Epidermolysis Bullosa Dystrophica
Form of epidermolysis bullosa characterized by atrophy of blistered areas, severe scarring, and nail changes. It is most often present at birth or in early infancy and occurs in both autosomal dominant and recessive forms. All forms of dystrophic epidermolysis bullosa result from mutations in COLLAGEN TYPE VII, a major component fibrils of BASEMENT MEMBRANE and EPIDERMIS.
Collagen Type VII
Collagen
Collagen Type I
Mucopolysaccharidosis VII
Factor VII
Collagen Type III
Glycogen Storage Disease Type VII
An autosomal recessive glycogen storage disease in which there is deficient expression of 6-phosphofructose 1-kinase in muscle (PHOSPHOFRUCTOKINASE-1, MUSCLE TYPE) resulting in abnormal deposition of glycogen in muscle tissue. These patients have severe congenital muscular dystrophy and are exercise intolerant.
Epidermolysis Bullosa Acquisita
Collagen Type IV
A non-fibrillar collagen found in the structure of BASEMENT MEMBRANE. Collagen type IV molecules assemble to form a sheet-like network which is involved in maintaining the structural integrity of basement membranes. The predominant form of the protein is comprised of two alpha1(IV) subunits and one alpha2(IV) subunit, however, at least six different alpha subunits can be incorporated into the heterotrimer.
Collagen Type II
Collagen Type V
A fibrillar collagen found widely distributed as a minor component in tissues that contain COLLAGEN TYPE I and COLLAGEN TYPE III. It is a heterotrimeric molecule composed of alpha1(V), alpha2(V) and alpha3(V) subunits. Several forms of collagen type V exist depending upon the composition of the subunits that form the trimer.
Fibrillar Collagens
Basement Membrane
A darkly stained mat-like EXTRACELLULAR MATRIX (ECM) that separates cell layers, such as EPITHELIUM from ENDOTHELIUM or a layer of CONNECTIVE TISSUE. The ECM layer that supports an overlying EPITHELIUM or ENDOTHELIUM is called basal lamina. Basement membrane (BM) can be formed by the fusion of either two adjacent basal laminae or a basal lamina with an adjacent reticular lamina of connective tissue. BM, composed mainly of TYPE IV COLLAGEN; glycoprotein LAMININ; and PROTEOGLYCAN, provides barriers as well as channels between interacting cell layers.
Procollagen
Collagen Type VI
Collagen Type XI
Skin
Epidermolysis Bullosa
Receptors, Collagen
Collagen receptors are cell surface receptors that modulate signal transduction between cells and the EXTRACELLULAR MATRIX. They are found in many cell types and are involved in the maintenance and regulation of cell shape and behavior, including PLATELET ACTIVATION and aggregation, through many different signaling pathways and differences in their affinities for collagen isoforms. Collagen receptors include discoidin domain receptors, INTEGRINS, and glycoprotein VI.
Factor VII Deficiency
Ehlers-Danlos Syndrome
A heterogeneous group of autosomally inherited COLLAGEN DISEASES caused by defects in the synthesis or structure of FIBRILLAR COLLAGEN. There are numerous subtypes: classical, hypermobility, vascular, and others. Common clinical features include hyperextensible skin and joints, skin fragility and reduced wound healing capability.
Fibril-Associated Collagens
Extracellular Matrix Proteins
Macromolecular organic compounds that contain carbon, hydrogen, oxygen, nitrogen, and usually, sulfur. These macromolecules (proteins) form an intricate meshwork in which cells are embedded to construct tissues. Variations in the relative types of macromolecules and their organization determine the type of extracellular matrix, each adapted to the functional requirements of the tissue. The two main classes of macromolecules that form the extracellular matrix are: glycosaminoglycans, usually linked to proteins (proteoglycans), and fibrous proteins (e.g., COLLAGEN; ELASTIN; FIBRONECTINS; and LAMININ).
Extracellular Matrix
Fibroblasts
Pepsin A
Formed from pig pepsinogen by cleavage of one peptide bond. The enzyme is a single polypeptide chain and is inhibited by methyl 2-diaazoacetamidohexanoate. It cleaves peptides preferentially at the carbonyl linkages of phenylalanine or leucine and acts as the principal digestive enzyme of gastric juice.
Cells, Cultured
Collagen Type XVIII
Microbial Collagenase
A metalloproteinase which degrades helical regions of native collagen to small fragments. Preferred cleavage is -Gly in the sequence -Pro-Xaa-Gly-Pro-. Six forms (or 2 classes) have been isolated from Clostridium histolyticum that are immunologically cross-reactive but possess different sequences and different specificities. Other variants have been isolated from Bacillus cereus, Empedobacter collagenolyticum, Pseudomonas marinoglutinosa, and species of Vibrio and Streptomyces. EC 3.4.24.3.
Amnion
Laminin
Collagen Type X
Cartilage
Collagen Type XII
Fibronectins
Glycoproteins found on the surfaces of cells, particularly in fibrillar structures. The proteins are lost or reduced when these cells undergo viral or chemical transformation. They are highly susceptible to proteolysis and are substrates for activated blood coagulation factor VIII. The forms present in plasma are called cold-insoluble globulins.
Hydroxyproline
Mucopolysaccharidoses
Molecular Sequence Data
Descriptions of specific amino acid, carbohydrate, or nucleotide sequences which have appeared in the published literature and/or are deposited in and maintained by databanks such as GENBANK, European Molecular Biology Laboratory (EMBL), National Biomedical Research Foundation (NBRF), or other sequence repositories.
RNA, Messenger
RNA sequences that serve as templates for protein synthesis. Bacterial mRNAs are generally primary transcripts in that they do not require post-transcriptional processing. Eukaryotic mRNA is synthesized in the nucleus and must be exported to the cytoplasm for translation. Most eukaryotic mRNAs have a sequence of polyadenylic acid at the 3' end, referred to as the poly(A) tail. The function of this tail is not known for certain, but it may play a role in the export of mature mRNA from the nucleus as well as in helping stabilize some mRNA molecules by retarding their degradation in the cytoplasm.
Collagen Diseases
Historically, a heterogeneous group of acute and chronic diseases, including rheumatoid arthritis, systemic lupus erythematosus, progressive systemic sclerosis, dermatomyositis, etc. This classification was based on the notion that "collagen" was equivalent to "connective tissue", but with the present recognition of the different types of collagen and the aggregates derived from them as distinct entities, the term "collagen diseases" now pertains exclusively to those inherited conditions in which the primary defect is at the gene level and affects collagen biosynthesis, post-translational modification, or extracellular processing directly. (From Cecil Textbook of Medicine, 19th ed, p1494)
Cryoultramicrotomy
Glycogen Storage Disease
Connective Tissue
Decorin
A small leucine-rich proteoglycan that interacts with FIBRILLAR COLLAGENS and modifies the EXTRACELLULAR MATRIX structure of CONNECTIVE TISSUE. Decorin has also been shown to play additional roles in the regulation of cellular responses to GROWTH FACTORS. The protein contains a single glycosaminoglycan chain and is similar in structure to BIGLYCAN.
Collagen Type IX
Amino Acid Sequence
Microscopy, Electron
Microscopy using an electron beam, instead of light, to visualize the sample, thereby allowing much greater magnification. The interactions of ELECTRONS with specimens are used to provide information about the fine structure of that specimen. In TRANSMISSION ELECTRON MICROSCOPY the reactions of the electrons that are transmitted through the specimen are imaged. In SCANNING ELECTRON MICROSCOPY an electron beam falls at a non-normal angle on the specimen and the image is derived from the reactions occurring above the plane of the specimen.
Fluorescent Antibody Technique
Test for tissue antigen using either a direct method, by conjugation of antibody with fluorescent dye (FLUORESCENT ANTIBODY TECHNIQUE, DIRECT) or an indirect method, by formation of antigen-antibody complex which is then labeled with fluorescein-conjugated anti-immunoglobulin antibody (FLUORESCENT ANTIBODY TECHNIQUE, INDIRECT). The tissue is then examined by fluorescence microscopy.
Fibrosis
Cartilage, Articular
Base Sequence
Cornea
The transparent anterior portion of the fibrous coat of the eye consisting of five layers: stratified squamous CORNEAL EPITHELIUM; BOWMAN MEMBRANE; CORNEAL STROMA; DESCEMET MEMBRANE; and mesenchymal CORNEAL ENDOTHELIUM. It serves as the first refracting medium of the eye. It is structurally continuous with the SCLERA, avascular, receiving its nourishment by permeation through spaces between the lamellae, and is innervated by the ophthalmic division of the TRIGEMINAL NERVE via the ciliary nerves and those of the surrounding conjunctiva which together form plexuses. (Cline et al., Dictionary of Visual Science, 4th ed)
Collagen Type VIII
Keratinocytes
Epidermal cells which synthesize keratin and undergo characteristic changes as they move upward from the basal layers of the epidermis to the cornified (horny) layer of the skin. Successive stages of differentiation of the keratinocytes forming the epidermal layers are basal cell, spinous or prickle cell, and the granular cell.
Mycobacterium marinum
Tendons
Peptide Fragments
Disease Models, Animal
Cattle
Gels
Hydroxylysine
Transforming Growth Factor beta1
A subtype of transforming growth factor beta that is synthesized by a wide variety of cells. It is synthesized as a precursor molecule that is cleaved to form mature TGF-beta 1 and TGF-beta1 latency-associated peptide. The association of the cleavage products results in the formation a latent protein which must be activated to bind its receptor. Defects in the gene that encodes TGF-beta1 are the cause of CAMURATI-ENGELMANN SYNDROME.
Transforming Growth Factor beta
A factor synthesized in a wide variety of tissues. It acts synergistically with TGF-alpha in inducing phenotypic transformation and can also act as a negative autocrine growth factor. TGF-beta has a potential role in embryonal development, cellular differentiation, hormone secretion, and immune function. TGF-beta is found mostly as homodimer forms of separate gene products TGF-beta1, TGF-beta2 or TGF-beta3. Heterodimers composed of TGF-beta1 and 2 (TGF-beta1.2) or of TGF-beta2 and 3 (TGF-beta2.3) have been isolated. The TGF-beta proteins are synthesized as precursor proteins.
Gene Expression
Autoantibodies
Phenotype
Immunohistochemistry
Tissue Engineering
Integrins
A family of transmembrane glycoproteins (MEMBRANE GLYCOPROTEINS) consisting of noncovalent heterodimers. They interact with a wide variety of ligands including EXTRACELLULAR MATRIX PROTEINS; COMPLEMENT, and other cells, while their intracellular domains interact with the CYTOSKELETON. The integrins consist of at least three identified families: the cytoadhesin receptors(RECEPTORS, CYTOADHESIN), the leukocyte adhesion receptors (RECEPTORS, LEUKOCYTE ADHESION), and the VERY LATE ANTIGEN RECEPTORS. Each family contains a common beta-subunit (INTEGRIN BETA CHAINS) combined with one or more distinct alpha-subunits (INTEGRIN ALPHA CHAINS). These receptors participate in cell-matrix and cell-cell adhesion in many physiologically important processes, including embryological development; HEMOSTASIS; THROMBOSIS; WOUND HEALING; immune and nonimmune defense mechanisms; and oncogenic transformation.
Electrophoresis, Polyacrylamide Gel
Glycosaminoglycans
Genetic Therapy
Integrin alpha2
HSP47 Heat-Shock Proteins
Chondrogenesis
Mutation
Aggrecans
Integrin alpha1beta1
Integrin alpha1beta1 functions as a receptor for LAMININ and COLLAGEN. It is widely expressed during development, but in the adult is the predominant laminin receptor (RECEPTORS, LAMININ) in mature SMOOTH MUSCLE CELLS, where it is important for maintenance of the differentiated phenotype of these cells. Integrin alpha1beta1 is also found in LYMPHOCYTES and microvascular endothelial cells, and may play a role in angiogenesis. In SCHWANN CELLS and neural crest cells, it is involved in cell migration. Integrin alpha1beta1 is also known as VLA-1 and CD49a-CD29.
