Abstract:
:Fras1 and the structurally related proteins Frem1, Frem2, and Frem3, comprise a novel family of extracellular matrix proteins, which localize in a similar fashion underneath the lamina densa of epithelial basement membranes. They are involved in the structural adhesion of the skin epithelium to its underlying mesenchyme. Deficiency in the individual murine Fras1/Frem genes gives rise to the bleb phenotype, which is equivalent to the human hereditary disorder Fraser syndrome, characterized by cryptophthalmos (hidden eyes), embryonic skin blistering, renal agenesis, and syndactyly. Recent studies revealed a functional cooperation between the Fras1/Frem gene products, in which Fras1, Frem1 and Frem2 are simultaneously stabilized at the lowermost region of the basement membrane by forming a macromolecular ternary complex. Loss of any of these proteins results in the collapse of the protein assembly, thus providing a molecular explanation for the highly similar phenotypic defects displayed by the respective mutant mice. Here, we summarize the current knowledge regarding the structure, function, and interplay between the proteins of the Fras1/Frem family and further propose a possible scenario for the evolution of the corresponding genes.
journal_name
Connect Tissue Resjournal_title
Connective tissue researchauthors
Petrou P,Makrygiannis AK,Chalepakis Gdoi
10.1080/03008200802148025subject
Has Abstractpub_date
2008-01-01 00:00:00pages
277-82issue
3eissn
0300-8207issn
1607-8438pii
795331836journal_volume
49pub_type
杂志文章abstract::The use of electron microscopic observations to characterize the state of aggregation of collagen molecules in solution has been examined from the point of view that the observed structures must be compatible with the physical chemical properties of the solution. The common procedure of addition of droplets of a colla...
journal_title:Connective tissue research
pub_type: 杂志文章
doi:10.3109/03008207809152630
更新日期:1978-01-01 00:00:00
abstract::Bovine articular chondrocytes cultured in agarose gel comprise a heterogeneous population when judged by morphological and histochemical criteria. The purpose of the present experiments was to compare, under the same conditions of culture, sub-populations of chondrocytes derived from different depths of articular cart...
journal_title:Connective tissue research
pub_type: 杂志文章
doi:10.3109/03008208809016808
更新日期:1988-01-01 00:00:00
abstract::Oral mandibular epithelium determines the position and number of tooth organs. Cranial neural crest-derived ectomesenchyme provides inductive signals for (i) tooth shape, and (ii) sequential determination and expression of several different epithelial-derived enamel proteins. Inductive signalling follows a precise seq...
journal_title:Connective tissue research
pub_type: 杂志文章,评审
doi:10.3109/03008208909023878
更新日期:1989-01-01 00:00:00
abstract::Activation of protein kinase C (PKC) can upregulate tooth eruption molecules such as osteoprotegerin (OPG) and vascular endothelial growth factor (VEGF). In this study, we examined the in vivo gene expression of classic isoforms of PKC in the dental follicle of postnatal rats. The expression level of PKC-alpha was sig...
journal_title:Connective tissue research
pub_type: 杂志文章
doi:10.1080/03008200490882628
更新日期:2004-01-01 00:00:00
abstract::Quantitative levels of mRNAs coding for elastin, types I and III procollagen and gamma-actin were measured in porcine vascular material following balloon catheterization. A balloon catheter was introduced into the thoracic aorta and jugular vein of 3-6 week old pigs; following distention and six days of postoperative ...
journal_title:Connective tissue research
pub_type: 杂志文章
doi:10.3109/03008208809008059
更新日期:1988-01-01 00:00:00
abstract::Muscle-derived cells can differentiate into chondrogenic cells. In our present study, we investigated the pattern of expression of Sox9, a transcription factor known to play a key role in chondrogenesis, in a rat myoblastic cell line, L6. In addition, we evaluated expression of type II collagen and myogenic regulatory...
journal_title:Connective tissue research
pub_type: 杂志文章
doi:10.1080/03008200490514130
更新日期:2004-01-01 00:00:00
abstract::Scanning electron microscopy and high angle X-ray diffraction were used to define the relationship between collagen and elastin of bovine aortic wall. The diffraction pattern shows on one hand that the broad rings at 4.5 A and 9 A, due to elastin, do not orient on stretching and on the other hand, that the collagen ri...
journal_title:Connective tissue research
pub_type: 杂志文章
doi:10.3109/03008207709152610
更新日期:1977-01-01 00:00:00
abstract::Purpose: Complications including arthrofibrosis have been reported after anterior cruciate ligament reconstruction (ACLR) even under accelerated rehabilitation. To overcome this, we developed an animal model of ACLR-induced arthrofibrosis without immobilization.Materials and Methods: Thirteen male Wistar rats were div...
