Human Trichorhinophalangeal Syndrome Protein I (TRPS1) ELISA Kit | TRPS1 elisa kit

Human Trichorhinophalangeal Syndrome Protein I (TRPS1) ELISA Kit

Cat. Num. AAA2707784

• Reactivity: Human
• Synonyms: Trichorhinophalangeal Syndrome Protein I (TRPS1); Human Trichorhinophalangeal Syndrome Protein I (TRPS1) ELISA Kit; LGCR; Tricho-rhino-phalangeal syndrome type I protein; Zinc finger protein GC79; TRPS1 elisa kit

• Reactivity: Human
• Specificity: This assay has high sensitivity and excellent specificity for detection of Trichorhinophalangeal Syndrome Protein I (TRPS1).; No significant cross-reactivity or interference between Trichorhinophalangeal Syndrome Protein I (TRPS1) and analogues was observed.
• Sequence Length: 1294

• Assay Type: Double-antibody Sandwich
• Samples: Tissue Homogenates, Cell Lysates and Other Biological Fluids
• Detection Range: 0.156-10ng/mL
• Sensitivity: The minimum detectable dose of this kit is typically less than 0.056ng/mL
• Precision: Intra-assay Precision (Precision within an assay): 3 samples with low, middle and high level Trichorhinophalangeal Syndrome Protein I (TRPS1) were tested 20 times on one plate, respectively.; Inter-assay Precision (Precision between assays): 3 samples with low, middle and high level Trichorhinophalangeal Syndrome Protein I (TRPS1) were tested on 3 different plates, 8 replicates in each plate.; CV(%) = SD/meanX100; Intra-Assay: CV<10%; Inter-Assay: CV<12%
• Preparation and Storage: The stability of kit is determined by the loss rate of activity. The loss rate of this kit is less than 5% within the expiration date under appropriate storage condition.; To minimize extra influence on the performance, operation procedures and lab conditions, especially room temperature, air humidity, incubator temperature should be strictly controlled. It is also strongly suggested that the whole assay is performed by the same operator from the beginning to the end.

Typical Testing Data/Standard Curve (for reference only)

Related Product Information for TRPS1 elisa kit

The test principle applied in this kit is Sandwich enzyme immunoassay. The microtiter plate provided in this kit has been pre-coated with an antibody specific to Trichorhinophalangeal Syndrome Protein I (TRPS1). Standards or samples are then added to the appropriate microtiter plate wells with a biotin-conjugated antibody specific to Trichorhinophalangeal Syndrome Protein I (TRPS1). Next, Avidin conjugated to Horseradish Peroxidase (HRP) is added to each microplate well and incubated. After TMB substrate solution is added, only those wells that contain Trichorhinophalangeal Syndrome Protein I (TRPS1), biotin-conjugated antibody and enzyme-conjugated Avidin will exhibit a change in color. The enzyme-substrate reaction is terminated by the addition of sulphuric acid solution and the color change is measured spectrophotometrically at a wavelength of 450nm ± 10nm. The concentration of Trichorhinophalangeal Syndrome Protein I (TRPS1) in the samples is then determined by comparing the O.D. of the samples to the standard curve.

NCBI and Uniprot Product Information

• NCBI GI #: 90652851
• NCBI Accession #: NP_054831.2
• NCBI GenBank Nucleotide #: NP_054831.2
• UniProt Accession #: Q9UHF7
• 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