Reverse Transcriptase Polymerase Chain Reaction
Osteogenesis Imperfecta
Rabbits
Matrix Metalloproteinase 1
Chick Embryo
Platelet Adhesiveness
Descemet Membrane
Protein Binding
Blotting, Western
Microscopy, Electron, Scanning
Microscopy in which the object is examined directly by an electron beam scanning the specimen point-by-point. The image is constructed by detecting the products of specimen interactions that are projected above the plane of the sample, such as backscattered electrons. Although SCANNING TRANSMISSION ELECTRON MICROSCOPY also scans the specimen point by point with the electron beam, the image is constructed by detecting the electrons, or their interaction products that are transmitted through the sample plane, so that is a form of TRANSMISSION ELECTRON MICROSCOPY.
Tissue Scaffolds
Phosphofructokinase-1
An allosteric enzyme that regulates glycolysis by catalyzing the transfer of a phosphate group from ATP to fructose-6-phosphate to yield fructose-1,6-bisphosphate. D-tagatose- 6-phosphate and sedoheptulose-7-phosphate also are acceptors. UTP, CTP, and ITP also are donors. In human phosphofructokinase-1, three types of subunits have been identified. They are PHOSPHOFRUCTOKINASE-1, MUSCLE TYPE; PHOSPHOFRUCTOKINASE-1, LIVER TYPE; and PHOSPHOFRUCTOKINASE-1, TYPE C; found in platelets, brain, and other tissues.
Procollagen-Lysine, 2-Oxoglutarate 5-Dioxygenase
Non-Fibrillar Collagens
Cell Differentiation
Bone and Bones
Antibodies
Sweat Glands
Biglycan
Desmosomes
A type of junction that attaches one cell to its neighbor. One of a number of differentiated regions which occur, for example, where the cytoplasmic membranes of adjacent epithelial cells are closely apposed. It consists of a circular region of each membrane together with associated intracellular microfilaments and an intercellular material which may include, for example, mucopolysaccharides. (From Glick, Glossary of Biochemistry and Molecular Biology, 1990; Singleton & Sainsbury, Dictionary of Microbiology and Molecular Biology, 2d ed)
Epithelium
Matrix Metalloproteinase 2
Cloning, Molecular
Exons
Corneal Stroma
Arthritis, Experimental
Antigens, CD29
Integrin beta-1 chains which are expressed as heterodimers that are noncovalently associated with specific alpha-chains of the CD49 family (CD49a-f). CD29 is expressed on resting and activated leukocytes and is a marker for all of the very late activation antigens on cells. (from: Barclay et al., The Leukocyte Antigen FactsBook, 1993, p164)
Proline
Protein Conformation
The characteristic 3-dimensional shape of a protein, including the secondary, supersecondary (motifs), tertiary (domains) and quaternary structure of the peptide chain. PROTEIN STRUCTURE, QUATERNARY describes the conformation assumed by multimeric proteins (aggregates of more than one polypeptide chain).
Factor VIIa
Collagen Type XIII
Peptides
Members of the class of compounds composed of AMINO ACIDS joined together by peptide bonds between adjacent amino acids into linear, branched or cyclical structures. OLIGOPEPTIDES are composed of approximately 2-12 amino acids. Polypeptides are composed of approximately 13 or more amino acids. PROTEINS are linear polypeptides that are normally synthesized on RIBOSOMES.
Protein Structure, Tertiary
The level of protein structure in which combinations of secondary protein structures (alpha helices, beta sheets, loop regions, and motifs) pack together to form folded shapes called domains. Disulfide bridges between cysteines in two different parts of the polypeptide chain along with other interactions between the chains play a role in the formation and stabilization of tertiary structure. Small proteins usually consist of only one domain but larger proteins may contain a number of domains connected by segments of polypeptide chain which lack regular secondary structure.
Proteinases of the bone morphogenetic protein-1 family convert procollagen VII to mature anchoring fibril collagen. (1/98)
Collagen VII is the major structural component of the anchoring fibrils at the dermal-epidermal junction in the skin. It is secreted by keratinocytes as a precursor, procollagen VII, and processed into mature collagen during polymerization of the anchoring fibrils. We show that bone morphogenetic protein-1 (BMP-1), which exhibits procollagen C-proteinase activity, cleaves the C-terminal propeptide from human procollagen VII. The cleavage occurs at the BMP-1 consensus cleavage site SYAA/DTAG within the NC-2 domain. Mammalian tolloid-like (mTLL)-1 and -2, two other proteases of the astacin enzyme family, were able to process procollagen VII at the same site in vitro. Immunohistochemical and genetic evidence supported the involvement of these enzymes in cleaving type VII procollagen in vivo. Both BMP-1 and mTLL-1 are expressed in the skin and in cultured cutaneous cells. A naturally occurring deletion in the human COL7A1 gene, 8523del14, which is associated with dystrophic epidermolysis bullosa and eliminates the BMP-1 consensus sequence, abolished processing of procollagen VII, and in mutant skin procollagen VII accumulated at the dermal-epidermal junction. On the other hand, deficiency of BMP-1 in the skin of knockout mouse embryos did not prevent processing of procollagen VII to mature collagen, suggesting that mTLL-1 and/or mTLL-2 can substitute for BMP-1 in the processing of procollagen VII in situ. (+info)The majority of keratinocytes incorporate intradermally injected plasmid DNA regardless of size but only a small proportion of cells can express the gene product. (2/98)
The expression of intradermally injected DNA by keratinocytes is found mainly in the upper and middle layers of the epidermis. To investigate the mechanism of this selective expression, we observed the sequential changes in the distribution of interleukin-6-expressing keratinocytes after the introduction of the interleukin-6 gene. Transgene expression first occurred in basal keratinocytes and subsequently expanded to all epidermal layers and then remained in the upper layers. Semiquantitative analysis indicated that keratinocytes in the lower layers incorporated and lost DNA earlier than those in the upper layers. In order to examine the effect of the DNA size on the transgene expression, we constructed a plasmid containing a full-length 9 kb cDNA of type VII collagen and introduced it into keratinocytes. The expression pattern of type VII collagen in the epidermis was the same as those for smaller genes. This suggests that plasmid size has little or no effect on the expression pattern of the transfected gene. To trace the introduced plasmid, we intradermally injected a green fluorescence protein expression plasmid coupled with a rhodamine flag. Almost all keratinocytes in the injected areas showed rhodamine fluorescence. Furthermore, some cells also expressed green fluorescence protein. A lack of rhodamine fluorescence in the nucleus suggested an impairment of plasmid DNA transport from the cytoplasm to the nucleus. Collectively, our results show that the majority of keratinocytes take up the intradermally injected DNA regardless of its size, but that the transfer of DNA from the cytoplasm to the nucleus is limiting the transgene expression. (+info)The epidermolysis bullosa acquisita antigen (type VII collagen) is present in human colon and patients with crohn's disease have autoantibodies to type VII collagen. (3/98)
Epidermolysis bullosa acquisita is an autoimmune blistering disease of the skin characterized by IgG autoantibodies against type VII collagen. Systemic diseases are often associated with epidermolysis bullosa acquisita, Crohn's disease being the most frequent. This study sought to determine if type VII collagen, the epidermolysis bullosa acquisita autoantigen, was present in normal human colon by western blotting and immunofluorescence. The 290 kDa type VII collagen alpha chain was demonstrated by western blotting in four normal intraoperative colon specimens. Antibodies to type VII collagen labeled the junction between the intestinal epithelium and the lamina propria. We also used an enzyme-linked immunosorbent assay to test sera from patients with Crohn's disease (n = 19), ulcerative colitis (n = 31), celiac disease (n = 17), rheumatoid arthritis (n = 15), and normal controls (n = 16). It was found that 13 of 19 patients with Crohn's disease and four of 31 patients with ulcerative colitis demonstrated reactivity to type VII collagen. Sera from control subjects, patients with celiac disease or rheumatoid arthritis were negative. The sera from Crohn's disease patients also reacted with type VII collagen by immunoblot analysis. It was concluded that patients with inflammatory bowel disease may have IgG autoantibodies to type VII collagen, which exists in both the skin and the gut. (+info)Autoantibodies to type VII collagen mediate Fcgamma-dependent neutrophil activation and induce dermal-epidermal separation in cryosections of human skin. (4/98)
Epidermolysis bullosa acquisita is an autoimmune subepidermal blistering disease associated with autoantibodies to type VII collagen, the major constituent of anchoring fibrils. Previous attempts to demonstrate the blister-inducing potential of autoantibodies to this protein have failed. To address this question, we used an in vitro model involving cryosections of human skin incubated with patients' autoantibodies and leukocytes from healthy donors. We show that sera from 14 of 16 epidermolysis bullosa acquisita patients, in contrast to sera from healthy controls, induced dermal-epidermal separation in the cryosections. Recruitment and activation of neutrophils at the dermal-epidermal junction was necessary for split induction, whereas mononuclear cells were not required. Importantly, patients' autoantibodies affinity-purified against a recombinant form of the noncollagenous 1 domain of type VII collagen retained their blister-inducing capacity in a dose-dependent manner, whereas patients' IgG that was depleted of reactivity to type VII collagen lost this ability. Monoclonal antibody LH7.2 to the noncollagenous 1 domain of type VII collagen also induced subepidermal splits in the cryosections; F(ab')(2) fragments of autoantibodies to type VII collagen were not pathogenic. We demonstrate the capacity of autoantibodies to type VII collagen to trigger an Fcgamma-dependent inflammation leading to split formation in cryosections of human skin. (+info)Genotype-phenotype correlation in italian patients with dystrophic epidermolysis bullosa. (5/98)
Dystrophic epidermolysis bullosa (DEB) is a rare skin disorder that is clinically heterogeneous and is transmitted either in dominant (DDEB) or recessive (RDEB) mode. Nevertheless, all variants of DEB are caused by mutations in type VII collagen gene (COL7A1). We report an analysis of COL7A1 mutations in 51 Italian DEB patients, 27 affected with Hallopeau-Siemens RDEB, 19 with non Hallopeau-Siemens RDEB, two with DDEB, two with pretibial RDEB, and one with inversa RDEB. Forty-one mutations were identified, 18 of which are novel. Mutation consequences were analyzed at the mRNA and protein level and genotype-phenotype correlation was determined. Recessive inheritance of a new case of pretibial RDEB was also established. In RDEB patients, six recurrent mutations were identified: 7344G-->A, 425A-->G, 8441-14del21, 4783-1G-->A, 497insA, and G1664A, the last three being found only in Italian patients. Indeed, haplotype analysis supported propagation of ancestral mutated alleles within the Italian population for these particular mutations. Altogether recurrent mutations account for approximately 43% of RDEB alleles in Italian patients and therefore new DEB patients should first be screened for the presence of these mutations. (+info)Injection of genetically engineered fibroblasts corrects regenerated human epidermolysis bullosa skin tissue. (6/98)
Current therapeutic strategies for genetic skin disorders rely on the complex process of grafting genetically engineered tissue to recipient wound beds. Because fibroblasts synthesize and secrete extracellular matrix, we explored their utility in recessive dystrophic epidermolysis bullosa (RDEB), a blistering disease due to defective extracellular type VII collagen. Intradermal injection of RDEB fibroblasts overexpressing type VII collagen into intact RDEB skin stably restored correctly localized type VII collagen expression in vivo and normalized hallmark RDEB disease features, including subepidermal blistering and anchoring fibril defects. This article was published online in advance of the print edition. The date of publication is available from the JCI website, http://www.jci.org. (+info)Identification of COL7A1 alternative splicing inserting 9 amino acid residues into the fibronectin type III linker domain. (7/98)
