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货号： bs-23501R-AF555 基本售价： 2980.0 元 规格： 100ul
产品信息
- 产品编号
- bs-23501R-AF555
- 英文名称
- Anti-PGAM2/AF555
- 中文名称
- AF555标记的PGAM2抗体
- 别 名
- BPG dependent PGAM 2;BPG-dependent PGAM 2;GSD10;MGC88743;Muscle specific phosphoglycerate mutase;Muscle-specific phosphoglycerate mutase;OTTHUMP00000207787;PGAM 2;PGAM M;PGAM-M;Pgam2;PGAM2_HUMAN;PGAMM.
- 规格价格
- 100ul/2980元购买 大包装/询价
- 说 明 书
- 100ul
- 研究领域
- 抗体来源
- Rabbit
- 克隆类型
- Polyclonal
- 交叉反应
- Human, Mouse, Rat, Dog, Pig, Cow, Horse, Rabbit, Sheep,
- 产品应用
- ICC=1:50-200 IF=1:50-200
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
- 分 子 量
- 29kDa
- 性 状
- Lyophilized or Liquid
- 浓 度
- 2mg/1ml
- 免 疫 原
- KLH conjugated synthetic peptide derived from human PGAM2
- 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.
- 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:
Phosphoglycerate mutase (PGAM) catalyzes the reversible reaction of 3-phosphoglycerate (3-PGA) to 2-phosphoglycerate (2-PGA) in the glycolytic pathway. The PGAM is a dimeric enzyme containing, in different tissues, different proportions of a slow-migrating muscle (MM) isozyme, a fast-migrating brain (BB) isozyme, and a hybrid form (MB). This gene encodes muscle-specific PGAM subunit. Mutations in this gene cause muscle phosphoglycerate mutase eficiency, also known as glycogen storage disease X. [provided by RefSeq, Sep 2009]
Function:
Interconversion of 3- and 2-phosphoglycerate with 2,3-bisphosphoglycerate as the primer of the reaction. Can also catalyze the reaction of EC 5.4.2.4 (synthase) and EC 3.1.3.13 (phosphatase), but with a reduced activity.
Tissue Specificity:
In mammalian tissues there are two types of phosphoglycerate mutase isozymes: type-M in muscles and type-B in other tissues.
DISEASE:
Defects in PGAM2 are the cause of glycogen storage disease type 10 (GSD10) [MIM:261670]. A metabolic disorder characterized by myoglobinuria, increased serum creatine kinase levels, decreased phosphoglycerate mutase activity, myalgia, muscle pain, muscle cramps and excercise intolerance.
Similarity:
Belongs to the phosphoglycerate mutase family. BPG-dependent PGAM subfamily.
Database links:Entrez Gene: 515067 Cow
Entrez Gene: 5224 Human
Entrez Gene: 56012 Mouse
Entrez Gene: 100188980 Pig
Entrez Gene: 24959 Rat
Omim: 612931 Human
SwissProt: Q32KV0 Cow
SwissProt: P15259 Human
SwissProt: O70250 Mouse
SwissProt: P16290 Rat
Unigene: 23217 Cow
Unigene: 632642 Human
Unigene: 219627 Mouse
Unigene: 9738 Rat
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.