scholarly journals Structure and expression of fibrillin-2, a novel microfibrillar component preferentially located in elastic matrices

1994 ◽  
Vol 124 (5) ◽  
pp. 855-863 ◽  
Author(s):  
H Zhang ◽  
SD Apfelroth ◽  
W Hu ◽  
EC Davis ◽  
C Sanguineti ◽  
...  
Keyword(s):  
Gene Product ◽  
Elastic Fiber ◽  
Extracellular Proteins ◽  
Connective Tissues ◽  
New Family ◽  
Fibrillin 1 ◽  
Partial Cdna ◽  
Point Determination ◽  
Human Chromosome 5

During the previous cloning of the fibrillin gene (FBN1), we isolated a partial cDNA coding for a fibrillin-like peptide and mapped the corresponding gene (FBN2) to human chromosome 5. (Lee, B., M. Godfrey, E. Vitale, H. Hori, M. G. Mattei, M. Sarfarazi, P. Tsipouras, F. Ramirez, and D. W. Hollister. 1991. Nature [Lond.]. 352:330-334). The study left, however, unresolved whether or not the FBN2 gene product is an extracellular component structurally related to fibrillin. Work presented in this report clarifies this important point. Determination of the entire primary structure of the FBN2 gene product demonstrated that this polypeptide is highly homologous to fibrillin. Immunoelectron microscopy localized both fibrillin proteins to elastin-associated extracellular microfibrils. Finally, immunohistochemistry revealed that the fibrillins co-distribute in elastic and non-elastic connective tissues of the developing embryo, with preferential accumulation of the FBN2 gene product in elastic fiber-rich matrices. These results support the original hypothesis that the fibrillins may have distinct but related functions in the formation and maintenance of extracellular microfibrils. Accordingly, we propose to classify the FBN1 and FBN2 gene products as a new family of extracellular proteins and to name its members fibrillin-1 and fibrillin-2, respectively.

scholarly journals Human Microfibrillar-Associated Protein 4 (MFAP4) Gene Promoter: A TATA-Less Promoter That Is Regulated by Retinol and Coenzyme Q10 in Human Fibroblast Cells

2020 ◽  
Vol 21 (21) ◽  
pp. 8392
Author(s):  
Ying-Ju Lin ◽  
An-Ni Chen ◽  
Xi Jiang Yin ◽  
Chunxiang Li ◽  
Chih-Chien Lin
Keyword(s):  
Coenzyme Q10 ◽  
Elastic Fiber ◽  
Gene Promoter ◽  
Fiber Formation ◽  
Elastic Fibers ◽  
Structural Components ◽  
Connective Tissues ◽  
Human Fibroblast Cells ◽  
Fibrillin 1 ◽  
Species Specific

Elastic fibers are one of the major structural components of the extracellular matrix (ECM) in human connective tissues. Among these fibers, microfibrillar-associated protein 4 (MFAP4) is one of the most important microfibril-associated glycoproteins. MFAP4 has been found to bind with elastin microfibrils and interact directly with fibrillin-1, and then aid in elastic fiber formation. However, the regulations of the human MFAP4 gene are not so clear. Therefore, in this study, we firstly aimed to analyze and identify the promoter region of the human MFAP4 gene. The results indicate that the human MFAP4 promoter is a TATA-less promoter with tissue- and species-specific properties. Moreover, the promoter can be up-regulated by retinol and coenzyme Q10 (coQ10) in Detroit 551 cells.

In situ freezing point determination of cryolite baths utilising resistometer measurements

2014 ◽  
Vol 18 (12) ◽  
pp. 3339-3344 ◽  
Author(s):  
Graeme A. Snook ◽  
Katherine McGregor ◽  
Andrew J. Urban
Keyword(s):  
Freezing Point ◽  
Point Determination

scholarly journals LTBP1 promotes fibrillin incorporation into the extracellular matrix

2021 ◽  
Author(s):  
Matthias Przyklenk ◽  
Veronika Georgieva ◽  
Fabian Metzen ◽  
Sebastian Mostert ◽  
Birgit Kobbe ◽  
...  
Keyword(s):  
Extracellular Matrix ◽  
Matrix Protein ◽  
Wide Spectrum ◽  
Extracellular Matrix Protein ◽  
Connective Tissues ◽  
Pathogenic Variants ◽  
Human Pathology ◽  
Fibrillin 1

LTBP1 is a large extracellular matrix protein and an associated ligand of fibrillin-microfibrils. Knowledge of LTBP1 functions is largely limited to its role in targeting and sequestering TGFβ growth factors within the extracellular matrix, thereby regulating their bioavailability. However, the recent description of a wide spectrum of phenotypes in multiple tissues in patients harboring LTBP1 pathogenic variants suggests a multifaceted role of the protein in the homeostasis of connective tissues. To better understand the human pathology caused by LTBP1 deficiency it is important to investigate its functional role in extracellular matrix formation. In this study, we show that LTBP1 coordinates the incorporation of fibrillin-1 and -2 into the extracellular matrix in vitro. We also demonstrate that this function is differentially exerted by the two isoforms, the short and long forms of LTBP1. Thereby our findings uncover a novel TGFβ-independent LTBP1 function potentially contributing to the development of connective tissue disorders.

Single point determination of intrinsic viscosity

1963 ◽  
Vol 7 (4) ◽  
pp. S30-S31 ◽  
Author(s):  
R. Z. Naar ◽  
H. H. Zabusky ◽  
R. F. Heitmiller
Keyword(s):  
Intrinsic Viscosity ◽  
Single Point ◽  
Point Determination

Test on the Structure of Biological Sequences via Chaos Game Representation

2005 ◽  
Vol 4 (1) ◽  
Author(s):  
Peggy Cénac
Keyword(s):  
Markov Chain ◽  
Statistical Tests ◽  
Biological Sequences ◽  
Chaos Game Representation ◽  
New Family ◽  
Chaos Game ◽  
Tests For Independence ◽  
Game Representation

In this paper biological sequences are modelled by stationary ergodic sequences. A new family of statistical tests to characterize the randomness of the inputs is proposed and analyzed. Tests for independence and for the determination of the appropriate order of a Markov chain are constructed with the Chaos Game Representation (CGR), and applied to several genomes.

Gel point determination of a thermoset prepreg by means of rheology

2019 ◽  
Vol 78 ◽  
pp. 105950 ◽  
Author(s):  
M.R. Martínez-Miranda ◽  
V. García-Martínez ◽  
M.R. Gude
Keyword(s):  
Gel Point ◽  
Point Determination
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