Type VII collagen is the major component of anchoring fibrils within the cutaneous basement membrane zone. The large amino-terminal noncollagenous domain of type VII collagen interacts with various extracellular matrix proteins and contributes to the dermal-epidermal attachment. The purpose of this study was to detect alternative splicing of COL7A1 transcript encoding the noncollagenous 1 domain. The alternative splicing in this region may affect interactions of the noncollagenous 1 domain with extracellular matrix proteins and also dermal-epidermal adhesion. Thus we examined expression of the alternative splicing in situations relating to wound healing and skin remodeling that required dermal-epidermal binding and detachment. Amplification of overlapping cDNA from keratinocytes using reverse transcription-polymerase chain reaction identified alternative splicing, which was generated by a different exon 18 acceptor site 27 bp upstream from the common acceptor site. Expression of this alternatively spliced transcript differed among several cell types. The nine amino acid residues GPLTLPLSP from the 27 bp nucleotides were inserted into the linker of fibronectin type III domains. This insertion was suggested to contribute to flexibility of the linker of fibronectin type III domains and may affect the interactions between the noncollagenous 1 domain and extracellular matrix proteins. Treatment with transforming growth factor-beta 1, which is known to promote wound healing and skin remodeling, enhanced the expression of this 27 bp transcript. Furthermore, keratinocyte biopsies from the wound edge of patients with epithelizing skin ulcers showed a significant increase in the 27 bp transcript expression compared with normal keratinocytes from steady-state body sites. These results suggest that amino acid variation of this alternative splicing may have some role in dermal-epidermal adhesion, wound healing, and skin remodeling. To the best of our knowledge, this is the first evidence of alternative splice insertion of a small peptide into the linker region of the fibronectin type III domains, a common motif within modular proteins. (+info)Genetic correction of canine dystrophic epidermolysis bullosa mediated by retroviral vectors. (8/98)
We have assessed the suitability of retroviral vectors for gene therapy of recessive dystrophic epidermolysis bullosa (RDEB) in dogs expressing a mutated collagen type VII. Isolation and analysis of the 9 kb dog collagen type VII cDNA identified the causative genetic mutation G1906S and disclosed the interspecies conservation of collagen type VII. Highly efficient transfer of the wild-type collagen type VII cDNA to both dog RDEB and human primary RDEB collagen type VII-null keratinocytes using recombinant vectors derived from LZRS-Ires-zeo and MSCV retroviruses achieved sustained and permanent expression of the transgene product. The expression and post-translational modification profile of the recombinant collagen type VII was comparable to that of the wild-type counterpart. The recombinant canine collagen type VII in human RDEB keratinocytes and dog cells corrected the observable defects caused by RDEB keratinocytes in cell cultures and in vitro reconstructed skin. Hypermotility was fully reverted in human RDEB keratinocytes, and strongly reduced in the dog RDEB cells. This observation suggests that not only infection efficiency but also high expression levels are required to ensure therapeutic efficacy in the presence of mutated gene products. Our results set the basis for preclinical gene therapy assays in the first immune-competent large animal model for an inherited skin disease and broaden the spectrum of preclinical and clinical applications of retroviral vectors in the transfer of large recombinant genes in epithelial cells. (+info)
Collagen, type VII, alpha 1 - Wikipedia
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Injection of genetically engineered fibroblasts corrects regenerated human epidermolysis bullosa skin tissue
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Novel and emerging therapies in the treatment of recessive dystrophic epidermolysis bullosa
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Collagen, type VII, alpha 1
This gene encodes the alpha chain of type VII collagen. The type VII collagen fibril, composed of three identical alpha ... Epidermolysis bullosa acquisita involves an autoimmune reaction to this form of collagen. Collagen, type VII, alpha 1 forms a ... of type VII collagen is amino-terminal and chimeric. Homology to cartilage matrix protein, the type III domains of fibronectin ... "Cleavage of type VII collagen by interstitial collagenase and type IV collagenase (gelatinase) derived from human skin". The ...
Von Willebrand factor type C domain
Its occurrence in collagen types VI, VII, XII and XIV, the integrins and other proteins by averaged structure predictions". J. ... Collagen, type I, alpha 1 (COL1A1) Collagen, type II, alpha 1 (COL2A1) Collagen, type III, alpha 1 (COL3A1) Collagen, type V, ... collagen types VI, VII, XII and XIV; and other extracellular proteins. Although the majority of VWA-containing proteins are ... "Type A modules: interacting domains found in several non-fibrillar collagens and in other extracellular matrix proteins". ...
Von Willebrand factor type A domain
Its occurrence in collagen types VI, VII, XII and XIV, the integrins and other proteins by averaged structure predictions". J. ... collagen types VI, VII, XII and XIV; and other extracellular proteins. Although the majority of vWA-containing proteins are ... Colombatti A, Bonaldo P, Doliana R (1993). "Type A modules: interacting domains found in several non-fibrillar collagens and in ... This type A domain is the prototype for a protein superfamily (InterPro: IPR036465; see also Pfam clan). The vWA domain is ...
Anchoring fibrils
... (composed largely of type VII collagen) extend from the basal lamina of epithelial cells and attach to the ... Keene, Douglas R.; Sakai, Lynn Y.; Lunstrum, Gregory P.; Morris, Nicholas P.; Burgeson, Robert E. (1987). "Type VII collagen ... Burgeson, Robert E. (1993). "Type VII Collagen, Anchoring Fibrils, and Epidermolysis Bullosa". Journal of Investigative ... Correlation with Type VII Collagen Expression". Journal of Investigative Dermatology. 100 (4): 366-72. doi:10.1111/1523-1747. ...
Type IV collagen
Collagen IV (ColIV or Col4) is a type of collagen found primarily in the basal lamina. The collagen IV C4 domain at the C- ... Collagen IV is the more common usage, as opposed to the older terminology of "type-IV collagen".[citation needed] Collagen IV ... type IV collagen excretion reflects renal morphological alterations and type IV collagen expression in patients with type 2 ... Type III Procollagen, Type IV Collagen, Laminin, Tissue Inhibitor of Metalloproteinase, or Prolyl Hydroxylase?". Alcoholism: ...
Collagen, type XV, alpha 1
Type XV collagen is known to be a tumor suppressor that can be used to understand tumor cells environment. Type XV collagen ... This gene encodes the alpha chain of type XV collagen, a member of the FACIT collagen family (fibril-associated collagens with ... "Epitope-defined monoclonal antibodies against multiplexin collagens demonstrate that type XV and XVIII collagens are expressed ... Hägg PM, Hägg PO, Peltonen S, Autio-Harmainen H, Pihlajaniemi T (June 1997). "Location of type XV collagen in human tissues and ...
Laminin, alpha 5
"Laminin 5 binds the NC-1 domain of type VII collagen". The Journal of Cell Biology. 138 (3): 719-28. doi:10.1083/jcb.138.3.719 ... Laminins function as heterotrimeric complexes of alpha, beta, and gamma chains, with each chain type representing a different ... collagens I, III, IV, c-myc and p53". Archives of Oral Biology. 46 (6): 545-55. doi:10.1016/S0003-9969(01)00014-0. PMID ... 23 (6): 742-7. doi:10.1165/ajrcmb.23.6.4202. PMID 11104726. Parsons SF, Lee G, Spring FA, Willig TN, Peters LL, Gimm JA, Tanner ...
Collagen, type IV, alpha 2
"Duplication of type IV collagen COOH-terminal repeats and species-specific expression of alpha 1(IV) and alpha 2(IV) collagen ... "Entrez Gene: COL4A2 collagen, type IV, alpha 2". Hinek A (1995). "Nature and the multiple functions of the 67-kD elastin-/ ... Like the other members of the type IV collagen gene family, this gene is organized in a head-to-head conformation with another ... This gene encodes one of the six subunits of type IV collagen, the major structural component of basement membranes. The C- ...
Collagen, type I, alpha 2
... types I, II, III, and XI), fibril-associated collagen (type IX), and network-forming collagen (type X) cause a spectrum of ... This gene encodes one of the chains for type I collagen, the fibrillar collagen found in most connective tissues. Mutations in ... Type-I collagen Collagen GRCh38: Ensembl release 89: ENSG00000164692 - Ensembl, May 2017 GRCm38: Ensembl release 89: ... "Entrez Gene: COL1A2 collagen, type I, alpha 2". Byers PH, Wallis GA, Willing MC (1991). "Osteogenesis imperfecta: translation ...
Collagen, type IX, alpha 3
Type IX collagen, a heterotrimeric molecule, is usually found in tissues containing type II collagen, a fibrillar collagen. ... sites in bovine cartilage type IX collagen reveals an antiparallel type II-type IX molecular relationship and type IX to type ... This gene encodes one of the three alpha chains of type IX collagen, the major collagen component of hyaline cartilage. ... "Entrez Gene: COL9A3 collagen, type IX, alpha 3". GeneReviews/NCBI/NIH/UW entry on Multiple Epiphyseal Dysplasia, Dominant ...
Collagen, type VI, alpha 1
"Entrez Gene: COL6A1 collagen, type VI, alpha 1". Bertini E, Pepe G (2002). "Collagen type VI and related disorders: Bethlem ... "Type VI collagen anchors endothelial basement membranes by interacting with type IV collagen". J. Biol. Chem. 272 (42): 26522-9 ... The protein encoded by this gene is the alpha 1 subunit of type VI collagen (alpha1(VI) chain). Mutations in the genes that ... 1996). "Type VI collagen mutations in Bethlem myopathy, an autosomal dominant myopathy with contractures". Nat. Genet. 14 (1): ...
Collagen, type IX, alpha 1
Type IX collagen is usually found in tissues containing type II collagen, a fibrillar collagen. Studies in knockout mice have ... sites in bovine cartilage type IX collagen reveals an antiparallel type II-type IX molecular relationship and type IX to type ... This gene encodes one of the three alpha chains of type IX collagen, a collagen component of hyaline cartilage. ... "Entrez Gene: COL9A1 collagen, type IX, alpha 1". GeneReviews/NCBI/NIH/UW entry on Multiple Epiphyseal Dysplasia, Dominant ...
Collagen, type III, alpha 1
... type III collagen is also an important regulator of the diameter of type I and II collagen fibrils. Type III collagen is also ... types I, II, III, and XI), fibril-associated collagen (type IX), and network-forming collagen (type X) cause a spectrum of ... Type III collagen could also be important in several other human diseases. Increased amounts of type III collagen are found in ... Type III collagen is one of the fibrillar collagens whose proteins have a long, inflexible, triple-helical domain. Type III ...
Collagen, type I, alpha 1
... types I, II, III, and XI), fibril-associated collagen (type IX), and network-forming collagen (type X) cause a spectrum of ... Collagen, type I, alpha 1, also known as alpha-1 type I collagen, is a protein that in humans is encoded by the COL1A1 gene. ... the structure of type I collagen is compromised. Tissues that are rich in type I collagen, such as the skin, bones, and tendons ... Ehlers-Danlos type IV is most attributed to abnormalities in the reticular fibers (collagen Type III). Ehlers-Danlos syndrome, ...
Collagen, type V, alpha 3
... related to type XI collagen and it is possible that the collagen chains of types V and XI constitute a single collagen type ... Fibrillar collagen molecules are trimers that can be composed of one or more types of alpha chains. Type V collagen is found in ... type I collagen and appears to regulate the assembly of heterotypic fibers composed of both type I and type V collagen. This ... "Entrez Gene: COL5A3 collagen, type V, alpha 3". van der Rest M, Garrone R (1991). "Collagen family of proteins". FASEB J. 5 (13 ...
