多谷氨酰胺结合蛋白1抗体
规格：1mg/1ml
英文名： PQBP1
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 t
分子量： 30kDa
储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型：Polyclonal
亚型：IgG
纯化方法：affinity purified by Protein A
**原：KLH conjugated synthetic peptide derived from human PQBP1 (1
交叉反应：Human, Mouse, Rat, Dog, Pig, Cow, Horse, Rabbit, Sheep,
细胞定位：细胞核
产品介绍：background: Polyglutamine(Q) tract binding protein-1 (PQBP-1) is a transcription repressor that associates with polyglutamine tract-containing 多谷氨酰胺结合蛋白1抗体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多谷氨酰胺结合蛋白1抗体 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. Tissue Specificity: Widely expressed with high level in heart, skeletal muscle, pancreas, spleen, thymus, prostate, ovary, small intestine and peripheral blood leukocytes. 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, 多谷氨酰胺结合蛋白1抗体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 Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
多谷氨酰胺结合蛋白1抗体产品应用：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.
研究领域：肿瘤 细胞生物 神经生物学 表观遗传学
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid