Overview

  • Product name

  • Description

    Rabbit polyclonal to TRPS1
  • Host species

    Rabbit
  • Tested applications

    Suitable for: ICC/IFmore details
  • Species reactivity

    Reacts with: Human
    Predicted to work with: Mouse, Rat, Xenopus laevis
  • Immunogen

    Recombinant fragment corresponding to Human TRPS1 aa 1085-1175.
    Sequence:

    SPIEKYQYPLFGLPFVHNDFQSEADWLRFWSKYKLSVPGNPHYLSHVPGL PNPCQNYVPYPTFNLPPHFSAVGSDNDIPLDLAIKHSRPGP


    Database link: Q9UHF7

  • Positive control

    • MCF7 cells

Properties

Applications

Our Abpromise guarantee covers the use of ab221118 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

Application Abreviews Notes
ICC/IF Use a concentration of 1 - 4 µg/ml.

Fixation/Permeabilization: PFA/Triton X-100.

Target

  • 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.
  • Tissue specificity

    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.
  • Involvement in disease

    Defects 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 similarities

    Contains 7 C2H2-type zinc fingers.
    Contains 1 GATA-type zinc finger.
  • Post-translational
    modifications

    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.
  • Cellular localization

    Nucleus.
  • Information by UniProt
  • Database links

  • Alternative names

    • GC79 antibody
    • LGCR antibody
    • Transcriptional repressor GATA binding 1 antibody
    • Tricho rhino phalangeal syndrome I antibody
    • Tricho-rhino-phalangeal syndrome type I protein antibody
    • Trichorhinophalangeal syndrome I antibody
    • Trichorhinophalangeal syndrome I homolog antibody
    • TRPS 1 antibody
    • trpS1 antibody
    • TRPS1 gene antibody
    • TRPS1_HUMAN antibody
    • Zinc finger protein GC79 antibody
    • Zinc finger transcription factor TRPS 1 antibody
    • Zinc finger transcription factor Trps1 antibody
    see all

Images

  • Immunofluorescent analysis of PFA-fixed, Triton X-100 permeabilized MCF7 cells labeling TRPS1 with ab221118 at 4 µg/ml (green).

References

ab221118 has not yet been referenced specifically in any publications.

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