产品订购信息：
英文名称  Anti-PQBP1 

中文名称   多谷氨酰胺结合蛋白1抗体品牌 

别    名  38 kDa nuclear protein containing a WW domain; Mental retardation, X linked 55; MRX55; MRXS3; MRXS8; Npw38; Nuclear protein containing WW domain 38 kD; Polyglutamine binding protein 1; Polyglutamine tract binding protein 1; Polyglutamine tract-binding protein 1; Polyglutamine-binding protein 1; PQBP 1; PQBP-1; PQBP1; PQBP1_HUMAN; RENS1; SHS; Sutherland Haan X linked mental retardation syndrome.

浓    度   1mg/1ml

规 格  0.2ml/200μg

抗体来源   Rabbit

克隆类型   polyclonal

交叉反应   Human, Mouse, Rat, Dog, Pig, Cow, Horse, Rabbit, Sheep 

产品类型   一抗  

研究领域     细胞生物 神经生物学 表观遗传学

蛋白分子量  predicted molecular weight: 30kDa

性    状   Lyophilized or Liquid

免 疫 原 KLH conjugated synthetic peptide derived from human PQBP1 (185-265aa) 

亚    型   IgG

纯化方法   affinity purified by Protein A

储 存 液   Preservative: 15mM Sodium Azide, Constituents: 1% BSA, 0.01M PBS, pH 7.4

多谷氨酰胺结合蛋白1抗体品牌 产品应用    WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500

（石蜡切片需做抗原修复） 

 not yet tested in other applications.

 optimal dilutions/concentrations should be determined by the end user.  

保存条件  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. 

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

产品介绍 Polyglutamine(Q) tract binding protein-1 (PQBP-1) is a transcription repressor that associates with polyglutamine tract-containing transcription regulators and causative genes for neurodegenerative disorders. Hepta- and di-amino acid repeat sequences rich in polar residues are essential for PQBP-1 to interact with polyglutamine tract-containing proteins (i.e. huntingtin, androgen receptor and Brain-2). PQBP-1 contains a WWP/WW domain that binds proline-rich motifs and a C2 domain that can influence Ca2+-dependent phospholipid signaling. PQBP-1 localizes to the nucleus and is present in neurons throughout the brain, with abundant levels in hippocampus, cerebellar cortex and olfactory bulb. The human PQBP-1 gene maps to chromosome Xp11.23.

Function : May suppress the ability of POU3F2 to transactivate the DRD1 gene in a POU3F2 dependent manner. Can activate transcription directly or via association with the transcription machinery. May be involved in ATXN1 mutant-induced cell death. The interaction with ATXN1 mutant reduces levels of phosphorylated RNA polymerase II large subunit.

Subunit : nteracts with POU3F2/Brn-2, ATXN1, TXNL4A, HTT and AR.Interaction with ATXN1 correlates positively with the length of thepolyglutamine tract. Interacts with RNA polymerase II large subunitin a phosphorylation-dependent manner. Forms a ternary complex withATXN1 mutant and phosphorylated RNA polymerase II.

Subcellular Location : Nucleus. Co-localized with POU3F2. Co-localized with ATXN1 in nuclear inclusion bodies.

DISEASE : Defects in PQBP1 are the cause of Renpenning syndrome 1 (RENS1) [MIM:309500]; also known as Sutherland-Haan X-linked mental retardation syndrome (SHS) or X-linked mental retardation syndromes MRXS3/MRXS8/MRX55. The clinical features are mental retardation, microcephaly, short stature, and small testes. The craniofacies tends to be narrow and tall with upslanting palpebral fissures, abnormal nasal configuration, cupped ears, and short philtrum. The nose may appear long or bulbous, with overhanging columella. Less consistent manifestations include ocular colobomas, cardiac malformations, cleft palate, and anal anomalies. RENS1 is more frequently in males than in females where little or no expression is found.

Similarity : Contains 1 WW domain.

Database links : UniProtKB/Swiss-Prot: O60828.1

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抗体的生物素化标记实验要点：

1. 多谷氨酰胺结合蛋白1抗体品牌 如在反应混合液中有叠氮钠或游离氨基存在,会抑制标记反应。因此,蛋白质在反应前要对 0.1mol/L碳酸氢钠缓冲液或0.5mol/L硼酸缓冲液充分透析；

2.所用的NHSB及待生物素化蛋白质之间的分子比按蛋白质表面的ε-氨基的密度会有所不同,选择不当则影响标记的效率,应先用几个不同的分子比来筛选最适条件；

3.用NHSB量过量也是不利的,抗原的结合位点可能因此被封闭,导致抗体失活；

4.由于抗体的氨基不易接近可能造成生物素化不足,此时可加入去污剂如 Triton x-100, Tween20等；

5.当游离ε-氨基(赖氨酸残基的氨基)存在于抗体的抗原结合位点时,或位于酶的催化位点时,生物素化会降低或损伤抗体蛋白的结合力或活性；

6.生物素还可能与不同的功能基团,如羰基、氨基、巯基、异咪唑基及*基,也可与糖基共价结合；

7.交联反应后,应充分透析,否则,残余的生物素会对生物素化抗体与亲和素的结合产生竞争作用；

8.在细胞的荧光标记实验中,中和亲和素的本底低,但由于链霉亲和素含有少量正电荷,故对某些细胞可导致高本底。

抗体的鉴定：

1）多谷氨酰胺结合蛋白1抗体品牌 抗体的效价鉴定：不管是用于诊断还是用于,制备抗体的目的都是要求较高效价。不同的抗原制备的抗体,要求的效价不一。鉴定效价的方法很多,包括有试管凝集反应,琼脂扩散试验,酶联免疫吸附试验等。常用的抗原所制备的抗体一般都有约成的鉴定效价的方法,以资比较。如制备抗抗体的效价,一般就采用琼脂扩散试验来鉴定。

2）抗体的特异性鉴定：抗体的特异性是指与相应抗原或近似抗原物质的识别能力。抗体的特异性高,它的识别能力就强。衡量特异性通常以交叉反应率来表示。交叉反应率可用竞争抑制试验测定。以不同浓度抗原和近似抗原分别做竞争抑制曲线,计算各自的结合率,求出各自在IC50时的浓度,并按公式计算交叉反应率。 

如果所用抗原浓度IC50浓度为pg/管,而一些近似抗原物质的IC50浓度几乎是无穷大时,表示这一抗血清与其他抗原物质的交叉反应率近似为0,即该血清的特异性较好。

3）抗体亲和力：是指抗体和抗原结合的牢固程度。亲和力的高低是由抗原分子的大小,抗体分子的结合位点与抗原决定簇之间立体构型的合适度决定的。有助于维持抗原抗体复合物稳定的分子间力有氢键,疏水键,侧链相反电荷基因的库仑力,范德华力和空间斥力。亲和力常以亲和常数K表示,K的单位是L/mol。抗体亲和力的测定对抗体的筛选,确定抗体的用途,验证抗体的均一性等均有重要意义。