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Rabbit Anti-GTF2IRD1/BF647 Conjugated antibody (bs-16354R-BF647)
訂購熱線:400-901-9800
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說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產品編號 bs-16354R-BF647
英文名稱 Rabbit Anti-GTF2IRD1/BF647 Conjugated antibody
中文名稱 BF647標記的通用轉錄因子III/WBSCR11抗體
別    名 CREAM1; General transcription factor II I repeat domain containing protein 1; General transcription factor II-I repeat domain-containing protein 1; General transcription factor III; GT2D1_HUMAN; GTF2I repeat domain containing protein 1; GTF2I repeat domain-containing protein 1; GTF2IRD1; GTF3; Muscle TFII I repeat domain-containing protein 1; Muscle TFII-I repeat domain-containing protein 1; MUSTRD1; MusTRD1/BEN; RBAP2; Slow muscle fiber enhancer binding protein; Slow-muscle-fiber enhancer-binding protein; USE B1 binding protein; USE B1-binding protein; WBSCR11; WBSCR12; Williams Beuren syndrome chromosome region 11 protein; Williams-Beuren syndrome chromosomal region 11 protein; Williams-Beuren syndrome chromosomal region 12 protein.  
規格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
研究領域 細胞生物  轉錄調節因子  表觀遺傳學  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 (predicted: Human, Mouse, Rat, Dog, Pig, Cow, Horse, Sheep, )
產品應用 ICC=1:50-200 IF=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 106kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human GTF2IRD1
亞    型 IgG
純化方法 affinity purified by Protein A
儲 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存條件 Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
產品介紹 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. [provided by RefSeq, Nov 2010]

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.

Subcellular Location:
Nucleus.

Tissue Specificity:
Highly expressed in adult skeletal muscle, heart, fibroblast, bone and fetal tissues. Expressed at lower levels in all other tissues tested.

DISEASE:
Note=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.

Similarity:
Belongs to the TFII-I family.
Contains 5 GTF2I-like repeats.

Database links:

Entrez Gene: 9569 Human

Entrez Gene: 57080 Mouse

Entrez Gene: 246770 Rat

Omim: 604318 Human

SwissProt: Q9UHL9 Human

SwissProt: Q9JI57 Mouse

Unigene: 647056 Human

Unigene: 332735 Mouse



Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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