Product Name	Anti-RanBP2/APC
Chinese Name	APC标记的RANBinding protein2/核孔蛋白抗体
Alias	Nuclear pore complex protein Nup358; Nucleoporin 358; Nucleoporin Nup358; NUP358; P270; RAN binding protein 2; Ran-binding protein 2; RANBP2; RBP2_HUMAN; Transformation related protein 2; TRP 1; TRP 2; TRP1; TRP2; 358 kDa nucleoporin; E3 SUMO-protein ligase RanBP2.
Research Area	Cell biology  Cyclin  Cell differentiation  Epigenetics
Immunogen Species	Rabbit
Clonality	Polyclonal
React Species	(predicted: Human, Mouse, Rat, Cow, )
Applications	IF=1:50-200  not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
Molecular weight	355kDa
Form	Lyophilized or Liquid
Concentration	1mg/ml
immunogen	KLH conjugated synthetic peptide derived from human RanBP2
Lsotype	IgG
Purification	affinity purified by Protein A
Storage Buffer	0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
Storage	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.
Product Detail	background: The nuclear pore complex protein, Ran-binding protein 2 (Ran BP-2 or Nup358), contains four Ran-binding domains. Ran BP-2 is a large scaffold cyclophilin-related protein expressed in photoreceptor cells. Ran BP-2 localization at cytoplasmic fibrils emanating from the nuclear pore complex and interaction with the Ran-GTPase support its role in nucleocytoplasmic transport processes. In humans, the Ran BP-2 gene is partially duplicated in a gene cluster and lies in a hot spot for recombination on chromosome 2q. This genetic heterogeneity renders further significance of this genomic region in human disease due to its possible involvement in genetically linked disorders such as juvenile nephronophthisis, congenital hepatic fibrosis and chorioretinal dysplasia. Function: E3 SUMO-protein ligase which facilitates SUMO1 and SUMO2 conjugation by UBE2I. Involved in transport factor (Ran-GTP, karyopherin)-mediated protein import via the F-G repeat-containing domain which acts as a docking site for substrates. Could also have isomerase or chaperone activity and may bind RNA or DNA. Component of the nuclear export pathway. Specific docking site for the nuclear export factor exportin-1. Subunit: Forms a tight complex with RANBP1 and UBE2I. Interacts with SUMO1 but not SUMO2. Interacts with PARK2. Interacts with sumoylated RANGAP1. Interacts with CDCA8. Subcellular Location: Nucleus, nuclear pore complex. Note=Cytoplasmic filaments. DISEASE: Defects in RANBP2 are the cause of encephalopathy acute infection-induced type 3 (IIAE3) [MIM:608033]. A rapidly progressive encephalopathy manifesting in susceptibile individuals with seizures and coma. It can occur within days in otherwise healthy children after common viral infections such as influenza and parainfluenza, without evidence of viral infection of the brain or inflammatory cell infiltration. Brain T2-weighted magnetic resonance imaging reveals characteristic symmetric lesions present in the thalami, pons and brainstem. Note=Mutations in the RANBP2 gene predispose to IIAE3, but by themselves are insufficient to make the phenotype fully penetrant; additional genetic and environmental factors are required (PubMed:19118815). Similarity: Contains 1 PPIase cyclophilin-type domain. Contains 4 RanBD1 domains. Contains 8 RanBP2-type zinc fingers. Contains 1 TPR repeat. Database links: Entrez Gene: 5903 Human Entrez Gene: 19386 Mouse Omim: 601181 Human SwissProt: P49792 Human SwissProt: Q8C9K9 Mouse SwissProt: Q9ERU9 Mouse Unigene: 199561 Human Unigene: 715056 Human Unigene: 431695 Mouse Unigene: 445648 Mouse Unigene: 482302 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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