SNRNP200蛋白抗体
规格：1mg/1ml
英文名： SNRNP200
别名： Activating signal cointegrator 1 complex subunit 3 like 1; ASCC3L1; BRR2; BRR2 homolog; HELIC2; RP33; SNRNP200; U5 200KD; U5 small nuclear ribonucleoprotein 200 kDa helicase; U5 snRNP specific 200 kDa
分子量： 244kDa
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 SNRNP200
交叉反应：Human, Mouse, Rat,
细胞定位：细胞核
SNRNP200蛋白抗体产品介绍：background: Pre-mRNA splicing is catalyzed by the spliceosome, a complex of specialized RNA and protein subunits that removes introns from a transcribed pre-mRNA segment. The spliceosome consists of small nuclear RNA proteins (snRNPs) U1, U2, U4, U5 and U6, together with approximately 80 conserved proteins. U5 snRNP contains nine specific proteins. This gene encodes one of the U5 snRNP-specific proteins. This protein belongs to the DEXH-box family of putative RNA helicases. It is a core component of U4/U6-U5 snRNPs and appears to catalyze an ATP-dependent unwinding of U4/U6 RNA duplices. Mutations in this gene cause autosomal-dominant retinitis pigmentosa. Alternatively spliced transcript variants encoding different isoforms have been found, but the full-length nature of these variants has not been SNRNP200蛋白抗体determined. [provided by RefSeq, Mar 2010] Function: Putative RNA helicase involved in the second step of RNA splicing. May promote one or more conformational changes in the dynamic network of RNA-RNA interactions in the spliceosome. Appears to catalyze an ATP-dependent unwinding of U4/U6 RNA duplices. Subcellular Location: Nucleus. Tissue Specificity: Widely expressed. DISEASE: Defects in SNRNP200 are the cause of retinitis pigmentosa type 33 (RP33) [MIM:610359]. It is a retinal dystrophy belonging to the group of pigmentary retinopathies. Retinitis pigmentosa is characterized by retinal pigment deposits visible on fundus examination and primary loss of rod photoreceptor cells followed bySNRNP200蛋白抗体 secondary loss of cone photoreceptors. Patients typically have night vision blindness and loss of midperipheral visual field. As their condition progresses, they lose their far peripheral visual field and eventually central vision as well. Similarity: Belongs to the helicase family. SKI2 subfamily. Contains 2 helicase ATP-binding domains. Contains 2 helicase C-terminal domains. Contains 2 SEC63 domains. Database links: Entrez Gene: 23020 Human Entrez Gene: 320632 Mouse Entrez Gene: 296126 Rat Omim: 601664 Human SwissProt: O75643 Human SwissProt: Q6P4T2 Mouse SwissProt: F1LNJ2 Rat Unigene: 246112 Human Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
SNRNP200蛋白抗体产品应用：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：细胞生物 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