多发性外生骨疣蛋白2抗体
规格：1尘驳/1尘濒
英文名： EXT2
别名： Exostoses (multiple) 2; Exostosin 2; Exostosin-2; EXT2; EXT2_HUMAN; Glucuronosyl N acetylglucosaminyl proteoglycan 4 alpha N acetylglucosaminyltransferase; Glucuronosyl-N-acetylglucosaminyl-proteoglyc
分子量： 82kDa
储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型：笔辞濒测肠濒辞苍补濒
亚型：滨驳骋
纯化方法：affinity purified by Protein A
**原：KLH conjugated synthetic peptide derived from human EXT2
交叉反应：Human, Mouse, Rat, Chicken, Dog, Pig, Cow, Sheep,
细胞定位：
多发性外生骨疣蛋白2抗体产物介绍：background: This gene encodes one of two glycosyltransferases involved in the chain elongation step of heparan sulfate biosynthesis. Mutations in this gene cause the type II form of multiple exostoses. Alternatively spliced transcript variants encoding different isoforms have been noted for this gene. [provided by RefSeq, Jul 2008] Function: Glycosyltransferase required for the biosynthesis of heparan-sulfate. The EXT1/EXT2 complex possesses substantially higher glycosyltransferase activity than EXT1 or EXT2 alone. Appears to be a tumor suppressor. Subunit: Forms a homo/hetero-oligomeric complex with EXT1. Interacts with GALNT5. Subcellular Location: Endoplasmic reticulum membrane. Golgi apparatus membrane. The EXT1/EXT2 complex is localized in the Golgi apparatus. Tissue Specificity: Ubiquitous.多发性外生骨疣蛋白2抗体 DISEASE: Defects in EXT2 are a cause of hereditary multiple exostoses type 2 (EXT2) [MIM:133701]. EXT is a genetically heterogeneous bone disorder caused by genes segregating on human chromosomes 8, 11, and 19 and designated EXT1, EXT2 and EXT3 respectively. EXT is a dominantly inherited skeletal disorder primarily affecting endochondral bone during growth. The disease is characterized by formation of numerous cartilage-capped, benign bone tumors (osteocartilaginous exostoses or osteochondromas) that are often accompanied by skeletal deformities多发性外生骨疣蛋白2抗体 and short stature. In a small percentage of cases exostoses have exhibited malignant transformation resulting in an osteosarcoma or chondrosarcoma. Osteochondromas development can also occur as a sporadic event. Defects in EXT2 are the cause of Potocki-Shaffer syndrome (PSS) [MIM:601224]. It is a contiguous gene syndrome due to proximal deletion of chromosome 11p11.2, including EXT2 and ALX4. Similarity: Belongs to the glycosyltransferase 47 family. Gene ID: 2132 Database links: Entrez Gene: 2132 Human Entrez Gene: 281151 Cow Entrez Gene: 14043 Mouse Entrez Gene: 311215 Rat Omim: 608210 Human SwissProt: O77783 Cow SwissProt: Q93063 Human SwissProt: P70428 Mouse Unigene: 368404 Human Unigene: 4336 Mouse Unigene: 2878 Rat Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
多发性外生骨疣蛋白2抗体产物应用：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 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