Latent-transforming growth factor beta-binding protein 2 is a protein that in humans is encoded by the LTBP2 gene.[1][2] The protein encoded by this gene belongs to the family of latent transforming growth factor (TGF)-beta binding proteins (LTBP), which are extracellular matrix proteins with multi-domain structure. This protein is the largest member of the LTBP family possessing unique regions and with most similarity to the fibrillins. It has thus been suggested that it may have multiple functions: as a member of the TGF-beta latent complex, as a structural component of microfibrils, and a role in cell adhesion.[2] ...
This gene encodes a secreted ligand of the TGF-beta (transforming growth factor-beta) superfamily of proteins. Ligands of this family bind various TGF-beta receptors leading to recruitment and activation of SMAD family transcription factors that regulate gene expression. The encoded preproprotein is proteolytically processed to generate a latency-associated peptide (LAP) and a mature peptide, and is found in either a latent form composed of a mature peptide homodimer, a LAP homodimer, and a latent TGF-beta binding protein, or in an active form consisting solely of the mature peptide homodimer. The mature peptide may also form heterodimers with other TGF-beta family members. This protein is involved in embryogenesis and cell differentiation, and may play a role in wound healing. Mutations in this gene are a cause of aortic aneurysms and dissections, as well as familial arrhythmogenic right ventricular dysplasia 1. [provided by RefSeq, Aug 2016 ...
0005] One embodiment of the invention provides a method of detecting cancer or a predisposition to developing cancer in a subject. The method comprises determining an expression level of a cancer-associated polynucleotide, protein, or polypeptide selected from the group consisting of Titin; HBA1; Insulin-like growth factor 1 receptor (IGF1R); Isoform 3 of zonadhesin precursor; latent transforming growth factor beta binding protein 4 (LTBP4); ASXL1 (additional sex combs like 1); beta globin (HBB); BMP15-bone morphogenetic protein; TRIM49; DNAJ homolog subfamily B member 11 precursor; uncharacterized hematopoietic stem/progenitor cells protein MDS027; uncharacterized protein ALB; isoform 3 of sushi, nidogen and EGF-like domain-containing protein 1 precursor; isoform 2 of peripherin; mitochondrial 28S ribosomal protein S22; translation initiation factor EIF-2B subunit epsilon; estradiol 17-beta-dehydrogenase 1; XRCC6BP1; brain-specific angiogenesis inhibitor 1 precursor; isoform 2 of ring finger ...
Established from an adenocarcinoma from a 44-year-old female. Tumorigenic in nude mice and has ability to grow in agar. They have human adrenergic alpha2A urokinase receptor (u-PAR), vitamin D (moderate expression) and urokinase receptor (u-PAR); The cell line can produce secretory component of IgA, carcinoembryonic antigen (CEA), transforming growth factor beta binding protein and mucin. Ultrastructural features include microvilli, microfilaments, large vacuolated mitochondria with dark granules, smooth and rough ER with free ribosomes, lipid droplets, few primary and many secondary lysosomes. There is a G -> A mutation in codon 273 of the p53 gene resulting in an Arg -> His substitution ...
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LTBPs have generally been considered proteins that target the effects of TGF-β by augmenting its secretion from the cells and targeting the latent complexes to appropriate sites in the ECM (Saharinen et al., 1999). So far relatively few other functions have been identified for LTBPs.. Fibrillins and other microfibril components, MAGP-2 (Gibson et al., 1999; Bax et al., 2003), mediate cell adhesion via RGD sequences. Fibrillin-1 seems to mediate cell adhesion also via non-RGD-dependent ways (Sakamoto et al., 1996). LTBP-2 possesses a putative cell adhesion-mediating RGD sequence, which led us to examine the effects of LTBP-2, and especially its NH2 terminus, on cell adhesion.. Although full-length LTBP-2 did not mediate cell adhesion, we found with Ig-tagged fragments an area that supported fibroblast binding in cell adhesion assays. This activity was localized to the NH2 terminus of LTBP-2 at the variable region toward the COOH terminus from the second EGF-like repeat and toward the NH2 ...
Transforming growth factor-beta (TGF-beta) is a potent growth regulatory protein secreted by virtually all cells in a latent form. A major mechanism of regulating TGF-beta activity occurs through factors that control the processing of the latent to the biologically active form of the molecule. We have shown previously that thrombospondin 1 (TSP1), a platelet alpha-granule and extracellular matrix protein, activates latent TGF-beta via a protease- and cell-independent mechanism and have localized the TGF-beta binding/activation region to the type 1 repeats of platelet TSP1. We now report that recombinant human TSP1, but not recombinant mouse TSP2, activates latent TGF-beta. Activation was further localized to the unique sequence RFK found between the first and the second type 1 repeats of TSP1 (amino acids 412-415) by the use of synthetic peptides. A peptide with the corresponding sequence in TSP2, RIR, was inactive. In addition, a hexapeptide GGWSHW, based on a sequence present in the type 1 ...
