Background:

The autosomal dominant tricho-rhino-phalangeal syndrome type 1 (TRPS1) is a rare disorder clinically characterized by sparse scalp hair, a bulbous nose, protruding ears, a thin upper lip, an elongated philtrum and bone deformities. The human TRPS1 gene maps to chromosome 8q24 and encodes a GATA-type zinc-finger protein. TRPS1 binds GATA sequences but does not activate GATA-dependent transcription. In fact, TRPS1 represses transcriptional activation mediated by other GATA factors. The noncompetitive mechanism for transcriptional repression depends upon an Ikaros-like C-terminal region. In mice, mutations in the GATA domain of TRPS1 cause facial abnormalities that parallel TRPS1 symptoms. TRPS1 is expressed during mouse embryonic development in developing joints, hair follicles, snout, lung, spine and brain.

Specificity:

TRPS1 polyclonal antibody detects endogenous levels of TRPS1 protein.

Alternative names:

Zinc finger transcription factor Trps1; Tricho-rhino-phalangeal syndrome type I protein; Zinc finger protein GC79

Species reactivity:

Human,Mouse,Rat

Host:

Rabbit

Ig type:

Rabbit IgG, 1mg/ml in PBS with 0.02% sodium azide, 50% glycerol, pH7.2

Immunogen:

Synthetic peptide, corresponding to amino acids 137-181 of Human TRPS1

Purification & Purity:

The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen and the purity is > 95% (by SDS-PAGE)

Molecular weight:

~ 141 kDa

Applications:

WB

Usage:

WB: 1:500~1:1000

Storage:

Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze-thaw cycles.

Precautions:

For research use only, not for use in diagnostic procedure.

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