journal_title:Connective tissue research
pub_type: 杂志文章
doi:10.1080/03008207.2019.1693548
更新日期:2019-11-26 00:00:00
abstract:PURPOSE/AIM:Glutamate is one of the signaling molecules responsible for transmission in the central nervous system. Periodontal ligament (PDL) cells were recently reported to express metabotropic glutamate receptors (mGluRs). However, the functions of mGluR signaling in PDL cells or PDL-related cells remain largely unk...
journal_title:Connective tissue research
pub_type: 杂志文章
doi:10.1080/03008207.2016.1195826
更新日期:2016-09-01 00:00:00
abstract::Currently, the mechanism governing the regeneration of the soft tissue-to-bone interface, such as the transition between the anterior cruciate ligament (ACL) and bone, is not known. Focusing on the ACL-to-bone insertion, this study tests the novel hypothesis that interactions between cells from the ligament (fibroblas...
journal_title:Connective tissue research
pub_type: 杂志文章
doi:10.1080/03008207.2016.1230106
更新日期:2016-11-01 00:00:00
abstract::The interaction of sodium dodecyl sulfate with elastin has been studied by complexometric titration. Approximately 1.2 mumoles of protons with a pKapp of 5.45 are taken up by 10 milligrams of insoluble elastin upon the binding of detergent, apparently due to the protonation of normally ionized carboxylate functions in...
journal_title:Connective tissue research
pub_type: 杂志文章
doi:10.3109/03008207809152627
更新日期:1978-01-01 00:00:00
abstract::The thermal stability and solubility profiles of human hypertrophic scar collagen have been measured and compared with those of the collagens present in human normal scar, human normal skin and a lathyritic dermal tissue. The thermal stability profiles of the hypertrophic scar collagens were found to be very similar t...
journal_title:Connective tissue research
pub_type: 杂志文章
doi:10.3109/03008208409152138
更新日期:1984-01-01 00:00:00
abstract:PURPOSE:Our previous study showed that partial-thickness articular cartilage defects (PTCDs) created in immature rats spontaneously healed to resemble normal hyaline cartilage, but that of mature rats did not. To identify molecules involved in the spontaneous cartilage repair observed in this model, gene expression was...
journal_title:Connective tissue research
pub_type: 杂志文章
doi:10.3109/03008207.2015.1121250
更新日期:2016-05-01 00:00:00
abstract::Cell cultures propagated from fetal bovine ligamentum nuchae were found to accumulate an extensive extracellular matrix containing collagen and elastic fibers when maintained for periods up to 8 weeks. The synthesis by these cells of two glycoproteins, designated MFP I and MFP II, which are immunologically related to ...
journal_title:Connective tissue research
pub_type: 杂志文章
doi:10.3109/03008208109152368
更新日期:1981-01-01 00:00:00
abstract::Bone morphogenetic proteins (BMPs) are known to promote periodontal tissue regeneration, while noggin inhibits the biological activities of BMP-2, -4, and -7. To investigate the effect of BMPs and noggin gene transfer on cementogenesis, we used cloned murine cementoblasts (OCCM). Cells were transduced using adenovirus...
journal_title:Connective tissue research
pub_type: 杂志文章
doi:10.1080/03008200490278142
更新日期:2004-01-01 00:00:00
abstract::This study aims to elucidate the feasible geometry of the scaffolds in bone and periodontal tissue engineering. Several biomaterials with different geometries are compared in terms of their patterns of ectopic BMP-induced chondrogenesis and osteogenesis. The materials include a honeycomb-shaped hydroxyapatite (HCHAP) ...
journal_title:Connective tissue research
pub_type: 杂志文章
doi:10.1080/03008200290001104
更新日期:2002-01-01 00:00:00
abstract::The diameter of collagen fibrils has been measured in electron micrographs of the tunica media and adventitia of the common carotid artery of 14 rats (19,262 counts). The fibrils of the media were measured in each of its three interlamellar spaces. The mean fibril diameters in the innermost space and the middle space ...
journal_title:Connective tissue research
pub_type: 杂志文章
doi:10.3109/03008208709002000
更新日期:1987-01-01 00:00:00
abstract::Part of the hyaluronic acid (HA) synthesized in peripheral tissues enters the blood circulation through the lymph. It is rapidly taken up by the endothelial cells in the liver (half-life in blood is 2.5-5.5 minutes) and degraded. Pure primary cultures of liver endothelial cells were obtained by a newly developed techn...
journal_title:Connective tissue research
pub_type: 杂志文章
doi:10.3109/03008208609001971
更新日期:1986-01-01 00:00:00
abstract::Many organisms from a wide variety of taxa produce amorphous calcium carbonate (ACC), despite the fact that it is inherently unstable and relatively soluble in its pure state. These properties also make it difficult to detect and characterize ACC. Raman spectroscopy is a particularly useful method for investigating AC...