Transforming growth factor-beta (TGF-beta) is a potent growth regulatory protein secreted by virtually all cells in a latent form. A major mechanism of regulating TGF-beta activity occurs through factors that control the processing of the latent to the biologically active form of the molecule. We have shown previously that thrombospondin 1 (TSP1), a platelet alpha-granule and extracellular matrix protein, activates latent TGF-beta via a protease- and cell-independent mechanism and have localized the TGF-beta binding/activation region to the type 1 repeats of platelet TSP1. We now report that recombinant human TSP1, but not recombinant mouse TSP2, activates latent TGF-beta. Activation was further localized to the unique sequence RFK found between the first and the second type 1 repeats of TSP1 (amino acids 412-415) by the use of synthetic peptides. A peptide with the corresponding sequence in TSP2, RIR, was inactive. In addition, a hexapeptide GGWSHW, based on a sequence present in the type 1 ...
Clone CH6-17E5.1 reacts with the N-terminal latency-associated peptide (LAP) of the transforming growth factor β1 (TGF-β1) dimer. TGF-β1 belongs to a family of homologous, disulfide-linked, homodimeric proteins. These highly pleiotropic cytokines inhibit proliferation of most cells, but can promote the growth of mesenchymal cells and enhance extracellular matrix formation. The pivotal function of TGF-β1 in the immune system is to mediate immunosuppression and maintain tolerance by regulating lymphocyte proliferation, differentiation, and survival. TGF-β1 is produced by many cell types, but is reported to be most abundant in mammalian platelets and bone. It is secreted predominantly as an inactive latent complex. After proteolytical processing of the TGF-β1 precursor, the resulting N-terminal latency-associated peptide (LAP) remains non-covalently associated with the TGF-β1 dimer. Mature and biologically active TGF-β1 can be released from the complex by action of proteases and/or conformational
The research profile of our laboratory include the biology of TGF-beta, especially its role in the regulation of cell proliferation and extracellular proteolysis and the roles of netrins on glioblastoma growth and invasion. We analyze the biology of latent forms of TGF-ß family growth factors, their association with the extracellular matrix structures by LTBPs (latent TGF-ß binding proteins) and proteolytic release and activation of the latent complexes. A link to the regulation of extracellular proteolysis is via plasminogen activators and metalloproteinases. We have analyzed plasmin mediated processing/activation of gelatinase A, and identified membrane type-1 MMP (MT1-MMP) as a major effector in the cell surface associated activation of gelatinase A. Glioblastoma is a highly aggressive and lethal cancer type. It is characterized by extensive angiogenesis in tumor tissue, and lethality occurs by infiltration/invasion of tumor cells to brain tissue. The focus of our research is to reveal ...
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As long-time collaborators, Deryugina and Quigley have led research that shows that the initial steps of tumor metastasis can occur when a primary tumor is barely detectable. This work sparked their interest in the role of LTBP3, short for Latent TGFβ Binding Protein 3. They knew that LTBP3 partners with TGFβ to regulate its secretion, activation and maturation, but wondered what else LTBP3 might control.. Could LTBP3 set TGFβ on its harmful path of action in early-stage tumors, and might LTBP3 have its own role, independent of TGFβ, in cancer metastasis?. Researchers used chick embryo tumor models and a rodent model of head and neck cancer to discover how LTBP3 is involved in the spread of aggressive tumor cells. They knocked down LTBP3 expression and secretion in human tumor cell lines representing carcinoma, head and neck carcinoma and a fibrosarcoma. In each model, the team found that without LTBP3, primary tumor cells could not metastasize efficiently.. Our experimental findings showed ...
Structure of betaglycan zona pellucida (ZP)-C domain provides insights into ZP-mediated protein polymerization and TGF-beta binding.. Proc Natl Acad Sci U S A. 2011 Mar 29;108(13):5232-6. Authors: Lin SJ, Hu Y, Zhu J, Woodruff TK, Jardetzky TS ...
Perform reliable PCR with Bio-Rads LTBP3 primer pair, for Human. Designed for EvaGreen-based detection with digital PCR (ddPCR).
Transforming growth factor-beta receptor III (TGF-beta RIII) is one of three receptors for the secreted growth factor TGF-beta. Unlike type I and type II TGF-beta receptors, TGF-beta RIII does not participate directly in the propagation of intracellular signaling in response to TGF-beta binding.
MalaCards based summary : Cyp1b1-Related Primary Congenital Glaucoma is related to primary congenital glaucoma. An important gene associated with Cyp1b1-Related Primary Congenital Glaucoma is CYP1B1 (Cytochrome P450 Family 1 Subfamily B Member 1 ...