Collagen, type VI, alpha 3
This protein is an alpha chain of type VI collagen that aids in microfibril formation. As part of type VI collagen, this ... This gene encodes the alpha 3 chain, one of the three alpha chains of type VI collagen, a beaded filament collagen found in ... "The 1.6 A structure of Kunitz-type domain from the alpha 3 chain of human type VI collagen". Journal of Molecular Biology. 246 ... "Anisotropic behaviour of the C-terminal Kunitz-type domain of the alpha3 chain of human type VI collagen at atomic resolution ( ...
Collagen, type II, alpha 1
... chain of type II collagen. This gene encodes the alpha-1 chain of type II collagen, a fibrillar collagen found in cartilage and ... "A COL2A1 mutation in achondrogenesis type II results in the replacement of type II collagen by type I and III collagens in ... type 2 by affecting tissues that are rich in type II collagen. Platyspondylic lethal skeletal dysplasia, Torrance type:Fewer ... chain that cannot be incorporated into type II collagen fibers. As a result, cells make a reduced amount of type II collagen. ...
Collagen, type XIX, alpha 1
This gene encodes the alpha chain of type XIX collagen, a member of the FACIT collagen family (fibril-associated collagens with ... other members of this collagen family are found in association with fibril-forming collagens such as type I and II, and serve ... collagen (COL12A1), alpha 1(IX) collagen (COL9A1), and alpha 1(XIX) collagen (COL19A1) to human chromosome 6q12-q13". Genomics ... "Entrez Gene: COL19A1 collagen, type XIX, alpha 1". Yoshioka H, Zhang H, Ramirez F, et al. (1992). "Synteny between the loci for ...
Collagen, type XIV, alpha 1
"Entrez Gene: COL14A1 collagen, type XIV, alpha 1 (undulin)". "COL14A1 - Collagen alpha-1(XIV) chain precursor - Homo sapiens ( ... 2005). "Collagen types XII and XIV are present in basement membrane zones during human embryonic development". J. Mol. Histol. ... Tono-Oka S, Tanase S, Miike T, Tanaka H (1996). "Transient expression of collagen type XIV during muscle development and its ... Collagen alpha-1(XIV) chain is a protein that in humans is encoded by the COL14A1 gene. It likely plays a role in collagen ...
Collagen, type XI, alpha 2
Type XI collagen also helps maintain the spacing and diameter of type II collagen fibrils. Type II collagen is an important ... types I, II, III, and XI), fibril-associated collagen (type IX), and network-forming collagen (type X) cause a spectrum of ... Instead, another type of collagen chain replaces pro-alpha2(XI) to form type XI collagen in the vitreous of the eye. COL11A2 ... The COL11A2 gene produces one component of this type of collagen, called the pro-alpha2(XI) chain. Type XI collagen adds ...
Collagen, type VIII, alpha 2
"Entrez Gene: COL8A2 collagen, type VIII, alpha 2". Shuttleworth CA (1998). "Type VIII collagen". Int. J. Biochem. Cell Biol. 29 ... Illidge C, Kielty C, Shuttleworth A (1998). "The alpha1(VIII) and alpha2(VIII) chains of type VIII collagen can form stable ... 1991). "The alpha 2(VIII) collagen gene. A novel member of the short chain collagen family located on the human chromosome 1". ... the gene encoding the alpha2 chain of type VIII collagen, cause two forms of corneal endothelial dystrophy". Hum Mol Genet. 10 ...
Collagen, type IV, alpha 6
... this gene is organized in a head-to-head conformation with another type IV collagen gene, alpha 5 type IV collagen, so that the ... of type IV collagen in synovial capillaries by immunohistochemistry using a monoclonal antibody against human type IV collagen ... collagen chain and comparison with other type IV collagen genes". J. Biol. Chem. 270 (45): 26863-7. doi:10.1074/jbc.270.45. ... "Entrez Gene: COL4A6 collagen, type IV, alpha 6". Ständer M, Naumann U, Wick W, Weller M (1999). "Transforming growth factor- ...
Collagen, type IV, alpha 4
"Entrez Gene: COL4A4 collagen, type IV, alpha 4". Hinek A (1995). "Nature and the multiple functions of the 67-kD elastin-/ ... 2003). "Type-IV collagen related diseases". J. Nephrol. 16 (2): 314-6. PMID 12768082. Torra R, Tazón-Vega B, Ars E, Ballarín J ... Like the other members of the type IV collagen gene family, this gene is organized in a head-to-head conformation with another ... Murata K, Motayama T, Kotake C (1986). "Collagen types in various layers of the human aorta and their changes with the ...
Collagen, type XVIII, alpha 1
This gene encodes the alpha chain of type XVIII collagen. This collagen is one of the multiplexins, extracellular matrix ... 2002). "Epitope-defined monoclonal antibodies against multiplexin collagens demonstrate that type XV and XVIII collagens are ... "Cloning of cDNA and genomic DNA encoding human type XVIII collagen and localization of the alpha 1(XVIII) collagen gene to ... "Entrez Gene: COL18A1 collagen, type XVIII, alpha 1". Pufe T, Kurz B, Petersen W, et al. (2006). "The influence of biomechanical ...
Collagen, type IV, alpha 3
Collagen alpha-3(IV) chain is a protein that in humans is encoded by the COL4A3 gene. Type IV collagen, the major structural ... 2003). "Type-IV collagen related diseases". J. Nephrol. 16 (2): 314-6. PMID 12768082. Torra R, Tazón-Vega B, Ars E, Ballarín J ... Like the other members of the type IV collagen gene family, this gene is organized in a head-to-head conformation with another ... "Entrez Gene: COL4A3 collagen, type IV, alpha 3 (Goodpasture antigen)". Hinek A (1995). "Nature and the multiple functions of ...
Epidermolysis bullosa dystrophica
... within the human COL7A1 gene encoding the protein type VII collagen (collagen VII). DEB-causing mutations can be either ... Type VII collagen mutations and phenotype-genotype correlations in the dystrophic subtypes". Journal of Medical Genetics. 44 (3 ... which are not related to type VII collagen deficiency. These arise from mutations in the genes encoding other proteins of the ... an autoimmune response against type VII collagen can result in an acquired form of epidermolysis bullosa called epidermolysis ...
Epidermolysis bullosa
... within the human COL7A1 gene encoding the protein type VII collagen (collagen VII). DEB-causing mutations can be either ... Type VII collagen mutations and phenotype-genotype correlations in the dystrophic subtypes". Journal of Medical Genetics. 44 (3 ... Type VII collage formation was observed at the dermis-epidermis junction in significant amounts. A 2020 study demonstrated the ... A 2017 clinical trial with male RDEB (recessive dystrophic EB) patients conducted successful grafting of type VII gene ...
Collagen, type XXVII, alpha 1
Type XXVII collagen is related to the "fibrillar" class of collagens and may play a role in development of the skeleton. ... COL27A1 is a type XXVII collagen. It was discovered by James Pace. This gene appears to be turned on in cartilage, the eye, and ... Fibrillar collagens, such as COL27A1, compose one of the most ancient families of extracellular matrix molecules. They form ... Collagen alpha-1 (XXVII) chain (COL27A1) is a protein that in humans is encoded by the COL27A1 gene. ...
Type V collagen
... as well as in association with tissues containing type I collagen. Autoimmunity against type V collagen is associated with lung ... Type V collagen is a form of fibrillar collagen associated with classical Ehlers-Danlos syndrome. It is found within the dermal ... "Type V collagen controls the initiation of collagen fibril assembly". J. Biol. Chem. 279 (51): 53331-7. doi:10.1074/jbc. ... Collagen+Type+V at the US National Library of Medicine Medical Subject Headings (MeSH) v t e (CS1 errors: missing periodical, ...
Collagen, type VI, alpha 2
"Entrez Gene: COL6A2 collagen, type VI, alpha 2". Bertini E, Pepe G (2002). "Collagen type VI and related disorders: Bethlem ... "Type VI collagen anchors endothelial basement membranes by interacting with type IV collagen". J. Biol. Chem. 272 (42): 26522-9 ... This gene encodes one of the three alpha chains of type VI collagen, a beaded filament collagen found in most connective ... 1996). "Type VI collagen mutations in Bethlem myopathy, an autosomal dominant myopathy with contractures". Nat. Genet. 14 (1): ...
Catenin
Several types of catenins work with N-cadherins to play an important role in learning and memory. Cell-cell adhesion complexes ... Spivey KA, Chung I, Banyard J, Adini I, Feldman HA, Zetter BR (October 2011). "A role for collagen XXIII in cancer cell ... For instance, higher levels of collagen XXIII have been associated with higher levels of catenins in cells. These heightened ... levels of collagen helped facilitate adhesions and anchorage-independent cell growth and provided evidence of collagen XXIII's ...
Equine anatomy
Due to its many twists and turns, it is a common place for a type of horse colic called an impaction. The small colon is 3.0 to ... Once a tendon is damaged the tendon will always be weaker, because the collagen fibres tend to line up in random arrangements ... As tendons develop they lay down collagen, which is the main structural protein of connective tissue. As tendons pass near bony ... It is also known as the "water gut" or "hind gut". It is a cul-de-sac pouch, about 1.2 m (4 ft) long that holds 26 to 30 L (7 ...
Topical fluoride
Fluoride-containing toothpaste can be classified into two types, namely low-fluoride and high-fluoride toothpaste. Low-fluoride ... SDF, in addition to performing the functions of conventional topical fluorides, is suggested to have collagen-conserving ... 7 (3): 79. doi:10.3390/dj7030079. ISSN 2304-6767. PMC 6784469. PMID 31374877. Chen, Lijie; Al-Bayatee, Suma; Khurshid, Zohaib; ... 59 (7): 3213-3230. doi:10.1007/s00394-019-02161-8. ISSN 1436-6207. PMC 7501109. PMID 31853641. Skaare, Anne; Kjærheim, Vibeke; ...
Fibroblast-like synoviocyte
... such as expression of several types of collagens and protein vimentin, a part of cytoskeletal filaments. Unlike fibroblasts, ... Fibroblast-like synoviocytes (FLS) represent a specialised cell type located inside joints in the synovium. These cells play a ... 2010). "Platelets Amplify Inflammation in Arthritis via Collagen-Dependent Microparticle Production". Science. 327 (5965): 580- ... The inner layer is mainly composed of two cell types, specialized macrophages (macrophage-like synovial cells) and fibroblast- ...
ABL (gene)
"Cytoskeletal protein PSTPIP1 directs the PEST-type protein tyrosine phosphatase to the c-Abl kinase to mediate Abl ... is constitutively tyrosine phosphorylated and associated with src homologous and collagen gene (SHC) in chronic myelogenous ... 8 (7): 783-95. doi:10.1101/gad.8.7.783. PMID 7926767. Heaney C, Kolibaba K, Bhat A, Oda T, Ohno S, Fanning S, Druker BJ ( ... 7 (1): 10-6. doi:10.1038/sj.cdd.4400626. PMID 10713716. Era T (2002). "Bcr-Abl is a "molecular switch" for the decision for ...
Senescence
While there may be some validity to the idea that for various types of specific damage detailed below that are by-products of ... Chemical damage to structural proteins can lead to loss of function; for example, damage to collagen of blood vessel walls can ... With respect to specific types of chemical damage caused by metabolism, it is suggested that damage to long-lived biopolymers, ... Horvath S (2013). "DNA methylation age of human tissues and cell types". Genome Biology. 14 (10): R115. doi:10.1186/gb-2013-14- ...