journal_title:Connective tissue research
pub_type: 杂志文章,评审
doi:
更新日期:2003-01-01 00:00:00
abstract::Depolymerization of Ch-4-S, Ch-6-S, HA and HP was observed by gel filtration after treatment with 2-10 N NaOH or Ba(OH)2 in the presence of NaBH4 at 80-83 degrees for 1-5 hr. Depolymerized products were isolated after these treatments. 4-Deoxy-alpha-L-threo-4-enohexopyranosyluronic acid residue as the nonreducing end ...
journal_title:Connective tissue research
pub_type: 杂志文章
doi:10.3109/03008207509152343
更新日期:1975-01-01 00:00:00
abstract::Human aortic smooth muscle cells (AoSMCs) change their extracellular matrix composition during aging, with direct effects on cellular events and cell migration. For example, active matrix metalloproteinase-2 (MMP-2) is synthesized only by young AoSMCs, whereas aged cells produce only the inactive zymogen form. The pro...
journal_title:Connective tissue research
pub_type: 杂志文章
doi:10.1080/03008200802143141
更新日期:2008-01-01 00:00:00
abstract::The in-vitro fluoride treatment technique has been introduced to investigate the composite behavior of bone tissue. Bone tissue with different mechanical properties can be obtained by varying the concentration, pH and immersion time in fluoride ion solutions. The chemical and physical changes in intact pieces of bone ...
journal_title:Connective tissue research
pub_type: 杂志文章
doi:
更新日期:2002-01-01 00:00:00
abstract:OBJECTIVE:We investigated whether COMP may modify cartilage metabolism and play a role as an endogenous disease aggravating factor in OA. MATERIALS AND METHODS:Full-length and momomeric COMP was recombinantly expressed in human embryonic kidney cells and purified it via affinity chromatography. Purified COMP was used ...
journal_title:Connective tissue research
pub_type: 杂志文章
doi:10.3109/03008207.2014.951440
更新日期:2014-10-01 00:00:00
abstract::Purpose: Non-pathological child cortical bone (NPCCB) studies can provide clinicians with vital information and insights. However, assessing the anisotropic elastic properties of NPCCB remains a challenge for the biomechanical engineering community. For the first time, this paper provides elastic moduli values for NPC...
journal_title:Connective tissue research
pub_type: 杂志文章
doi:10.1080/03008207.2019.1570170
更新日期:2019-07-01 00:00:00
abstract::In orthodontic tooth movement, prostaglandin E(2) (PGE(2)) released from osteoblasts can alter the normal process of bone remodeling. We examined the effect of compressive force (CF) on PGE(2) production, PGE receptors (Ep1-4) expression, phosphorylation of protein kinase A (p-PKA), and calcium content in Saos-2 cells...
journal_title:Connective tissue research
pub_type: 杂志文章
doi:10.1080/03008200701541775
更新日期:2007-01-01 00:00:00
abstract::Since cartilage is mainly an avascular tissue, chondrocytes exist in a low-level oxygen environment in vivo. In the present study, we investigated the effect of oxygen tension (20%, 5% and 1% gas phase oxygen concentrations) over a 20-day period on the extracellular matrix accumulation of bovine articular chondrocytes...
journal_title:Connective tissue research
pub_type: 杂志文章
doi:10.3109/03008200109014251
更新日期:2001-10-01 00:00:00
abstract::Of more than 2 million segmental bone defects repaired annually with bone autografts and allografts, 15-40% fail. Improving healing rates may be approached with tissue engineering and use of periosteum overlying an allograft. The present study documents gene expression in human periosteum-allograft constructs compared...
journal_title:Connective tissue research
pub_type: 杂志文章
doi:10.3109/03008207.2014.923851
更新日期:2014-08-01 00:00:00
abstract::In the early mineralized enamel crystals, ribbon-like crystals appear near the ameloblasts. Some ribbon-like crystals showed helical or spiral structure within restricted environment during the preparation of embryonic bovine specimens for electron microscope. These specimens did not suffer from the cutting damages no...
journal_title:Connective tissue research
pub_type: 杂志文章
doi:10.3109/03008209809017027
更新日期:1998-01-01 00:00:00
abstract::Cells derived from organ-explant culture of the cartilaginous component of osteochondrophytic spurs of human femoral heads were incubated with [35S]-sulfate in order to study sulfated proteoglycan biosynthesis in vitro. Secondary monolayer cultures incorporated [35S]-sulfate into macromolecules which were recovered in...
journal_title:Connective tissue research
pub_type: 杂志文章
doi:10.3109/03008208409013694
更新日期:1984-01-01 00:00:00
abstract::To explore crucial early molecular events involved in contact healing of the intrasynovial flexor tendon, integrin expression was evaluated at the transcriptional and post-transcriptional levels during the first two weeks following injury, repair and controlled passive motion in a canine model. Specifically, immunohis...
journal_title:Connective tissue research
pub_type: 杂志文章
doi:10.3109/03008209809021505
更新日期:1998-01-01 00:00:00