抗体

小鼠抗人类TGFR-1(ALK-5)

MBS690076

0.1毫克

475美元

单克隆

小鼠

未共轭

(7B09)

免疫印迹, 免疫组化

抗体

小鼠抗人类TGFR-1(ALK-5)

TGFR-1(ALK-5)

transforming growth factor; beta receptor 1; AAT5; ALK5; MSSE; SKR4; ALK-5; LDS1A; LDS2A; TGFR-1; ACVRLK4;TGF-beta receptor type-1 tbetaR-I; TGF-beta receptor type I; TGF-beta type I receptor; activin receptor-like kinase 5; transforming growth factor beta receptor I; serine/threonine-protein kinase receptor R4; activin A receptor type II-like kinase; 53kD; activin A receptor type II-like kinase; 53 kDa; transforming growth factor-beta receptor type I; activin A receptor type II-like protein kinase of 53kD; transforming growth factor; beta receptor I (activin A receptor type II-like kinase; 53kD)

TGF-beta receptor type-1 isoform 2; TGF-beta receptor type-1; TGF-beta receptor type-1; tbetaR-I; TGF-beta receptor type I; TGF-beta type I receptor; activin receptor-like kinase 5; multiple self-healing squamous epithelioma; transforming growth factor beta receptor I; serine/threonine-protein kinase receptor R4; activin A receptor type II-like kinase, 53kD; activin A receptor type II-like kinase, 53kDa; transforming growth factor-beta receptor type I; activin A receptor type II-like protein kinase of 53kD; transforming growth factor, beta receptor I (activin A receptor type II-like kinase, 53kD); transforming growth factor, beta receptor 1; Activin A receptor type II-like protein kinase of 53kD; Activin receptor-like kinase 5; ALK-5; ALK5; Serine/threonine-protein kinase receptor R4; SKR4; TGF-beta type I receptor; Transforming growth factor-beta receptor type I

TGFR-1(ALK-5)

TGFBR1; TGFBR1; AAT5; ALK5; ESS1; MSSE; SKR4; ALK-5; LDS1A; LDS2A; TGFR-1; ACVRLK4; ALK5; SKR4; TGFR-1; ALK-5; ALK5; SKR4; TGF-beta receptor type I; TbetaR-I

TGFR1_HUMAN

单克隆

IgG2

(7B09)

小鼠

人类

426

冻干

Lyophilized samples are stable for 2 years from date of receipt when stored at -70 degree C. Reconstituted antibody can be aliquoted and stored frozen at < -20 degree C for at least for six months without detectable loss of activity. Shipping: Ships with cold packs

免疫印迹(免疫印迹), 免疫组化(免疫组化)

WB: 1:200-800。 IHC: 1:50-200

Antibody Generation: This antibody was produced from a hybridoma (mouse myeloma fused with spleen cells from a mouse) immunized with purified human extracellular domain of ALK5. The IgG2 fraction of the culture supernatant was purified by Protein G affinity chromatography. Antigen: Human TGF R1 (ALK5) EC domain

Reconstitution: Reconstitute the antibody with 200 ul sterile PBS and the final concentration is 500 ug/ml. Reconstitution Buffer: PBS (Sterile)

Most cell types express three sizes of receptors for TGF-beta. These are designated Type I (53 kDa), Type II (70 - 85 kDa), and Type III (250 - 350 kDa). The Type I receptor (Alk-5) is a membrane-bound serine/threonine kinase that apparently requires the presence of the Type II receptor to bind TGF-beta. The Type II receptor is also a membrane-bound serine/threonine kinase that binds TGF-beta1 and TGF-beta 3 with high affinity and TGF-beta 2 with much lower affinity. The Type I and Type II receptors together form a heterodimeric signaling complex that is essential for the transduction of the anti-proliferative signals of TGF-beta. The Type III receptor is a transmembrane proteoglycan with a large extracellular domain and a 43 amino acid residue cytoplasmic domain. The cytoplasmic domain of the Type III receptor lacks an obvious signaling motif and the receptor may not be involved directly in signal transduction.