Achilles tendinitis
Excessive pronation of the foot (over 5 degrees) in the subtalar joint is a type of mechanical mechanism that can lead to ... This lack of blood supply can lead to the degradation of collagen fibers and inflammation. Tightness in the calf muscles has ... It is also a known side effect of fluoroquinolone antibiotics such as ciprofloxacin, as are other types of tendinitis. Swelling ... Increased water content and disorganized collagen matrix in tendon lesions may be detected by ultrasonography or magnetic ...
Paleolithic
It is also unlikely that Paleolithic hunter-gatherers were affected by modern diseases of affluence such as type 2 diabetes, ... 19 February 2019). "Exceptionally high δ15N values in collagen single amino acids confirm Neandertals as high-trophic level ... For instance, archaeological evidence from art and bear remains reveals that the bear cult apparently involved a type of ... collagen samples shed new light on their debated diet". Science Daily. Retrieved 21 February 2019. Wilford, John Noble (18 ...
List of diseases (C)
CDG syndrome type 1A CDG syndrome type 1B CDG syndrome type 1C CDG syndrome type 2 CDG syndrome type 3 CDG syndrome type 4 CDK4 ... hemolytic anemia Cold contact urticaria Cold urticaria Cole carpenter syndrome Coleman-Randall syndrome Colitis Collagen ... Tooth disease type 1C Charcot-Marie-Tooth disease type 2A Charcot-Marie-Tooth disease type 2B1 Charcot-Marie-Tooth disease type ... disease type 2C Charcot-Marie-Tooth disease type 2D Charcot-Marie-Tooth disease type 4A Charcot-Marie-Tooth disease type 4B ...
Mir-590 microRNA precursor family
This downregulation sees the removal of post-transcriptional repression of TGF-β1 and TGF-β receptor type II (TGF-βRII), and ... consequent collagen production. miR-590 downregulation has further been shown to be mediated by activation of alpha-7 nicotinic ...
Harvey Cantor
Shinohara ML, Kim J-H, Garcia VA, Cantor H. Engagement of the Type I interferon receptor on dendritic cells inhibits promotion ... Mobilization of natural killer cells inhibits development of collagen-induced arthritis. Proc Natl Acad Sci USA 2011;108:14584 ... an early component of type 1 (cell-mediated) immunity. Science 287: 860-864. †Panoutsakopoulou V, †Sanchirico ME, Huster KM, ...
Koenen's tumor
Tuberous sclerosis is also associated with a second type of angiofibroma, adenoma sebaceum, also termed facial angiofibroma, in ... spindle-shaped or star-shaped fibroblasts and ectatic blood vessels in a dense collagen fiber connective tissue background. In ... 7 (1): 66-70. doi:10.1159/000511743. PMC 7879313. PMID 33614724. Sobanko JF, Dagum AB, Davis IC, Kriegel DA (June 2007). "Soft ... 41 (7): 1992-2010. doi:10.1148/rg.2021210103. PMID 34534018. S2CID 237555929. Longhurst WD, Khachemoune A (November 2015). "An ...
Trimeric autotransporter adhesin
There are seven different type of neck domains. They are as follows: ISneck1, ISneck2, HANS connector, DALL-1, DALL-2, DALL-3, ... Nummelin H, Merckel MC, Leo JC, Lankinen H, Skurnik M, Goldman A (2004). "The Yersinia adhesin YadA collagen-binding domain ... The ISneck domain is a type of neck domain. There are two types of ISneck domain. This first is an ISneck which is interrupted ... There are several types of head domain. Each domain helps the head to bind to a different component of the extracellular matrix ...
Charles M. Russell National Wildlife Refuge
Lewis named this Burnt Lodge Creek (although today it is known as Seven Blackfoot Creek). On May 20, the expedition passed the ... Protein sequencing of the material showed it to be collagen. In November 2010, hunter David Bradt stumbled on an elasmosaur ... There are four major types of habitat within the refuge: river bottom, riparian zones and wetlands, shoreline, and upland ( ... September 7, 2010.[permanent dead link] Accessed 2012-04-27. McRae and Jewell, p. 336. Robbins, p. 266. Billington and Jackson ...
LECT2
... mice made deficient in LECT2 using a gene knockout method developed more severe osteoarthritis induced by anti-type II collagen ... and other types of inflammation-related disorders; the metabolic syndrome and diabetes; and various types of liver disease. The ... Mice made deficient in the Lect2 gene were compared to wild-type mice in a model of high fatty acid diet-induced obesity and ... Several cell types or tissues, e.g. osteoblasts, chondrocytes, cardiac tissue, gastrointestinal smooth muscle cells, and ...
Diagenesis
At high temperatures, the rate of collagen loss will be accelerated and extreme pH can cause collagen swelling and accelerated ... The study of diagenesis in rocks is used to understand the geologic history they have undergone and the nature and type of ... During this phase, most bone collagen is lost and porosity is increased. The dissolution of the mineral phase caused by low pH ... Due to the increase in porosity of bones through collagen loss, the bone becomes susceptible to hydrolytic infiltration where ...
Polyproline helix
A polyproline helix is a type of protein secondary structure which occurs in proteins comprising repeating proline residues. A ... This structure is somewhat similar to that adopted in the fibrous protein collagen, which is composed mainly of proline, ... most commonly in the first residue of a type II β-turn. The "mirror image" PPII backbone dihedral angles (75°, -150°) are ... doi:10.1007/s13361-018-2034-7. PMC 6503664. PMID 30069641. (Articles with short description, Short description matches Wikidata ...
Sprain
Collagen fibers have about a 4% elastic zone where fibers stretch out with increased load on the joint. However, exceeding this ... type of cold compress that is activated similarly to a blood pressure cuff) have been effective in reducing swelling and ... Ligaments are tough, inelastic fibers made of collagen that connect two or more bones to form a joint and are important for ... Chronic sprains are caused by repetitive movements leading to overuse.[citation needed] Ligaments are collagen fibers that ...
Stress incontinence
The most widely used substance, gluteraldehyde crosslinked collagen (GAX collagen) proved to be of value in many patients. The ... This type of intervention can take a person months to learn and would not be a therapy option for people who are not physically ... There is no evidence that one type of incontinence pad is superior with regard to skin health. A pessary is a medical device ... Rortveit, G.; Hannestad, Y. S.; Daltveit, A. K.; Hunskaar, S. (December 2001). "Age- and type-dependent effects of parity on ...
Liposuction
There are numerous types of liposuction. Some can be described as techniques or modalities. Often surgeons will use two or more ... 33 (1): 107-15, vii. doi:10.1016/j.cps.2005.09.001. PMID 16427979. Tabbal, Geo N.; Ahmad, Jamil; Lista, Frank; Rohrich, Rod J ... Referred to as Smart Lipo, this technique uses laser technology to coagulate and tighten the skin and boost collagen ... Referred to as "a vibrating cannula" in research studies, PAL uses a specific type of wand that creates an up and down, ...
Trabeculectomy
Eye surgery List of surgeries by type Cillino, S; Pace F Di; Cillino G; Casuccio A (Sep 2011). "Biodegradable collagen matrix ... Bleb leak - may cause flat bleb; leaking blebs can be revised with the use of ologen collagen matrix or bandage contact lens ... Dietlein TS, Rosentreter A. "Secondary subconjunctival implantation of biodegradable porous collagen matrix for treating ocular ... the use of biodegradable spacer or ologen collagen matrix implant may be implemented. Trabeculectomy is the most common ...
AP-1 transcription factor
"Glycated albumin stimulation of PKC-beta activity is linked to increased collagen IV in mesangial cells". The American Journal ... and angiotensin II-induced type 1 plasminogen activator inhibitor (PAI-1) gene expression in cultured human vascular smooth ... 22 (7): 753-754. doi:10.1038/s41556-020-0540-x. PMID 32591745. S2CID 220071911. Martínez-Zamudio R, Roux P, de Freitas J, et al ... 16 (7): 1695-709. doi:10.1093/emboj/16.7.1695. PMC 1169773. PMID 9130714. Vesely PW, Staber PB, Hoefler G, Kenner L (July 2009 ...
Liver support system
There are various types of hepatocytes that are used in these devices. Porcine hepatocytes are often used due to ease of ... Hepatocytes are suspended in a gel solution such as collagen, which is injected into a series of hollow fibers. In the case of ... A liver support system or diachysis is a type of therapeutic device to assist in performing the functions of the liver. Such ... Basically, three different types of supportive therapies have been developed: bio-artificial, artificial and hybrid liver ...
Glaucoma
... such as the Aquaflow collagen wick, ologen Collagen Matrix, or Xenoplast glaucoma implant. Laser-assisted NPDS is performed ... The most common type is open-angle (wide angle, chronic simple) glaucoma, in which the drainage angle for fluid within the eye ... A number of types of glaucoma surgeries may be used in people who do not respond sufficiently to other measures. Treatment of ... Since then, several types of implants have followed on from the original: the Baerveldt tube shunt, or the valved implants, ...
CUTL1
Chernousov MA, Stahl RC, Carey DJ (1996). "Schwann cells secrete a novel collagen-like adhesive protein that binds N-syndecan ... this deactivated gene is found across many cancer types in this study to be the underlying cause of the disease. Tne CUX1 gene ... 19 (7): 4918-26. doi:10.1128/mcb.19.7.4918. PMC 84297. PMID 10373541. Rong Zeng W, Soucie E, Sung Moon N, Martin-Soudant N, ... 19 (7): 4918-26. doi:10.1128/mcb.19.7.4918. PMC 84297. PMID 10373541. Ottolenghi S, Mantovani R, Nicolis S, Ronchi A, Giglioni ...
Mammary-type myofibroblastoma
... ropey collagen, and spindle cells in a myxoid (i.e. background connective tissue that stains blue or purple rather than the red ... Further complicating the issue, early studies on MFB classified it as one of various types of spindle cell tumors that, except ... Mammary-type myofibroblastoma (MFB), also named mammary and extramammary myofibroblastoma, was first termed myofibrolastoma of ... The World Health Organization, 2020, classified mammary type myofibroblastoma tumors and myofibroblastoma tumors (i.e. ...
Primary effusion lymphoma
Compared to Type I PEL, Type II PEL occurs more often in older individuals, is less often associated with EBV, and more often ... tightly woven collagen fibers) which forms around breast implants. Less frequently, individuals present with extracavitary ... than the malignant cells in Type I PEL. The response to treatment and prognosis of Type II PEL is poor but may be somewhat ... Type II PEL may be a less severe disease than Type I PEL, at lease in certain cases. PEL is generally resistant to cancer ...
Bioarchaeology
"Establishing collagen quality criteria for sulphur isotope analysis of archaeological bone collagen". Archaeological and ... Surface hypoplasias record stressors occurring from about one to seven years, or up to 13 years if the third molar is included ... Many methods tracking these types of changes have been developed using a variety of skeletal series. For instance, in children ... Though bone collagen is abundant in skeletal remains, less than 1% of the tissue is made of sulfur, making it imperative that ...
Halofuginone
... is also an inhibitor of collagen type I gene expression and as a consequence it may inhibit tumor cell growth. ... 8 (3): 311-7. doi:10.1038/nchembio.790. PMC 3281520. PMID 22327401. (Drugs with non-standard legal status, Articles with ...
Collagen Type 1 & 3 Powder 7 oz| Where to Buy Collagen Powder | PipingRock Health Products
Where to Buy Collagen Powder and Other Vitamins & Supplements online at PipingRock.com ... This product is as good as Neo Cell Super Collagen Type 1 & 3 but 1/2 the price when you buy two from Piping Rock.This product ... Ultra Collagen Powder Type I & III, 7 oz (198 g) Bottle. 8601,h2 class=body-more-heading-third,,strong,What is Collagen?,/ ... Ultra Collagen Powder Type I & III, 7 oz (198 g) Bottle. 8601,h2 class=body-more-heading-third,,strong,What is Collagen?,/ ...
