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Rabbit Anti-PGAM2/BF350 Conjugated antibody (bs-23502R-BF350)
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說(shuō) 明 書(shū): 100ul  
100ul/2980.00元
大包裝/詢(xún)價(jià)
產(chǎn)品編號(hào) bs-23502R-BF350
英文名稱(chēng)1 Rabbit Anti-PGAM2/BF350 Conjugated antibody
中文名稱(chēng) BF350標(biāo)記的PGAM2抗體
別    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.  
規(guī)格價(jià)格 100ul/2980元 購(gòu)買(mǎi)        大包裝/詢(xún)價(jià)
說(shuō) 明 書(shū) 100ul  
研究領(lǐng)域
抗體來(lái)源 Rabbit
克隆類(lèi)型 Polyclonal
交叉反應(yīng) (predicted: Human, Mouse, Rat, Dog, Pig, Cow, Horse, Rabbit, Sheep, )
產(chǎn)品應(yīng)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    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human PGAM2
亞    型 IgG
純化方法 affinity purified by Protein A
儲(chǔ) 存 液 Preservative: 15mM Sodium Azide, Constituents: 1% BSA, 0.01M PBS, pH 7.4.
保存條件 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.
產(chǎn)品介紹 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.
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