195963412

NP_001124388.1

NM_001130916.1

P36897

55,960 Da

ALK1 Signaling Events Pathway (137968); Adherens Junction Pathway (83070); Adherens Junction Pathway (481); Chagas Disease (American Trypanosomiasis) Pathway (147809); Chagas Disease (American Trypanosomiasis) Pathway (147795); Chronic Myeloid Leukemia Pathway (83116); Chronic Myeloid Leukemia Pathway (528); Colorectal Cancer Pathway (83106); Colorectal Cancer Pathway (518); Cytokine-cytokine Receptor Interaction Pathway (83051)

The protein encoded by this gene forms a heteromeric complex with type II TGF-beta receptors when bound to TGF-beta, transducing the TGF-beta signal from the cell surface to the cytoplasm. The encoded protein is a serine/threonine protein kinase. Mutations in this gene have been associated with Loeys-Dietz aortic aneurysm syndrome (LDAS). Multiple transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Aug 2008]

Function: Transmembrane serine/threonine kinase forming with the TGF-beta type II serine/threonine kinase receptor, TGFBR2, the non-promiscuous receptor for the TGF-beta cytokines TGFB1, TGFB2 and TGFB3. Transduces the TGFB1, TGFB2 and TGFB3 signal from the cell surface to the cytoplasm and is thus regulating a plethora of physiological and pathological processes including cell cycle arrest in epithelial and hematopoietic cells, control of mesenchymal cell proliferation and differentiation, wound healing, extracellular matrix production, immunosuppression and carcinogenesis. The formation of the receptor complex composed of 2 TGFBR1 and 2 TGFBR2 molecules symmetrically bound to the cytokine dimer results in the phosphorylation and the activation of TGFBR1 by the constitutively active TGFBR2. Activated TGFBR1 phosphorylates SMAD2 which dissociates from the receptor and interacts with SMAD4. The SMAD2-SMAD4 complex is subsequently translocated to the nucleus where it modulates the transcription of the TGF-beta-regulated genes. This constitutes the canonical SMAD-dependent TGF-beta signaling cascade. Also involved in non-canonical, SMAD-independent TGF-beta signaling pathways. For instance, TGFBR1 induces TRAF6 autoubiquitination which in turn results in MAP3K7 ubiquitination and activation to trigger apoptosis. Also regulates epithelial to mesenchymal transition through a SMAD-independent signaling pathway through PARD6A phosphorylation and activation. Ref.10 Ref.11 Ref.12 Ref.15 Ref.22 Ref.23 Ref.25. Catalytic activity: ATP + [receptor-protein] = ADP + [receptor-protein] phosphate. Cofactor: Magnesium or manganese . By similarity. Enzyme regulation: Kept in an inactive conformation by FKBP1A preventing receptor activation in absence of ligand. CD109 is another inhibitor of the receptor. Ref.13. Subunit structure: Homodimer; in the endoplasmic reticulum but also at the cell membrane. Heterohexamer; TGFB1, TGFB2 and TGFB3 homodimeric ligands assemble a functional receptor composed of two TGFBR1 and TGFBR2 heterodimers to form a ligand-receptor heterohexamer. The respective affinity of TGBRB1 and TGFBR2 for the ligands may modulate the kinetics of assembly of the receptor and may explain the different biological activities of TGFB1, TGFB2 and TGFB3. Interacts with CD109; inhibits TGF-beta receptor activation in keratinocytes. Interacts with RBPMS. Interacts (unphosphorylated) with FKBP1A; prevents TGFBR1 phosphorylation by TGFBR2 and stabilizes it in the inactive conformation. Interacts with SMAD2, SMAD3 and ZFYVE9; ZFYVE9 recruits SMAD2 and SMAD3 to the TGF-beta receptor. Interacts with TRAF6 and MAP3K7; induces MAP3K7 activation by TRAF6. Interacts