Results 21 to 30 of about 41,499 (247)
Cartilage-specific ablation of site-1 protease in mice results in the endoplasmic reticulum entrapment of type IIb procollagen and down-regulation of cholesterol and lipid homeostasis. [PDF]
The proprotein convertase site-1 protease (S1P) converts latent ER-membrane bound transcription factors SREBPs and ATF6 to their active forms. SREBPs are involved in cholesterol and fatty acid homeostasis whereas ATF6 is involved in unfolded protein ...
Debabrata Patra +3 more
doaj +1 more source
Role of procollagen mRNA levels in controlling the rate of procollagen synthesis. [PDF]
Two factors must be present for primary avian tendon cells to commit 50% of their total protein production to procollagen: ascorbate and high cell density. Scorbutic primary avian tendon cells at high cell density (greater than 4 X 10(4) cells per cm2) responded to the addition of ascorbate by a sixfold increase in the rate of procollagen synthesis ...
Rowe, L B, Schwarz, R I
openaire +2 more sources
A possible relationship between fibrosis along the route of cerebrospinal fluid (CSF) flow and the subsequent development of hydrocephalus has been indicated in previous studies.
Hao Xu +8 more
doaj +1 more source
Heat shock protein 47 (HSP47), encoded by the SERPINH1 gene, is a molecular chaperone essential for correct folding of collagens. We report a homozygous p.(R222S) substitution in HSP47 in a child with severe osteogenesis imperfecta leading to early ...
Delfien Syx +12 more
doaj +1 more source
Fibronectin matrix as a scaffold for procollagen proteinase binding and collagen processing
The extracellular matrix (ECM) proteins fibronectin (FN) and type I collagen (collagen I) are codistributed in many tissues, and collagens have been shown to depend on an FN matrix for fibrillogenesis.
Jared T Saunders, J. Schwarzbauer
semanticscholar +1 more source
ER-to-Golgi trafficking of procollagen in the absence of large carriers
Secretion and assembly of collagen is fundamental to the function of the extracellular matrix. Defects in the assembly of a collagen matrix lead to pathologies including fibrosis and osteogenesis imperfecta.
Janine McCaughey +3 more
semanticscholar +1 more source
COPII-coated membranes function as transport carriers of intracellular procollagen I
The coat protein complex II (COPII) is essential for the secretion of large cargo, such as the 300 nm precursor fibrils of procollagen I (PC1). Previous work has shown that the CUL3-KLHL12 complex increases the size of COPII vesicles to over 300 nm in ...
A. Gorur +5 more
semanticscholar +1 more source
Background and Aims Arthrofibrosis is a severe scarring condition characterized by joint stiffness and pain. Fundamental to developing arthrofibrotic scars is the accelerated production of procollagen I, a precursor of collagen I molecules that form ...
Benjamin A. Hendy +5 more
doaj +1 more source
Glycine (Gly) substitutions in collagen Gly‐X‐Y repeats disrupt folding of type I procollagen triple helix and cause severe bone fragility and malformations (osteogenesis imperfecta [OI]).
Lynn S. Mirigian +7 more
semanticscholar +1 more source
UnlabelledHepatic stellate cell (HSC) activation is an essential event during alcoholic liver fibrosis. Evidence suggests that adenosine aggravates liver fibrosis via the adenosine A2A receptor (A2AR).
He Wang +7 more
doaj +1 more source

