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The antibody against GTF2IRD1 was raised in rabbit using the Synthesized peptide derived from the N-terminal region of Human WBSCR11. as the immunogen. This antibody exists as a non-conjugated isotype IgG. The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen. This antibody has been validated on WB, IHC, ELISA.
The antibody against GTF2IRD1 was raised in rabbit using the Synthesized peptide derived from the N-terminal region of Human WBSCR11. as the immunogen. This antibody exists as a non-conjugated isotype IgG. The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen. This antibody has been validated on WB, IHC, ELISA.
$167.00
Western Blot analysis of 293 cells using WBSCR11 Polyclonal Antibody
| Cat.No | ADC-37371A | Clonality | Polyclonal |
|---|---|---|---|
| Host Species | Rabbit | Target Name | GTF2IRD1 |
| Form | Liquid | Species Reactivity | Human, Mouse, Rat |
| Isotype | IgG | Storage Buffer | 0.5% BSA and 0.02% sodium azide., Liquid in PBS containing 50% glycerol |
| Purification Method | The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen. | Conjugate | Non-conjugated |
| Application | ELISA, IHC, WB | Storage | Upon receipt |
| Immunogen Description | Synthesized peptide derived from the N-terminal region of Human WBSCR11. | Target Species | Human |
|---|---|---|---|
| Immunogen Sequence | Complete sequences for the immunogen, target protein, and peptides are available upon request. | Uniprot ID | Q9UHL9 |
Uniprot Id
Q9UHL9
Target Species
Human
Target Name
GTF2IRD1
Target Full Name
General transcription factor II-I repeat domain-containing protein 1
Target Function
May be a transcription regulator involved in cell-cycle progression and skeletal muscle differentiation. May repress GTF2I transcriptional functions, by preventing its nuclear residency, or by inhibiting its transcriptional activation. May contribute to slow-twitch fiber type specificity during myogenesis and in regenerating muscles. Binds troponin I slow-muscle fiber enhancer (USE B1). Binds specifically and with high affinity to the EFG sequences derived from the early enhancer of HOXC8.
Target Involvement
GTF2IRD1 is located in the Williams-Beuren syndrome (WBS) critical region. WBS results from a hemizygous deletion of several genes on chromosome 7q11.23, thought to arise as a consequence of unequal crossing over between highly homologous low-copy repeat sequences flanking the deleted region. Haploinsufficiency of GTF2IRD1 may be the cause of certain cardiovascular and musculo-skeletal abnormalities observed in the disease.
Target Subcellular Location
Nucleus.
Target Protein Families
TFII-I family
Target Tissue Specificity
Highly expressed in adult skeletal muscle, heart, fibroblast, bone and fetal tissues. Expressed at lower levels in all other tissues tested.
Target Synonyms
BEN; WBS; GTF3; RBAP2; CREAM1; MUSTRD1; WBSCR11; WBSCR12; hMusTRD1alpha1; GTF2IRD1
Target Background
The protein encoded by this gene contains five GTF2I-like repeats and each repeat possesses a potential helix-loop-helix (HLH) motif. It may have the ability to interact with other HLH-proteins and function as a transcription factor or as a positive transcriptional regulator under the control of Retinoblastoma protein. This gene plays a role in craniofacial and cognitive development and mutations have been associated with Williams-Beuren syndrome, a multisystem developmental disorder caused by deletion of multiple genes at 7q11.23. Alternative splicing results in multiple transcript variants.
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