Rabbit anti-Human, Mouse TRPS1 Polyclonal Antibody | anti-TRPS1 antibody
TRPS1 Conjugated Antibody
AF350 conjugated: most applications: 1:50-1:250
AF405 conjugated: most applications: 1:50-1:250
AF488 conjugated: most applications: 1:50-1:250
AF555 conjugated: most applications: 1:50-1:250
AF594 conjugated: most applications: 1:50-1:250
AF647 conjugated: most applications: 1:50-1:250
AF680 conjugated: most applications: 1:50-1:250
AF750 conjugated: most applications: 1:50-1:250
Biotin conjugated: working with enzyme-conjugated streptavidin, most applications: 1:50-1:1000
NCBI and Uniprot Product Information
NCBI Description
This gene encodes a transcription factor that represses GATA-regulated genes and binds to a dynein light chain protein. Binding of the encoded protein to the dynein light chain protein affects binding to GATA consensus sequences and suppresses its transcriptional activity. Defects in this gene are a cause of tricho-rhino-phalangeal syndrome (TRPS) types I-III. [provided by RefSeq, Jul 2008]
Uniprot Description
TRPS1: Transcriptional repressor. Binds specifically to GATA sequences and represses expression of GATA-regulated genes at selected sites and stages in vertebrate development. Regulates chondrocyte proliferation and differentiation. Executes multiple functions in proliferating chondrocytes, expanding the region of distal chondrocytes, activating proliferation in columnar cells and supporting the differentiation of columnar into hypertrophic chondrocytes. Defects in TRPS1 are the cause of tricho-rhino-phalangeal syndrome type 1 (TRPS1). TRPS1 is an autosomal dominant disorder characterized by craniofacial and skeletal abnormalities. It is allelic with tricho-rhino-phalangeal type 3. Typical features include sparse scalp hair, a bulbous tip of the nose, protruding ears, a long flat philtrum and a thin upper vermilion border. Skeletal defects include cone-shaped epiphyses at the phalanges, hip malformations and short stature. Defects in TRPS1 are a cause of tricho-rhino-phalangeal syndrome type 2 (TRPS2). A syndrome that combines the clinical features of trichorhinophalangeal syndrome type 1 and multiple exostoses type 1. Affected individuals manifest multiple dysmorphic facial features including large, laterally protruding ears, a bulbous nose, an elongated upper lip, as well as sparse scalp hair, winged scapulae, multiple cartilaginous exostoses, redundant skin, and mental retardation. A chromosomal aberration resulting in the loss of functional copies of TRPS1 and EXT1 has been found in TRPS2 patients. Defects in TRPS1 are the cause of tricho-rhino-phalangeal syndrome type 3 (TRPS3). TRPS3 is an autosomal dominant disorder characterized by craniofacial and skeletal abnormalities. It is allelic with tricho-rhino-phalangeal type 1. In TRPS3 a more severe brachydactyly and growth retardation are observed. 3 isoforms of the human protein are produced by alternative splicing.
Protein type: Transcription factor; C2H2-type zinc finger protein
Chromosomal Location of Human Ortholog: 8q24.12
Cellular Component: nucleoplasm; nucleus
Molecular Function: protein binding; zinc ion binding; sequence-specific DNA binding; transcription factor activity
Biological Process: transcription from RNA polymerase II promoter; regulation of histone deacetylation; transmembrane receptor protein serine/threonine kinase signaling pathway; chondrocyte differentiation; negative regulation of transcription from RNA polymerase II promoter; regulation of chondrocyte differentiation; NLS-bearing substrate import into nucleus; skeletal development
Disease: Trichorhinophalangeal Syndrome, Type I; Trichorhinophalangeal Syndrome, Type Iii
Research Articles on TRPS1
Similar Products
Product Notes
The TRPS1 trps1 (Catalog #AAA9444957) is an Antibody produced from Rabbit and is intended for research purposes only. The product is available for immediate purchase. The TRPS1 Conjugated Antibody reacts with Human, Mouse and may cross-react with other species as described in the data sheet. AAA Biotech's TRPS1 can be used in a range of immunoassay formats including, but not limited to, Most Applications. Suggested Dilution: AF350 conjugated: most applications: 1:50-1:250 AF405 conjugated: most applications: 1:50-1:250 AF488 conjugated: most applications: 1:50-1:250 AF555 conjugated: most applications: 1:50-1:250 AF594 conjugated: most applications: 1:50-1:250 AF647 conjugated: most applications: 1:50-1:250 AF680 conjugated: most applications: 1:50-1:250 AF750 conjugated: most applications: 1:50-1:250 Biotin conjugated: working with enzyme-conjugated streptavidin, most applications: 1:50-1:1000. Researchers should empirically determine the suitability of the TRPS1 trps1 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 "TRPS1, 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
If you are ready to order, navigate to Shopping Cart and get ready to checkout.
