Rabbit anti-Human LTBP4 Polyclonal Antibody | anti-LTBP4 antibody
LTBP4 Antibody
Constituents: 50% Glycerol, 0.01M PBS, PH 7.4
Immunohistochemistry (IHC)
(Immunohistochemistry of paraffin-embedded human kidney tissue using MBS7044357 at dilution of 1:100)
Immunofluorescence (IF)
(Immunofluorescent analysis of HepG2 cells using MBS7044357 at a dilution of 1:100 and Alexa Fluor 488-congugated AffiniPure Goat Anti-Rabbit IgG(H+L))
NCBI and Uniprot Product Information
NCBI Description
The protein encoded by this gene binds transforming growth factor beta (TGFB) as it is secreted and targeted to the extracellular matrix. TGFB is biologically latent after secretion and insertion into the extracellular matrix, and sheds TGFB and other proteins upon activation. Defects in this gene may be a cause of cutis laxa and severe pulmonary, gastrointestinal, and urinary abnormalities. Three transcript variants encoding different isoforms have been found for this gene.[provided by RefSeq, May 2010]
Uniprot Description
LTBP4: May be involved in the assembly, secretion and targeting of TGFB1 to sites at which it is stored and/or activated. May play critical roles in controlling and directing the activity of TGFB1. May have a structural role in the extra cellular matrix (ECM). Defects in LTBP4 are the cause of Urban-Rifkin-Davis syndrome (URDS); also known as Cutis laxa with severe pulmonary gastrointestinal and urinary abnormalities. URDS is a syndrome characterized by disrupted pulmonary, gastrointestinal, urinary, musculoskeletal, craniofacial and dermal development. Clinical features include cutis laxa, mild cardiovascular lesions, respiratory distress with cystic and atelectatic changes in the lungs, and diverticulosis, tortuosity and stenosis at various levels of the intestinal tract. Craniofacial features include microretrognathia, flat midface, receding forehead and wide fontanelles. Belongs to the LTBP family. 4 isoforms of the human protein are produced by alternative splicing.
Protein type: Extracellular matrix; Secreted; Secreted, signal peptide
Chromosomal Location of Human Ortholog: 19q13.1-q13.2
Cellular Component: extracellular matrix; extracellular region; extracellular space; proteinaceous extracellular matrix
Molecular Function: protein binding; transforming growth factor beta binding
Biological Process: growth hormone secretion; multicellular organismal development; protein folding; regulation of cell differentiation; regulation of cell growth; regulation of proteolysis; regulation of transforming growth factor beta receptor signaling pathway
Disease: Cutis Laxa, Autosomal Recessive, Type Ic
Research Articles on LTBP4
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Product Notes
The LTBP4 ltbp4 (Catalog #AAA7044357) is an Antibody produced from Rabbit and is intended for research purposes only. The product is available for immediate purchase. The LTBP4 Antibody reacts with Human and may cross-react with other species as described in the data sheet. AAA Biotech's LTBP4 can be used in a range of immunoassay formats including, but not limited to, ELISA (EIA), Immunohistochemistry (IHC), Immunofluorescence (IF). Recommended dilution: IHC:1:20-1:200, IF:1:50-1:200. Researchers should empirically determine the suitability of the LTBP4 ltbp4 for an application not listed in the data sheet. Researchers commonly develop new applications and it is an integral, important part of the investigative research process. It is sometimes possible for the material contained within the vial of "LTBP4, Polyclonal Antibody" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.Disclaimer
Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.Item has been added to Shopping Cart
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