红细胞2,3 - 二磷酸甘油酸合成酶抗体
规格:1尘驳/1尘濒
英文名: BPGM
别名: 2,3-bisphosphoglycerate mutase; 2,3-bisphosphoglycerate synthase; 3-bisphosphoglycerate mutase; 3-bisphosphoglycerate synthase; 3-diphosphoglycerate mutase; Ab2 098; AI323730; AL022789; Bisphosphoglyc
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: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 BPGM
交叉反应:Human, Mouse, Rat, Cow, Rabbit, Sheep,
细胞定位:
红细胞2,3 - 二磷酸甘油酸合成酶抗体产物介绍:background: BPGM (2,3-bisphosphoglycerate mutase) is a 259 amino acid protein that belongs to the phosphoglycerate mutase family and exists as a homodimer that plays a crucial role in the regulation of hemoglobin oxygen. Specifically, BPGM catalyzes the conversion of 3-phospho-D-glyceroyl phosphate to 2,3-bisphospho-D-glycerate (2,3-BPG), a reaction that is essential for controlling the concentration of 2,3-BPG within the cell. The gene encoding BPGM maps to human chromosome 7, which houses over 1,000 genes and comprises nearly 5% of the human genome. Defects in some of the genes localized to chromosome 7 have been linked to Osteogenesis imperfecta, Williams-Beuren syndrome, Pendred syndrome, Lissencephaly, Citrullinemia and Shwachman-Diamond syndrome. Involvement in disease: Defects in BPGM are the cause of bisphosphoglycerate mutase deficiency (BPGMD) . A disease characterized by hemolytic anemia, splenomegaly, cholelithiasis and cholecystitis. Function: Plays a major role in regulating hemoglobin oxygen affinity by controlling the levels of its allosteric 红细胞2,3 - 二磷酸甘油酸合成酶抗体effector 2,3-bisphosphoglycerate (2,3-BPG). Also exhibits mutase (EC 5.4.2.1) and phosphatase (EC 3.1.3.13) activities. Subunit: Homodimer. Tissue Specificity: Expressed in red blood cells. Expressed in non-erythroid cells of the placenta; present in the syncytiotrophoblast layer of the placental villi at the feto-maternal interface (at protein level). Post-translational modifications: Glycation of Lys-159 in diabetic patients inactivates the enzyme. DISEASE: Defects in BPGM are the cause of bisphosphoglycerate mutase deficiency (BPGMD) [MIM:222800]. 红细胞2,3 - 二磷酸甘油酸合成酶抗体A disease characterized by hemolytic anemia, splenomegaly, cholelithiasis and cholecystitis. Similarity: Belongs to the phosphoglycerate mutase family. BPG-dependent PGAM subfamily. Database links: Entrez Gene: 669 Human Entrez Gene: 12183 Mouse Entrez Gene: 100009096 Rabbit Entrez Gene: 296973 Rat Omim: 222800 Human SwissProt: Q3T014 Cow SwissProt: P07738 Human SwissProt: P15327 Mouse SwissProt: P07952 Rabbit Unigene: 198365 Human Unigene: 282863 Mouse Unigene: 204528 Rat Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
产红细胞2,3 - 二磷酸甘油酸合成酶抗体品应用:WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:50-200 (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 心血管 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