Storage instructionsShipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C long term. Avoid freeze / thaw cycle.
Storage bufferPreservative: 0.09% Sodium azide
Constituents: 99% Tris buffered saline, 0.1% BSA
pH 7 to 8
Concentration information loading...
PurityImmunogen affinity purified
Our Abpromise guarantee covers the use of ab176712 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
|WB||1/2000 - 1/10000. Predicted molecular weight: 142 kDa.|
|IP||Use at 2-10 µg/mg of lysate.|
FunctionTranscriptional 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.
Tissue specificityUbiquitously expressed in the adult. Found in fetal brain, lung, kidney, liver, spleen and thymus. More highly expressed in androgen-dependent than in androgen-independent prostate cancer cells.
Involvement in diseaseDefects in TRPS1 are the cause of tricho-rhino-phalangeal syndrome type 1 (TRPS1) [MIM:190350]. 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) [MIM:150230]. 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. Note=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) [MIM:190351]. 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.
Sequence similaritiesContains 7 C2H2-type zinc fingers.
Contains 1 GATA-type zinc finger.
modificationsSumoylated. Sumoylation in the repressor domain inhibits the transcription repression activity. Sumoylation on Lys-1201 is the major site. Appears to be sumoylated on multiple sites.
- Information by UniProt
- GC79 antibody
- LGCR antibody
- Transcriptional repressor GATA binding 1 antibody
All lanes : Anti-TRPS1 antibody (ab176712) at 0.04 µg/ml
Lane 1 : HeLa whole cell lysate at 50 µg
Lane 2 : HeLa whole cell lysate at 15 µg
Lane 3 : 293T whole cell lysate at 50 µg
Lane 4 : Jurkat whole cell lysate at 50 µg
Developed using the ECL technique.
Predicted band size: 142 kDa
Exposure time: 30 seconds
TRPS1 was immunoprecipitated from 1mg HeLa whole cell lysate using ab176712 at 6 μg/mg lysate (Lane 1), a rabbit anti-TRPS1 antibody recognizing a downstream epitope of TRPS1 (Lane 2) or control IgG (Lane 3). 20% of the Immunoprecipitate was loaded per lane and then immunobotted using ab176712 at 0.4 μg/ml.
Chemiluminescence with exposure times of 10 seconds.
ab176712 has not yet been referenced specifically in any publications.