Rabbit TRPS1 Polyclonal Antibody | anti-TRPS1 antibody

TRPS1 Polyclonal Antibody

Cat. Num. AAA3007444

• Reactivity: Human, Mouse, Rat
• Applications: Western Blot
• Purity: The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen and the purity is >95% (by SDS-PAGE)
• Synonyms: TRPS1; Polyclonal Antibody; TRPS1 Polyclonal Antibody; Zinc finger transcription factor Trps1; Tricho-rhino-phalangeal syndrome type I protein; Zinc finger protein GC79; anti-TRPS1 antibody

• Host: Rabbit
• Reactivity: Human, Mouse, Rat
• Clonality: Polyclonal
• Isotype: IgG
• Specificity: TRPS1 polyclonal antibody detects endogenous levels of TRPS1 protein.
• Purity/Purification: The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen and the purity is >95% (by SDS-PAGE)
• Form/Format: Rabbit IgG, 1mg/ml in PBS with 0.02% sodium azide, 50% glycerol, pH7.2
• Sequence Length: 1294

• Applicable Applications for anti-TRPS1 antibody: Western Blot (WB)
• Application Notes: WB: 1:500-1:1000
• Immunogen: Synthetic peptide, corresponding to amino acids 137-181 of Human TRPS1
• Preparation and Storage: Store at 4 degree C short term.; Aliquot and store at -20 degree C long term.; Avoid freeze-thaw cycles.

Western Blot (WB)

(Western Blot (WB) analysis of TRPS1 polyclonal antibody at 1:500 dilutionLane1:Hela whole cell lysate (40ug) Lane2:PC3 whole cell lysate (40ug) Lane3:The fetal Brain tissue lysate of Mouse (40ug) Lane4:The fetal Brain tissue lysate of Rat (40ug))

Related Product Information for anti-TRPS1 antibody

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.

NCBI and Uniprot Product Information

• NCBI GI #: 90652851
• NCBI Accession #: NP_054831.2
• NCBI GenBank Nucleotide #: NP_054831.2
• UniProt Accession #: Q9UHF7
• Molecular Weight: ~ 141kDa
• NCBI Official Full Name: zinc finger transcription factor Trps1 isoform 1
• UniProt Protein Name: Zinc finger transcription factor Trps1
• Protein Family: Tryptophan--tRNA ligase
• UniProt Gene Name: TRPS1
• UniProt Entry Name: TRPS1_HUMAN

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