供货周期: | 现货 |
品牌: | GenWay |
规格: | 0.1ml/100μg 0.2ml/200μg |
货号: | BJ-3905 |
CAS号: |
红细胞2,3 - 二磷酸甘油酸合成酶抗体英文名称 Anti-BPGM
中文名称 红细胞2,3 - 二磷酸甘油酸合成酶抗体
别 名 2,3-bisphosphoglycerate mutase; 2,3-bisphosphoglycerate synthase; 3-bisphosphoglycerate mutase; 3-bisphosphoglycerate synthase; 3-diphosphoglycerate mutase; Ab2 098; AI323730; AL022789; Bisphosphoglycerate mutase; BPG dependent PGAM; BPG-dependent PGAM ; Bpgm; BPGM; C86192; DPGM; Erythrocyte 2,3 bisphosphoglycerate mutase; PMGE_HUMAN; 2,3-bisphosphoglycerate mutase, erythrocyte; erythrocyte; 2 antibody.
浓 度 1mg/1ml
红细胞2,3 - 二磷酸甘油酸合成酶抗体规 格 0.2ml/200μg
抗体来源 Rabbit
克隆类型 polyclonal
交叉反应 Human, Mouse, Rat, Cow, Rabbit, Sheep
产品类型 一抗
研究领域 肿瘤 心血管 细胞生物 信号转导
蛋白分子量 predicted molecular weight: 30kDa
性 状 Lyophilized or Liquid
免 疫 原 KLH conjugated synthetic peptide derived from human BPGM
亚 型 IgG
纯化方法 affinity purified by Protein A
储 存 液 Preservative: 15mM Sodium Azide, Constituents: 1% BSA, 0.01M PBS, pH 7.4
产品应用 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.
保存条件 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.
Important Note This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
产品介绍 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 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 c红细胞2,3 - 二磷酸甘油酸合成酶抗体ells 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]. A disease characterized by hemolytic anemia, splenomegaly, cholelithiasis and cholecystitis.
Similarity : Belongs to the phosphoglycerate mutase family. BPG-dependent PGAM subfamily.
Database links : UniProtKB/Swiss-Prot: P07738.2
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