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rabbit anti-TRPS1 monoclonal antibody (ZR382) 6394

$160.00$528.00

Antibody summary

  • Rabbit monoclonal to TRPS1
  • Suitable for: Immunohistochemistry (formalin-fixed, paraffin-embedded tissues)
  • Reacts with: Human
  • Isotype:IgG
  • Control: Normal breast or breast carcinoma
  • Visualization: Nucleus
  • 0.1, 0.5, 1.0 mL concentrated, 7 mL prediluted
SKU: 6394parent Category: Tags: , ,
Weight1 lbs
Dimensions9 × 5 × 2 in
host

mouse

isotype

IgG

clonality

monoclonal

concentration

concentrate, predilute

applications

IHC

reactivity

human

available size

0.1 mL, 0.5 mL, 1 mL concentrated, 7 mL prediluted

rabbit anti-TRPS1 monoclonal antibody ZR382 6394

antibody
Database link:
human Q9UHF7
Tested applications
IHC
Recommended dilutions
As directed
Immunogen
Synthesized peptide corresponding to the C-terminus of human TRPS1 protein
Size and concentration
7 mL prediluted or 0.1, 0.5, 1.0 mL and concentrated
Form
liquid
Storage Instructions
2-8°C for short term, for longer term at -20°C. Avoid freeze / thaw cycles.
Purity
affinity purified
Clonality
monoclonal
Isotype
IgG
Compatible secondaries
goat anti-rabbit IgG, H&L chain specific, peroxidase conjugated, conjugated polyclonal antibody 9512
goat anti-rabbit IgG, H&L chain specific, biotin conjugated polyclonal antibody 2079
goat anti-rabbit IgG, H&L chain specific, FITC conjugated polyclonal antibody 7863
goat anti-rabbit IgG, H&L chain specific, Cross Absorbed polyclonal antibody 2371
goat anti-rabbit IgG, H&L chain specific, biotin conjugated polyclonal antibody, crossabsorbed 1715
goat anti-rabbit IgG, H&L chain specific, FITC conjugated polyclonal antibody, crossabsorbed 1720
Isotype control
Rabbit polyclonal - Isotype Control
target relevance
Protein names
Zinc finger transcription factor Trps1 (Tricho-rhino-phalangeal syndrome type I protein) (Zinc finger protein GC79)
Mass
141521Da
Function
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.
Subellular location
Nucleus .
Tissues
Ubiquitously 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.
Structure
Interacts with RNF4; regulates TRPS1 repressor activity. Interacts specifically with the activator form of GLI3 (GLI3A) but not with the repressor form (GLI3R).
Post-translational modification
PTM: Sumoylated. 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.
Involvement in disease
Tricho-rhino-phalangeal syndrome 1 (TRPS1) [MIM:190350]: 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. Note=The disease is caused by variants affecting the gene represented in this entry.; Tricho-rhino-phalangeal syndrome 2 (TRPS2) [MIM:150230]: A syndrome that combines the clinical features of tricho-rhino-phalangeal 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 intellectual disability. Note=The gene represented in this entry is involved in disease pathogenesis. A chromosomal aberration resulting in the loss of functional copies of TRPS1 and EXT1 has been found in TRPS2 patients.; Tricho-rhino-phalangeal syndrome 3 (TRPS3) [MIM:190351]: 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. Note=The disease is caused by variants affecting the gene represented in this entry.
Target Relevance information above includes information from UniProt accession: Q9UHF7
The UniProt Consortium

Data

Formalin-fixed, paraffin-embedded human breast carcinoma stained with anti-TRPS1 antibody using peroxidase-conjugate and DAB chromogen. Note the nuclear staining of tumor cells
Formalin-fixed, paraffin-embedded human breast carcinoma stained with anti-TRPS1 antibody using peroxidase-conjugate and DAB chromogen. Note the nuclear staining of tumor cells

Publications

Published literature highly relevant to the biological target of this product and referencing this antibody or clone are retrieved from PubMed database provided by The United States National Library of Medicine at the National Institutes of Health.




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Protocols

relevant to this product
IHC

Documents

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