Laminin 5 binds the NC-1 domain of type VII collagen - Institut de Génomique Fonctionnelle de Lyon
Genetic basis of Bart's syndrome: A glycine substitution mutation in the type VII collagen gene<...
Genetic basis of Barts syndrome : A glycine substitution mutation in the type VII collagen gene. / Christiano, A. M.; Bart, B ... Genetic basis of Barts syndrome : A glycine substitution mutation in the type VII collagen gene. In: Journal of Investigative ... Dive into the research topics of Genetic basis of Barts syndrome: A glycine substitution mutation in the type VII collagen ... title = "Genetic basis of Barts syndrome: A glycine substitution mutation in the type VII collagen gene", ...
COL7A1 gene: MedlinePlus Genetics
... chain that is used to assemble a larger protein called type VII collagen. Learn about this gene and related health conditions. ... VII) chains that cannot form type VII collagen. As a result, little type VII collagen is available to make anchoring fibrils. ... thin bundles of mature type VII collagen.. Type VII collagen is the major component of structures in the skin called anchoring ... these mutations alter a part of type VII collagen known as the triple helical domain. This region gives type VII collagen its ...
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As we age, collagen depletion can lead to common signs of aging in the skin, hair, nails, muscles, tendons, ligaments, and ... Collagen is a complex structural protein that maintains strength and flexibility throughout the body. ... 6600 MG collagen Type 1 & 3 • Strong hair and nails. • Healthy bones • Clinically tested NeoCell Collagen - 92% had increased ... Super Collagen contains clinically studied BioActive Neocell Collagen which supports healthy collagen formation throughout the ...
Systemic Sclerosis: Background, Pathophysiology, Etiology
... and collagen types I, III, V, and VII. Increased collagen deposition in tissues is a characteristic feature of systemic ... Increased collagen production or disturbances in its degradation can cause excessive collagen deposition in tissues. ... Excessive collagen deposition causes skin and internal organ changes. Many factors, including environmental factors, can lead ... The prognosis depends on the type of systemic sclerosis (SSc). In lSSc, a patients condition can be stable for years. However ...
MMRRC:044366-MU
大疱性表皮松解症 - 参考文献 | BMJ Best Practice
Type VII collagen mutations and phenotype/genotype correlations in the dystrophic subtypes. J Med Genet. 2007;44:181-192. 摘要 ... Type VII collagen is required for Ras-driven human epidermal tumorigenesis. Science. 2005;307:1773-1776. 摘要 ... Intraepidermal type VII collagen. Evidence for abnormal intracytoplasmic processing of a major basement protein in rare ... Intracytoplasmic retention of type VII collagen and dominant dystrophic epidermolysis bullosa: reversal of defect following ...
Gene Expression Signatures for Predicting Prognosis of Squamous Cell and Adenocarcinomas of the Lung | Cancer Research |...
collagen, type VII, α1 COL7A1 7.75 9.66 0.27 204636_at collagen, type XVII, α1 COL17A1 6.43 8.97 0.17 ... 5). Forty-seven of these gene transcripts were mapped to the U133Plus GeneChip used in the validation data set from Duke ... Lung cancer classification: the relationship of disease extent and cell type to survival in a clinical trials population. J ... 7. Garber ME, Troyanskaya OG, Schluens K, et al. Diversity of gene expression in adenocarcinoma of the lung. Proc Natl Acad Sci ...
Microstomia: Background, Problem, Epidemiology
... and collagen types I, III, V, and VII, in the skin and other tissues. The degree of sclerosis increases when profibrotic ... Eventually, fibroblast formation and collagen deposition occur, along with scar tissue formation and contraction. ... types of epidermolysis bullosa, and, occasionally, Down syndrome and hemifacial microsomia (see the images below). ... 7] It generally involves some sort of appliance therapy. In patients with microstomia of longer duration, impairment of ...
Abeona Therapeutics Announces Positive Topline Results with Both Co-Primary Endpoints Met in Pivotal Phase 3 VIITAL™ Study of...
People with RDEB have a defect in the COL7A1 gene, leaving them unable to produce functioning type VII collagen, which is ... EB-101 is being investigated for its ability to enable normal Type VII collagen expression and to facilitate wound healing. The ... Among the benefits of Orphan Drug designation are seven years of market exclusivity following FDA approval, potentially ...
Kindler Syndrome Clinical Presentation: History, Physical, Causes
Best Protein Puddings & Gelatins w/ Reviews & Nutrition Info
Iversen LV[au] - Search Results - PubMed
SMART: VWC domain annotation
Rohit Khosla, M.D. | Stanford Medicine
... patients have mutations in the COL7A1 gene and thus lack functional type VII collagen (C7) protein; they have marked skin ... patients have mutations in the COL7A1 gene and thus lack functional type VII collagen (C7) protein; they have marked skin ... patients have mutations in the COL7A1 gene and thus lack functional type VII collagen (C7) protein; they have marked skin ... Hospitals types included pediatric hospitals, general hospitals, and nonaccredited childrens hospital. For each hospital type ...
What Is The Best Collagen Pills For Men - Spicer Castle
ALL-IN-ONE COLLAGEN SUPPLEMENT - The BeautiBe Multi Collagen Complex give you 5 types of collagen (Type I, II, III, V, and X) ... Our marine collagen capsules are packed with a variety of multi collagen protein types, including collagen type 1 and 3. Our ... Collagen type 2 with types 1, 3, 5, & 10 contain integral proteins and amino acids to restore lost collagen to your skin, hair ... Super-Absorbable All-In-One Super Collagen Blend - Your Collagen needs are covered with all 5 types of collagen hydrolysate, ...
Multiple blisters on the extremities - Clinical Advisor
... against type VII collagen. Anchoring fibrils that serve to anchor the BMZ into the papillary dermis consist in part of type VII ... Evidence that expression of autoimmunity to type VII collagen is HLA class II allele associated. J Invest Dermatol. 1988;91:228 ... 2011;29:365-372, vii. *Gammon WR, Heise ER, Burke WA, et al. Increased frequency of HLA-DR2 in patients with autoantibodies to ... Antibody adherence to the collagen disrupts the anchoring fibrils through an unknown mechanism, causing a subepithelial blister ...
Paul A. Khavari - Publications
Type VII collagen is required for Ras-driven human epidermal tumorigenesis. Science (New York, N.Y.). 307: 1773-6. PMID ... Long-term type VII collagen restoration to human epidermolysis bullosa skin tissue. Human Gene Therapy. 21: 1299-310. PMID ... A laminin-collagen complex drives human epidermal carcinogenesis through phosphoinositol-3-kinase activation. Cancer Research. ... 133: 824-7. PMID 23096709 DOI: 10.1038/Jid.2012.377 0.319. 2012. Lee CS, Ungewickell A, Bhaduri A, Qu K, Webster DE, Armstrong ...
MAKING MOVES IN JOINT HEALTH WITH NATIVE TYPE II COLLAGEN
Discover the benefits of native type II collagen for convenient and low-dose supplements. ... Collagen is becoming increasingly popular in the joint health market. ... This is why hydrolysed collagen is also known as collagen peptides, or denatured type II collagen. Native type II collagen is ... Native type II collagen has been shown to help modulate the immune response against endogenous type II collagen through its ...
POLA BA The Liquid Beauty Collagen Drink 20ml x 12 - Made in Japan - TAKASKI.COM
Also enriched with Herb Quartet extracted from four types of herbs, collagen peptide and olive fruit extract to build a ... Also enriched with Herb Quartet extracted from four types of herbs, collagen peptide and olive fruit extract to build a ... Home Health Supplements POLA BA The Liquid Beauty Collagen Drink 20ml x 12 - Made in Japan. ... POLA BA The Liquid Beauty Collagen Drink is a beauty supplement from POLAs top-of-the-line brand "B.A" (Bio Active). Enriched ...
Titles list | Archive ouverte UNIGE
Porfiria cutânea tardia. Estudo evolutivo das características clínicas e laboratoriais:...
In three cases all the antigens, i.e. BP180 (bullous pemphigoid antigen), laminin, type IV collagen and type VII collagen, were ... in one case type IV collagen was found on the roof and type VII collagen on both sides of the bulla (split occurred on the ... Seven patients were females (25%) and 21 were males (75%). Alcohol intake was the predominant etiological factor in male ... em um caso o colágeno tipo IV foi encontrado no teto e o colágeno tipo VII em ambos os lados da bolha (clivagem na sublâmina ...
Journal of Neuromuscular Diseases - Volume 7, issue 4 - Journals - IOS Press
Article Type: Research Article Abstract: Collagen VI-related dystrophies (COL6-RDs) and Duchenne muscular dystrophy (DMD) cause ... Patients developed respiratory failure at 5.82 months (±2.32) and 13.8 months (±5.6) in Type 1B and Type 1C, respectively (p , ... Objective: To analyze the progression, respiratory interventions and survival based on the type of respiratory support in type ... Article Type: Editorial Abstract: Guillain-Barré Syndrome is a popular eponym that comes from a 1916 paper by Drs. Guillain, ...
Krystal Biotech Receives Positive Opinion from EMA Pediatric Committee on the Pediatric Investigation Plan for B-VEC for the...
IJMS | Free Full-Text | The Anti-Wrinkle Mechanism of Melatonin in UVB Treated HaCaT Keratinocytes and Hairless Mice via...
Notably, melatonin prevented against epidermal thickness and dermal collagen degradation in UVB irradiated hairless mice by ... pro-collagen and cytotoxicity in HaCaT keratinocytes. Additionally, melatonin suppressed the expression of sonic hedgehog (SHH ... Marked loss of fibrillin-positive structures such as collagen type IV, VII and XVII in dermal and epidermal junctions ... Collagen fiber bundles are shown in blue area (Blue arrow) (×100) Scale bars = 40 μm. (e) Effect of melatonin on collagen IV ...
What is new in autoimmune vesicobullous disorders? - Indian Journal of Dermatology, Venereology and Leprology
T and B cells target identical regions of the non-collagenous domain 1 of type VII collagen in EBA. Clin Immunol 2010;1351:99- ... 26] Mueller et al.[27] used Collagen (COL) VII-NC1 ELISA in the diagnosis of EBA and found it to be a powerful tool in ... Clinical Types of Autoimmune Bullous Diseases : The Newly Described Ones. Traditionally, pemphigus has been classified as PV ... Pemphigus vulgaris of the pompholyx type. Eur J Dermatol 2010;20:516-7. ...
Gene expression profiling revealed novel mechanism of action of Taxotere and Furtulon in prostate cancer cells | BMC Cancer |...
