| 產(chǎn)品編號(hào) |
bs-19769R-PE-Cy7 |
| 英文名稱(chēng)1 |
Rabbit Anti-RBM28/PE-Cy7 Conjugated antibody
|
| 中文名稱(chēng) |
PE-Cy7標(biāo)記的RNA結(jié)合蛋白28抗體 |
| 別 名 |
2810480G15Rik;
FLJ10377;
RBM 28;
RBM28;
RBM28_HUMAN;
RNA binding motif protein 28;
RNA binding protein 28;
RNA-binding motif protein 28;
RNA-binding protein 28. |
| 規(guī)格價(jià)格 |
100ul/2980元
購(gòu)買(mǎi) 大包裝/詢(xún)價(jià) |
| 說(shuō) 明 書(shū) |
100ul
|
| 研究領(lǐng)域 |
細(xì)胞生物 發(fā)育生物學(xué) 神經(jīng)生物學(xué) 結(jié)合蛋白 表觀遺傳學(xué) |
| 抗體來(lái)源 |
Rabbit |
| 克隆類(lèi)型 |
Polyclonal |
| 交叉反應(yīng) |
(predicted: Human, Mouse, Dog, Horse, Rabbit, )
|
| 產(chǎn)品應(yīng)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 |
85kDa |
| 性 狀 |
Lyophilized or Liquid |
| 濃 度 |
1mg/ml |
| 免 疫 原 |
KLH conjugated synthetic peptide derived from human RBM28 |
| 亞 型 |
IgG |
| 純化方法 |
affinity purified by Protein A |
| 儲(chǔ) 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(chǎn)品介紹 |
background:
The protein encoded by this gene is a specific nucleolar component of the spliceosomal small nuclear ribonucleoprotein (snRNP)complexes . It specifically associates with U1, U2, U4, U5, and U6 small nuclear RNAs (snRNAs), possibly coordinating their transition through the nucleolus. Mutation in this gene causes alopecia, progressive neurological defects, and endocrinopathy (ANE syndrome), a pleiotropic and clinically heterogeneous disorder. Alternatively spliced transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Oct 2009]
Function:
Nucleolar component of the spliceosomal ribonucleoprotein complexes.
Subcellular Location:
Nucleus > nucleolus.
Tissue Specificity:
Ubiquitously expressed.
DISEASE:
Defects in RBM28 are the cause of alopecia neurologic defects and endocrinopathy syndrome (ANES) [MIM:612079]. Affected individuals have hair loss of variable severity, ranging from complete alopecia to near-normal scalp hair with absence of body hair. All have moderate to severe mental retardation, progressive motor deterioration and central hypogonadotropic hypogonadism with delayed or absent puberty and central adrenal insufficiency. Additional features included short stature, microcephaly, gynecomastia, pigmentary anomalies, hypodontia, kyphoscoliosis, ulnar deviation of the hands, and loss of subcutaneous fat.
Similarity:
Contains 4 RRM (RNA recognition motif) domains.
Database links:
Entrez Gene: 475203 Dog
Entrez Gene: 100071725 Horse
Entrez Gene: 55131 Human
Entrez Gene: 100338231 Rabbit
Omim: 612074 Human
SwissProt: Q9NW13 Human
Unigene: 274263 Human
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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