with PARD6A; involved in TGF-beta induced epithelial to mesenchymal transition. Interacts with SMAD7, NEDD4L, SMURF1 and SMURF2; SMAD7 recruits NEDD4L, SMURF1 and SMURF2 to the TGF-beta receptor. Interacts with USP15 and VPS39. Ref.10 Ref.12 Ref.13 Ref.14 Ref.15 Ref.16 Ref.17 Ref.18 Ref.19 Ref.20 Ref.21 Ref.22 Ref.23 Ref.24 Ref.25 Ref.31. Subcellular location: Cell membrane; Single-pass type I membrane protein. Cell junction tight junction Ref.10 Ref.17 Ref.22. Tissue specificity: Found in all tissues examined, most abundant in placenta and least abundant in brain and heart. Post-translational modification: Phosphorylated at basal levels in the absence of ligand. Activated upon phosphorylation by TGFBR2, mainly in the GS domain. Phosphorylation in the GS domain abrogates FKBP1A-binding. Ref.10 Ref.31N-Glycosylated. Ref.7Ubiquitinated; undergoes ubiquitination catalyzed by several E3 ubiquitin ligases including SMURF1, SMURF2 and NEDD4L2. Results in the proteasomal and/or lysosomal degradation of the receptor thereby negatively regulating its activity. Deubiquitinated by USP15, leading to stabilization of the protein and enhanced TGF-beta signal. Ref.18 Ref.19 Ref.21 Ref.28. Involvement in disease: Loeys-Dietz syndrome 1A (LDS1A) [MIM:609192]: An aortic aneurysm syndrome with widespread systemic involvement. The disorder is characterized by arterial tortuosity and aneurysms, craniosynostosis, hypertelorism, and bifid uvula or cleft palate. Other findings include exotropy, micrognathia and retrognathia, structural brain abnormalities, intellectual deficit, congenital heart disease, translucent skin, joint hyperlaxity and aneurysm with dissection throughout the arterial tree.Note: The disease is caused by mutations affecting the gene represented in this entry. Ref.38 Ref.40 Ref.41 Ref.45 Ref.46Loeys-Dietz syndrome 2A (LDS2A) [MIM:608967]: An aortic aneurysm syndrome with widespread systemic involvement. Physical findings include diffuse arterial aneurysms and dissections, prominent joint laxity, easy bruising, wide and atrophic scars, velvety and translucent skin with easily visible veins, spontaneous rupture of the spleen or bowel, and catastrophic complications of pregnancy, including rupture of the gravid uterus and the arteries, either during pregnancy or in the immediate postpartum period. Loeys-Dietz syndrome type 2 is characterized by the absence of craniofacial abnormalities with the exception of bifid uvula that can be present in some patients.Note: The disease is caused by mutations affecting the gene represented in this entry. TGFBR1 mutation Gln-487 has been reported to be associated with thoracic aortic aneurysms and dissection (TAAD) (Ref.41). This phenotype, also known as thoracic aortic aneurysms type 5 (AAT5), is distinguised from LDS2A by having aneurysms restricted to thoracic aorta. It is unclear, however, if this condition is fulfilled in individuals bearing Gln-487 mutation, that is why they are considered as LDS2A by the OMIM resource. Ref.41 Ref.42Multiple self-healing squamous epithelioma (MSSE) [MIM:132800]: A disorder characterized by multiple skin tumors that undergo spontaneous regression. Tumors appear most often on sun-exposed regions, are locally invasive, and undergo spontaneous resolution over a period of months leaving pitted scars.Note: The disease is caused by mutations affecting the gene represented in this entry. Ref.47. Sequence similarities: Belongs to the protein kinase superfamily. TKL Ser/Thr protein kinase family. TGFB receptor subfamily.Contains 1 GS domain.Contains 1 protein kinase domain.

0.1毫克

475美元