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Rabbit Anti-BPGM/HRP Conjugated antibody (bs-9519R-HRP)
~~~促销,代码KX240301~~~
~~~促销,代码KX240302~~~
订购热线:400-901-9800
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订购QQ:  400-901-9800
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说 明 书: 100ul  
100ul/2980.00元
大包装/询价
产品编号 bs-9519R-HRP
英文名称 Anti-BPGM/HRP
中文名称 辣根过氧化物酶标记的红细胞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.  
规格价格 100ul/2980元 购买        大包装/询价
说 明 书 100ul  
研究领域 肿瘤  心血管  细胞生物  信号转导  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 (predicted: Human, Mouse, Rat, Cow, Rabbit, Sheep, )
产品应用 WB=1:500-2000 ELISA=1:100-1000 IHC-P=1:50-200 IHC-F=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 30kDa
性    状 Lyophilized or Liquid
浓    度 1mg/ml
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 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
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.
产品介绍 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 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]. 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.
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