Results The recruitment of the polymorphonuclear cells induced by MSU injection into mouse peritoneal cavity was reduced by 35% with γ3MSH (1 nmol), whereas administration of a much lower dose of purified latent LAP-MMP-γ3MSH (0.03 nmol) attenuated leucocyte influx by 50%. Intramuscular gene delivery of plasmids coding LAP-MMP-VIP and LAP-MMP-αMSH at disease onset reduced the development of CIA compared with LAP-MMP, which does not contain any therapeutic moiety. Histological analysis confirmed a significantly lower degree of inflammation, bone and cartilage erosion in groups treated with LAP-MMP-VIP or LAP-MMP-αMSH. Antibody titres to collagen type II and inflammatory cytokine production were also reduced in these two groups.. ...
The integrin αvβ6 is upregulated in numerous carcinomas, where expression commonly correlates with poor prognosis. αvβ6 promotes tumour invasion, partly through regulation of proteases and cell migration, and is also the principal mechanism by which epithelial cells activate TGF-β1; this latter function complicates therapeutic targeting of αvβ6, since TGF-β1 has both tumour-promoting and -suppressive effects. It is unclear how these different αvβ6 functions are linked; both require actin cytoskeletal reorganisation, and it is suggested that tractive forces generated during cell migration activate TGF-β1 by exerting mechanical tension on the ECM-bound latent complex. We examined the functional relationship between cell invasion and TGF-β1 activation in pancreatic ductal adenocarcinoma (PDAC) cells, and confirmed that both processes are αvβ6-dependent. Surprisingly, we found that cellular functions could be biased towards either motility or TGF-β1 activation depending on the ...
Transforming Growth Factor beta3: A TGF-beta subtype that plays role in regulating epithelial-mesenchymal interaction during embryonic development. It is synthesized as a precursor molecule that is cleaved to form mature TGF-beta3 and TGF-beta3 latency-associated peptide. The association of the cleavage products results in the formation a latent protein which must be activated to bind its receptor.
Perform reliable qPCR with Bio-Rads pre-validated LTBP3 primer pair, for the Cow genome. Designed for SYBR Green-based detection.
A TGF-beta subtype that was originally identified as a GLIOBLASTOMA-derived factor which inhibits the antigen-dependent growth of both helper and CYTOTOXIC T LYMPHOCYTES. It is synthesized as a precursor molecule that is cleaved to form mature TGF-beta2 and TGF-beta2 latency-associated peptide. The association of the cleavage products results in the formation a latent protein which must be activated to bind its receptor ...
SCA124Hu, CLIA Kit for Transforming Growth Factor Beta 1 (TGFb1), 转化生长因子β1(TGFb1)检测试剂盒(化学发光免疫分析法), TGF-B1; CED; DPD1; LAP; Camurati-Engelmann Disease; Latency-associated peptide | 仅供体外研究使用,不用于临床诊断!请索取进口关税税单及报关单!
Autosomal recessive cutis laxa type I (ARCL type I) is characterized by generalized cutis laxa with pulmonary emphysema and/or vascular complications. Rarely, mutations can be identified in FBLN4 or FBLN5. Recently, LTBP4 mutations have been implicated in a similar phenotype. Studying FBLN4, FBLN5, and LTBP4 in 12 families with ARCL type I, we found bi-allelic FBLN5 mutations in two probands, whereas nine probands harbored biallelic mutations in LTBP4. FBLN5 and LTBP4 mutations cause a very similar phenotype associated with severe pulmonary emphysema, in the absence of vascular tortuosity or aneurysms. Gastrointestinal and genitourinary tract involvement seems to be more severe in patients with LTBP4 mutations. Functional studies showed that most premature termination mutations in LTBP4 result in severely reduced mRNA and protein levels. This correlated with increased transforming growth factor-beta (TGFβ) activity. However, one mutation, c.4127dupC, escaped nonsense-mediated decay. The ...
Primary congenital glaucoma is characterized by photophobia, epiphora, and blepharospasm. Read about hereditary, causes, symptoms, treatment, risk factors, and prognosis.
View more ,Fibrillins 1-3 are stromal extracellular matrix proteins that play important roles in regulating TGFβ activity, which stimulates fibroblasts to proliferate and synthesize collagen. In the developing ovary, the action of stroma is initially necessary for the formation of ovigerous cords and subsequently for the formation of follicles and the surface epithelium of the ovary. FBN3 is highly expressed only in early ovarian development and then it declines. In contrast, FBN1 and 2 are upregulated in later ovarian development. We examined the expression of FBN1-3 in bovine and human fetal ovaries. We used cell dispersion and monolayer culture, cell passaging and tissue culture. Cells were treated with growth factors, hormones or inhibitors to assess the regulation of expression of FBN1-3. When bovine fetal ovarian tissue was cultured, FBN3 expression declined significantly. Treatment with TGFβ-1 increased FBN1 and FBN2 expression in bovine fibroblasts, but did not affect FBN3 expression. ...