PeptidesAnchoring fibrilsCOL7A1Dystrophic epiderm2022ProteinsMutationsSuper CollagenPowder2018Multi Collagen ComplexLamininNeoCell CollagenBody'sConnective tissueGluten-freeSkin'sTendonsLess collagenBeauty CollagenBovine collagenNatural collagenBiotinSkinElasticityTriple helixAmino AcidsCompoundsStimulate201620172002SupplementsNailsStimulatesWrinklesProductionStructuralBasement membraneMoleculesBodyAmountsSodiumRegenerationTissueSupplementExtracellular matrixAbnormalPillsDermisInflammationChromosome
Peptides9
- Using Neocell's technologically advanced hydrolyzation process, large collagen molecules are enzymatically hydrolyzed into small peptides that are both bioavailable and bioactive in the body. (onefamshop.com)
- All-In-One Collagen Supplement: Our collagen supplements have a blend of hydrolyzed collagen peptides from beef (bovine), marine, chicken bone broth, and eggshell membrane sources, as well as types I, II, III, V and X collagen in a small capsule form making it your one-stop-shop for your collagen needs. (spicercastle.com)
- Beauty collagen peptides capsules give your body much needed proteins for regenerative health. (spicercastle.com)
- Give collagen peptides pills a try today! (spicercastle.com)
- Hydrolysed collagen is manufactured via a specific hydrolysis process, where enzymes "cut" the triple helix molecule into smaller pieces, i.e. short-chain peptides. (nutraingredients.com)
- This is why hydrolysed collagen is also known as collagen peptides, or denatured type II collagen. (nutraingredients.com)
- It also features plant-based collagen and peptides to firm and plump up the skin along with niacinamide to brighten skin tone. (beautynetkorea.com)
- Collagen deficiency is noticeable quite quickly because it is collagen peptides that affect the condition of the skin. (labosal.com)
- A 2008 animal study [4] used marine collagen peptides. (labosal.com)
Anchoring fibrils8
- Type VII collagen is the major component of structures in the skin called anchoring fibrils. (medlineplus.gov)
- When type VII collagen is abnormal or missing, anchoring fibrils cannot form properly. (medlineplus.gov)
- As a result, little type VII collagen is available to make anchoring fibrils. (medlineplus.gov)
- When the abnormally shaped collagen molecules are incorporated into anchoring fibrils, they interfere with the fibrils' normal function and prevent them from effectively connecting the epidermis and the dermis. (medlineplus.gov)
- Recent analysis of Bart's original kindred demonstrated ultrastructural abnormalities in the anchoring fibrils and linkage of the inheritance of the disease to the region of chromosome 3 near the type VII collagen gene (COL7A1). (umn.edu)
- It is caused by one or more mutations in a gene called COL7A1, which is responsible for the production of the protein type VII collagen (COL7) tha forms anchoring fibrils that bind the dermis (inner layer of the skin) to the epidermis (outer layer of the skin). (tmcnet.com)
- The reticular lamina contains type III collagen and anchoring fibrils of VII collagen, all secreted by cells of the immediately adjacent connective tissue. (mhmedical.com)
- Type VII collagen is the main component of the anchoring fibrils connecting the basement membrane to the underlying interstitial matrix. (nordicbioscience.com)
COL7A18
- The COL7A1 gene provides instructions for making a protein called pro-α1(VII) chain that is used to assemble a larger protein called type VII collagen. (medlineplus.gov)
- The recessive types of dystrophic epidermolysis bullosa (RDEB) result from mutations in both copies of the COL7A1 gene in each cell. (medlineplus.gov)
- Most of the COL7A1 gene mutations responsible for RDEB-sev gen result in production of abnormally short pro-α1(VII) chains that cannot form type VII collagen. (medlineplus.gov)
- Mutations in the COL7A1 gene can also cause a rare condition called epidermolysis bullosa with congenital localized absence of skin (also known as Bart syndrome or aplasia cutis congenita type VI). (medlineplus.gov)
- These results disclosed a G-to-A transition within exon 73 of COL7A1, which results in a glycine-to-arginine substitution within the triple-helical domain of type VII collagen in affected individuals. (umn.edu)
- deletion in the type VII collagen gene (COL7A1) in Hallopeau? (inserm.fr)
- A. M. Christiano, L. C. Chung-honet, A. Hovnanian, and J. Uitto , Epidermolysis Bullosa to the type VII collagen gene PCRbased detection of two exonic polymorphisms in the human type VII collagen gene (COL7a1) at 3p21 , J. clin. (inserm.fr)
- Caused by mutations within the type VII collagen gene (COL7A1). (cdc.gov)
Dystrophic epiderm3
- Researchers classify dystrophic epidermolysis bullosa into a few major types based on the inheritance pattern and features of the condition. (medlineplus.gov)
- Mutations in the gene encoding type VII collagen ( COLA7A1 ) have been excluded, distinguishing Kindler syndrome from dystrophic epidermolysis bullosa. (medscape.com)
- Mutations in the type VII collagen gene cause dystrophic epidermolysis bullosa. (nordicbioscience.com)
20222
Proteins8
- Collagens are a family of proteins that strengthen and support connective tissues, such as skin, bone, tendons, and ligaments, throughout the body. (medlineplus.gov)
- One of the body's most important and abundant proteins, collagen comprises most of your connective tissues and can be found in your skin, hair, joints, bones and nails. (pipingrock.com)
- The domain is named after the von Willebrand factor (VWF) type C repeat which is found in multidomain protein/multifunctional proteins involved in maintaining homeostasis. (embl.de)
- However, it is worth knowing that in the group of collagen proteins, there are as many as 28 different proteins - in the context of skin health and condition, the most important role is played by collagen types I (80% content) and III (about 15% content). (labosal.com)
- Proteins of animal origin are used in dietary supplements containing collagen. (labosal.com)
- The high level of bioavailability of fish collagen proteins is due to the fact that its structural structure. (labosal.com)
- Marine collagen proteins are in the form of a triple helix [3], which means that it is extremely similar to the collagen found in human skin. (labosal.com)
- In the context of beauty and the fight against imperfections, the best choice will be preparations containing marine collagen proteins. (labosal.com)
Mutations7
- These mutations alter the structure or disrupt the production of the pro-α1(VII) chain protein, which affects the production of type VII collagen. (medlineplus.gov)
- Somewhat less severe forms of RDEB, grouped as the generalized and localized types (RDEB-gen and -loc), are caused by other types of mutations. (medlineplus.gov)
- In many cases, these mutations alter a part of type VII collagen known as the triple helical domain. (medlineplus.gov)
- Mutations that substitute other amino acids for glycine in this region can disrupt the triple-stranded structure of type VII collagen. (medlineplus.gov)
- DDEB can also be caused by other types of mutations, particularly changes that affect the folding of type VII collagen. (medlineplus.gov)
- Mutations in this gene lead to reduced amounts or an alteration in function of collagen VII. (cdc.gov)
- The Stickler syndrome: genotype/phenotype correlation in 10 families with Stickler syndrome resulting from seven mutations in the type II collagen gene locus COL2A1. (cdc.gov)
Super Collagen2
- Super Collagen contains clinically studied BioActive Neocell Collagen which supports healthy collagen formation throughout the body. (onefamshop.com)
- NeoCell Super Collagen with Vitamin C, 360 Collagen Pills, #1 Collagen Tablet. (cherrypicksreviews.com)
Powder4
- PipingRock’s Ultra Collagen Powder delivers the benefits of this protein in an easy-to-use powder! (pipingrock.com)
- For adults, add approximately 1 scoop (6.7 g) of Hydrolyzed Collagen powder 1 to 3 times daily with 6-8 ounces of room temperature water or juice and mix thoroughly. (pipingrock.com)
- Collagen powder will clump in cold water. (pipingrock.com)
- A strong alternative to collagen powder and liquid collagen, collagen supplements are easy to swallow and small enough to hold in your pocket, handbag, or car. (spicercastle.com)
20181
- 2018 Jul 17;7: F1000 Faculty Rev-1097. (bmj.com)
Multi Collagen Complex1
- Our Multi Collagen Complex supplement helps support your health, strength and beauty. (spicercastle.com)
Laminin3
- The basal lamina is a thin meshwork of type IV collagen and laminin produced by the epithelial cells. (mhmedical.com)
- [ 7 ] Trauma has been shown to have a role in the production of basement membrane alterations involving expression of type IV collagen and laminin (alpha 1), thus indicating a possible relationship between these elements and denture stomatitis. (medscape.com)
- For instance, laminin appears as early as the 2-cell stage, entactin/nidogen appears at the 16-cell stage [7] , and fibronectin and type IV collagen appears later in the inner cell mass of 3-4 day-old blastocysts [8] . (cdc.gov)
NeoCell Collagen2
- Clinically tested NeoCell Collagen - 92% had increased skin hydration. (onefamshop.com)
- NeoCell is one of best-selling brand on Amazon for Vitamins, Minerals & Supplements, Our team has recommended top 30 NeoCell Collagen Supplements to make your shopping easy after considering different features about NeoCell Collagen Supplements products of the brand, 53 products have analyzed. (cherrypicksreviews.com)
Body's4
- Through this mode of action native type II collagen is recognised by the immune system as an endogenous substance, i.e. naturally occurring in the body, and deactivates the body's immune response against its own collagen. (nutraingredients.com)
- Your body's collagen levels can start to run low if you do not obtain enough vitamin C or if you smoke, eat too much sugar, or spend too much time in the sun. (iwilife.com)
- All of these can break down the body's collagen, contributing to wrinkles, decreased resiliency, and dry skin. (iwilife.com)
- Unfortunately, with age, the amount of collagen produced decreases in relation to the body's needs. (labosal.com)
Connective tissue5
- In diet and supplementation, collagen plays an important role - a complete protein with a high content of amino acids (including glycine or proline), which is a fundamental element of connective tissue. (labosal.com)
- It is not difficult to guess that the condition of the skin depends on the condition of the connective tissue, which is affected by the amount of collagen. (labosal.com)
- It is essentially a defect in the production of collagen, an essential component of connective tissue . (thezebramom.com)
- Collagen is essential for healthy connective tissue, which is found throughout the body supporting and connecting the different types of tissues and organs, including tendons, ligaments, blood vessels, internal organs, bones, the blood and skin. (thezebramom.com)
- There are a number of different genes responsible for making collagen and connective tissue, so there are different types of EDS depending on which genes are faulty. (thezebramom.com)
Gluten-free1
- Premium Quality: Our non-GMO skin and hair collagen pills are hormone-free, gluten-free and dairy-free. (spicercastle.com)
Skin's4
- A go-to treatment for great lines and wrinkles, Fraxel is a microscopic laser that permeates the skin to enhance the skin's collagen and elastin production. (prscd.com)
- Collagen is a type of protein that supports the elasticity and flexibility of your skin as it has an important role in your skin's structure. (iwilife.com)
- These potent creams and gels (Retin-A, Renova, Avita, and Avage) are acid derivatives of vitamin A. Over time, retinoids stimulate collagen production and increase the skin's water content, which can prevent sagging. (prevention.com)
- It usually develops when changes in fat and collagen (a fiber-like protein in your body) happen beneath the skin's surface. (cdc.gov)
Tendons2
- Each serving supplies types I & III collagen, which supports the health of your skin, bones, tendons, and joints. (pipingrock.com)
- Collagen is the main component of connective tissues that make up tendons, ligaments, skin and cartilage. (nutraingredients.com)
Less collagen2
- Rejuvenation: Having less collagen in your body leads to weaker bones, fragile nails, brittle hair and dry skin. (spicercastle.com)
- Histological examination revealed marked decrease in granuloma formation and less collagen deposition in the lungs of OPN-KO mice compared to WT. (cdc.gov)
Beauty Collagen1
- POLA BA The Liquid Beauty Collagen Drink is a beauty supplement from POLA's top-of-the-line brand "B.A" (Bio Active). (takaski.com)
Bovine collagen3
- Over the years, the most commonly used raw materials have been bovine collagen. (labosal.com)
- Marine Collagen or Bovine Collagen - what to choose? (labosal.com)
