomal recessive disorders characterised by a severe quantitative …
heterogeneous autosomal recessive disorders characterised by a severe quantitative …
Identification of MATN3 as a novel prognostic biomarker for gastric cancer through comprehensive TCGA and GEO data mining
P Wang, W Xiao, Y Li, X Wu, H Zhu, Y Tan - Disease Markers, 2021 - Wiley Online Library
Gastric cancer (GC) is still a vital malignant cancer across the world with unsatisfactory
prognostic results. Matrilin‐3 (MATN3) is a member of the extracellular matrix (ECM) protein …
prognostic results. Matrilin‐3 (MATN3) is a member of the extracellular matrix (ECM) protein …
MED resulting from recessively inherited mutations in the gene encoding calcium‐activated nucleotidase CANT1
K Balasubramanian, B Li, D Krakow… - American Journal of …, 2017 - Wiley Online Library
Multiple Epiphyseal Dysplasia (MED) is a relatively mild skeletal dysplasia characterized by
mild short stature, joint pain, and early‐onset osteoarthropathy. Dominantly inherited …
mild short stature, joint pain, and early‐onset osteoarthropathy. Dominantly inherited …
Mechanisms and models of endoplasmic reticulum stress in chondrodysplasia
SE Patterson, CN Dealy - Developmental Dynamics, 2014 - Wiley Online Library
Chondrodysplasias are a group of genetic disorders that affect the development and growth
of cartilage. These disorders can result in extreme short stature, craniofacial defects, joint …
of cartilage. These disorders can result in extreme short stature, craniofacial defects, joint …