Hu et al. (13) report that in hyperglycemic NOD mice, combining intravenous CD20 antibody with oral CD3 antibody treatment afforded remission of T1D in 66% of mice, lasting in most animals for 1 month, whereas single treatments were poorly effective. Interestingly, the effect was far more obvious in overtly diabetic compared with prediabetic mice, in which CD20 antibody alone already showed a significant effect. The proposed mechanistic cornerstone sustaining the therapeutic benefit is a boost in "immune regulation" through, first, an improvement in the suppressive capacity of CD4+FoxP3+ regulatory T cells (Tregs) and, second, the local induction in the intestine of CD4+FoxP3− interleukin (IL)-10-producing Tregs closely resembling Tr1 cells (13). Interestingly, a similar local induction of Tregs was observed in the models described by Weiner and colleagues (20-22), which, however, were transforming growth factor-β (TGF-β)-dependent, stained positive for the latency-associated peptide ...
During the past decade, the role of Treg cells in a variety of tolerance models has been explored. Many different markers, including latency-associated peptide (LAP) (78), glucocorticoid-induced TNFR (GITR) (79), CTLA-4 (66, 67), CD25 (80, 81, 82), CD45RBlow (83), and Foxp3 (84, 85, 86), have been used to describe Tregs. However, most investigators now recognize two major Treg subsets: a natural population that suppresses through cell-to-cell contact, and an induced population that secretes suppressive cytokines. The two markers that most clearly define natural Tregs are CD25 (80, 81, 82) and the forkhead transcription factor Foxp3 (84, 85, 86). Importantly, natural Tregs are anergic and can suppress T cell responses in vitro in an Ag-nonspecific manner (80, 82, 87, 88). The absence of natural Tregs (either CD25+ or Foxp3+) in both mice and humans results in severe autoimmune disease, emphasizing their vital role in the prevention of self-reactivity (84, 85, 86). Substantial evidence indicates ...
Transforming growth factor-beta (TGF-beta) is a potent growth regulatory protein secreted by virtually all cells in a latent form. A major mechanism of regulating TGF-beta activity occurs through factors that control the processing of the latent to t
Affiliation (Current):東京医療学院大学,保健医療学部,客員教授, Research Field:Respiratory organ internal medicine,Respiratory organ internal medicine,Medical sociology,Medical and hospital managemen, Keywords:慢性閉塞性肺疾患,肺気腫,COPD,Collagen,遺伝子多型,LTBP4,Morphometry,肺の成長・発育,幼若ラット,Lung development, # of Research Projects:13, # of Research Products:6, Ongoing Project:COPDと心血管疾患の臓器相関の新機序解明に向けたガレクチン-3の役割の探索
Looking for online definition of congenital glaucoma in the Medical Dictionary? congenital glaucoma explanation free. What is congenital glaucoma? Meaning of congenital glaucoma medical term. What does congenital glaucoma mean?
BACKGROUND Primary congenital glaucoma generally presents with a classic clinical triad of photophobia, blepharospasm, and epiphora caused by the corneal changes that occur secondary to increased intraocular pressure (IOP). The condition typically presents bilaterally and is rarely hereditary. Onset is from age 2 months to 2 to 3 years. CASE REPORT A 2-year, 5-month-old Hispanic boy presented with an enlarged right eye and an intermittent right exotropia, without tearing or photophobia. Examination also found high myopia and an optic nerve cup-to-disc ratio larger in the right than the left eye. Referral to a pediatric ophthalmologist was initiated. On the first examination under anesthesia (EUA), the child was diagnosed with unilateral megalocornea with a normal IOP. He did not have any other typical signs and symptoms of primary congenital glaucoma. An EUA 8 months later led to a diagnosis of primary congenital glaucoma based on the new appearance of Haabs striae, further enlargement of the cornea,
Castration experiments in rodents show that the stromal vasculature is critical to the androgen-mediated prostate growth regulation. However, the role of angiogenesis inhibitors, such as thrombospondin-1 (TSP-1), in this process is unclear. TSP-1 is a multifunctional glycoprotein that can function as a potent angiogenesis inhibitor and an in vivo activator of latent transforming growth factor-beta (TGF-beta) in some tissues. On the basis of these observations, we hypothesized that TSP-1 regulated androgen withdrawal-induced prostate regression and that this process was mediated not only through antiangiogenic activity but also through TGF-beta activation. To test this, we evaluated angiogenic activity in human prostate epithelial and stromal cells treated with androgens and hypoxia in vitro. TSP-1 knockout mice were characterized to investigate the in vivo functions of TSP-1. In vitro, we found that androgens and hypoxia differentially regulated TSP-1 and angiogenic activity. Androgens ...
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