- Although bovine collagen is characterized by high stability and density, its large molecules and chaotic chemical structure mean that it does not show sufficient biological activity. (labosal.com)
Natural collagen2
- All-Natural: Taking a natural collagen supplement helps your body generate fresh, healthy skin. (spicercastle.com)
- The natural collagen in your skin is less likely to break down when you use this night serum, giving you skin that looks younger and plumper, as per PotentLift Night Serum Reviews. (brandsreviews.com)
Biotin1
- The best solution is to use a whole line of products with biotin and collagen. (isportsweb.com)
Skin30
- In particular, type VII collagen plays an essential role in strengthening and stabilizing the skin. (medlineplus.gov)
- Collagen regeneration stimulates the body to renew tissues and maintain a radiant skin complexion. (spicercastle.com)
- Increased levels of type VII collagen in the skin have been reported in patients wi. (nordicbioscience.com)
- A total of 139 patients were treated, of whom 89 (64%) had Fitzpatrick Skin Types IV to VI. (jcadonline.com)
- The treatment of acne scarring is dependent on Fitzpatrick Skin Type and the type of scarring. (jcadonline.com)
- Fitzpatrick Skin Types IV to VI are at higher risk of postinflammatory hyperpigmentation (PIH), especially from treatment modalities characterized by significant heat deposition onto the surface of the skin, such as ablative lasers. (jcadonline.com)
- When the skin around the rolling scars is stretched, this type of scarring tends to fade or flatten out. (jcadonline.com)
- Various antiaging approaches have been developed with the stated aim of achieving young and healthy skin [ 7 ]. (medsci.org)
- A microfiber sheet mask infused with collagen and egg extracts to firm and plump the appearance of skin. (mtlblog.com)
- All skin types, particularly mature and aging skin, may use it, based on PotentLift Night Serum Reviews. (brandsreviews.com)
- A tool tipped with small needles will then be utilized over the skin to produce micro-punctures, which will activate the body to produce more collagen and elastin. (prscd.com)
- Fraxel works by producing micro-injuries in the skin that stimulate the development of collagen and elastin. (prscd.com)
- Vitamin C is a crucial nutrient for maintaining proper collagen production, and collagen is vital to the health and texture of your skin. (iwilife.com)
- Collagen also helps support hydrated skin - and if you are lacking in vitamin C, you may experience dry skin. (iwilife.com)
- The flexible material that they are made from makes them comfortable to wear and suitable for even the most sensitive of skin types. (hotreg.hu)
- Silky yet deeply hydrating for all skin types, this collagen-promoting moisturizer reveals a more youthful-looking complexion thanks to the magic of Adipofill'in™, a wrinkle-filler to help plump and lift lines and creases. (milamoursi.com)
- Argireline reduces wrinkles associated with repeated facial contractions, and Matrixyl 3000 encourages collagen synthesis for firmer, smoother, and more supple-looking skin. (milamoursi.com)
- u003c\/p\u003e\n\u003ch5\u003eIngredients\u003c\/h5\u003e\n\u003cp\u003e\u003cstrong\u003eKey Ingredients\u003c\/strong\u003e\u003c\/p\u003e\n\u003cp\u003e\u003cstrong\u003eMM-5 Complex:\u003c\/strong\u003e Transforms skin with a powerful multi-peptide complex that works in synergy with amino acids, plant-derived stem cells, collagen and elastin. (milamoursi.com)
- u003c\/p\u003e\n\u003cp\u003e\u003cstrong\u003eArgireline:\u003c\/strong\u003e Reduces the appearance of wrinkles caused by facial contractions\u003c\/p\u003e\n\u003cp\u003e\u003cstrong\u003eMatrixyl 3000:\u003c\/strong\u003e Encourages collagen synthesis for firmer, smoother, and more supple-looking skin. (milamoursi.com)
- In one study of 30 women over the age of 45, topical application of the gel was shown to increase collagen production and improve skin elasticity over a 90-day period. (davidwolfe.com)
- In addition, an improper diet, especially one rich in sugar, can accelerate the ageing process of the skin and lead to changes in the structure of collagen [1]. (labosal.com)
- The lack of collagen protein in the skin contributes to i.a. to the deterioration of its elasticity, problems with proper hydration and an increased tendency to dry out or a decrease in its firmness. (labosal.com)
- Collagen supplementation may prove to be one of the ways to maintain optimal skin conditions. (labosal.com)
- Fish collagen is perceived as a better biocomponent for human skin than that obtained from pork or beef skin. (labosal.com)
- After 90 days of treatment, it turned out that the use of fish collagen improved the antioxidant effect, and also led to an increase in skin thickness and the number of fibroblasts. (labosal.com)
- As a result, marine collagen was found to have protective properties against simulated skin aging. (labosal.com)
- Our anti-aging vitamin C serum delivers unparalleled results combating and reversing the signs of aging by increasing collagen protection, rejuvenating the skin and provides antioxidant protection against environmental and UV induced skin damage. (hebeskinhealth.com)
- This treatment is ideal for all skin types and perfect for anyone who wants to reduce mild to severe signs of aging. (bigappleskin.com)
- Evoke will force your body to manufacture new collagen to tighten your skin. (bigappleskin.com)
- With a powerful 1064 YAG laser, the Laserscope Lyra can quickly and painlessly remove hair from all kinds of skin types. (rockbottomlasers.com)
Elasticity1
- Unlike Botox, dermal fillers can be used to smoothen out static wrinkles caused by the loss of collagen, elasticity, and fat, as well as gravity and sun damage. (prscd.com)
Triple helix2
- In its natural form, collagen has a folded triple helix structure consisting of long polypeptide chains (see figure 1). (nutraingredients.com)
- It cleaves at multiple cleavage sites within the collagen triple helix. (sigmaaldrich.com)
Amino Acids2
- 7 More than 100 "active" compounds, including flavonoids, saponins, polysaccharides, and amino acids, have been identified in Astragalus thus far. (ndnr.com)
- Gut Friendly Protein: Pure Bone Broth Protein with gut friendly Collagen, Amino acids. (getyokd.com)
Compounds1
- These two compounds are powerful carotenoids - types of pigments that are found in plants. (iwilife.com)
Stimulate1
- Similarly, focal application of chemical peel products, such as carbolic acid or trichloroacetic acid using the CROSS technique, can stimulate collagen remodeling to release tethered icepick or depressed scars. (jcadonline.com)
20162
- V skúške normy EN 374-1:2016 (Ochrana proti nebezpečným chemikáliám a mikroorganizmom) získali jednorazové rukavice nitrylex® collagen výsledok KPT . (ganes.eu)
- Scientific paper from 2016 [5] to check the anti-aging effectiveness of fish collagen with the addition of antioxidants in a group of 41 volunteers. (labosal.com)
20172
- 2017 Dec 1;7(1):7002. (utmb.edu)
- In Japan, the allograft collagen nerve for peripheral nerves reconstruction was permitted in 2017, and we tried to use this allograft nerve and got a recommendable result. (springeropen.com)
20021
- enzymes that degrade the collagen, such as the Very few studies have investigated the rela- metallo-proteinases (Jenkins, 2002). (who.int)
Supplements3
- Discover the benefits of native type II collagen for convenient and low-dose supplements. (nutraingredients.com)
- You can usually find collagen supplements in most health food stores, typically made from animal products. (iwilife.com)
- It might seem that the selection of preparation with collagen is not a problem, especially in times of ubiquitous access to dietary supplements. (labosal.com)
Nails1
- Healthy Hair & Nails: Multi collagen is an incredible hair and nails supplement. (spicercastle.com)
Stimulates1
- The first involves application of the retinoid vitamin A, which increases the amount of type I collagen, stimulates elastic fiber organization and glycosaminoglycan synthesis, and decreases the amount of metalloproteinases [ 7 , 8 ]. (medsci.org)
Wrinkles1
- Botox will not have much of an impact on these types of wrinkles. (prscd.com)
Production2
- Since the body produces collagen on its own, taking vitamin C and other nutrients like zinc and copper can help to support collagen production. (iwilife.com)
- What Foods Help in Collagen Production? (apnewsday.com)
Structural4
- Collagen is a complex structural protein that maintains strength and flexibility throughout the body. (onefamshop.com)
- Although it has many important functions in the body, collagen is best known for its structural role - providing a framework for tissues throughout the body. (nutraingredients.com)
- [1] Of the 28 different types of collagen that have been identified, type II collagen is the main structural protein in cartilage. (nutraingredients.com)
- Moreover, there are quantitative and structural changes in collagen, elastic fibers, glycosaminoglycans, and proteoglycans of the extracellular matrix [ 2 , 3 ]. (medsci.org)
Basement membrane1
- A non-fibrillar collagen involved in anchoring the epidermal BASEMENT MEMBRANE to underlying tissue. (bvsalud.org)
Molecules2
- Then the molecules arrange themselves into long, thin bundles of mature type VII collagen. (medlineplus.gov)
- Matrix metalloproteinase 7 (MMP 7) (Matrilysin, PUMP-1) is known to cleave the major matrix molecules found within the IVD, i.e., the proteoglycan aggrecan and collagen type II. (shu.ac.uk)
Body2
- Take care of your body and restore your youth with our Multi Collagen pills. (spicercastle.com)
- Because collagen is everywhere in the body, there are hundreds of ways EDS can affect people. (thezebramom.com)
Amounts2
Sodium1
- The empirical formula of alendronate sodium is C 4 H 12 NNaO 7 P 2 ·3H 2 O and its formula weight is 325.12. (rxdrugnews.com)
Regeneration1
- Embryonic stem cells (ESCs) have emerged as potential cell sources for tissue engineering and regeneration owing to its virtually unlimited replicative capacity and the potential to differentiate into a variety of cell types. (cdc.gov)
Tissue4
- Dysbalance of fibrosis and fibrolysis in asthmatic lung tissue leads to reduced levels of the inflammation-protective collagen 4 (COL4A3). (nordicbioscience.com)
- The collagenase type I (from Clostridium histolyticum) is a crude collagenase preparation that can be used for the isolation of primary cells or for tissue dissociation by enzymatic means. (sigmaaldrich.com)
- High resolution proton NMR spectroscopy was employed for the analysis of lipid components in the lipid extracts of the joint tissue and plasma of collagen-induced arthritic and control rats. (who.int)
- The results demonstrated significantly altered lipid patterns in the joint tissue and plasma of collagen-induced arthritic rats as detected by one- and two-dimensional NMR spectroscopy compared with controls. (who.int)
Supplement2
- Widely used in beauty and cosmetics, as well as food and beverage applications, collagen is now making big moves in the joint health market, creating exciting opportunities for dietary supplement manufacturers to innovate in this sector. (nutraingredients.com)
- The development of science has meant that marine collagen is mentioned more and more often, which has been used, among others, in the dietary supplement Labosal Beauty Shot . (labosal.com)
Extracellular matrix1
- the binding of TGF-{beta}1 to its receptor triggers a signaling cascade that results in inflammatory signaling, accumulation of collagen and other components of the extracellular matrix, and immune system activation. (bvsalud.org)
Abnormal2
- Some research has suggested that abnormal forms of type VII collagen that retain a procollagen fragment called the NC1 domain may increase the risk of tumor formation. (medlineplus.gov)
- IMSEAR at SEARO: Abnormal lipid metabolism in collagen-induced arthritis rat model: In vitro, high resolution NMR spectroscopy based analysis. (who.int)
Pills3
- Do not worry, we have been keeping up with the latest trends and have compiled a list of the best Collagen Pills For Mens for you. (spicercastle.com)
- Researching about the quality, price, and features of these Collagen Pills For Mens is important, so that you can make an informed decision. (spicercastle.com)
- Here are the top Collagen Pills For Men we picked, check their features. (spicercastle.com)
Dermis1
- informative epidemiological studies of the role of Similarly there appears to be a reduced amount indoor tanning facilities in the induction of photo- of collagen I in the dermis and increased ageing. (who.int)
Inflammation1
- The condition is associated with injury to dermal collagen, granulomatous inflammation, and ulceration. (medscape.com)
Chromosome1
- Staphylococcal cassette chromosome mec type V sequences in our isolates were more closely related to each other than to the closest reference sequence (GenBank accession no. (cdc.gov)