凝集素甘露糖结合蛋白1抗体
规格：1mg/1ml
英文名： LMAN1
别名： Endoplasmic reticulum golgi intermediate compartment protein 53; ER-Golgi intermediate compartment 53 kDa protein; ERGIC-53; ERGIC53; ERGIC53 like protein; F5F8D; FMFD1; Gp58; Intracellular mannose sp
分子量： 54kDa
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 LMAN1
交叉反应：Human, Mouse, Rat, Dog, Cow, Horse, Rabbit, Sheep,
细胞定位：
凝集素甘露糖结合蛋白1抗体产品介绍：background: The protein encoded by this gene is a type I integral membrane protein localized in the intermediate region between the endoplasmic reticulum and the Golgi, presumably recycling between the two compartments. The protein is a mannose-specific lectin and is a member of a novel family of plant lectin homologs in the secretory pathway of animal cells. Mutations in the gene are associated with a coagulation defect. Using positional cloning, the gene was identified as the disease gene leading to combined factor V-factor VIII deficiency, a rare, autosomal recessive disorder in which both coagulation factors V and VIII are diminished. [provided by RefSeq, Jul 2008] Function: Mannose-specific lectin. May recognize sugar residues of glycoproteins, glycolipids, or glycosylphosphatidyl inositol anchors and may be involved in the sorting or recycling of proteins, lipids, or both. The LMAN1-MCFD2 complex forms a specific cargo receptor for the ER-to-Golgi transport of selected proteins. Subcellular Location: Endoplasmic reticulum-Golgi intermediate compartment membrane. Golgi apparatus membrane.凝集素甘露糖结合蛋白1抗体 Endoplasmic reticulum membrane. Tissue Specificity: Ubiquitous. Post-translational modifications: The N-terminal may be partly blocked. DISEASE: Defects in LMAN1 are THE cause of factor V and factor VIII combined deficiency type 1 (F5F8D1) [MIM:227300]; also known as multiple coagulation factor deficiency I (MCFD1). F5F8D1 is an autosomal recessive blood coagulation disorder characterized by bleeding symptoms similar to those in hemophilia or parahemophilia, that are caused by single deficiency of FV or FVIII, respectively. The most common symptoms are epistaxis, menorrhagia, and excessive bleeding during or after trauma. Plasma levels of coagulation factors V and VIII are凝集素甘露糖结合蛋白1抗体 in the range of 5 to 30% of normal. Similarity: Contains 1 L-type lectin-like domain. Gene ID: 3998 Database links: Entrez Gene: 3998 Human Entrez Gene: 70361 Mouse Entrez Gene: 116666 Rat Omim: 601567 Human SwissProt: P49257 Human SwissProt: Q9D0F3 Mouse SwissProt: Q62902 Rat Unigene: 465295 Human Unigene: 290857 Mouse Unigene: 449042 Mouse Unigene: 25734 Rat Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
凝集素甘露糖结合蛋白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：细胞生物 **学 信号转导
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid