6磷酸果糖激酶抗体

英文名称    Fructose 6 Phosphate Kinase    

中文名称    6磷酸果糖激酶抗体    

别    名    Fructose 6 Phosphate Kinase 6 Phosphofructokinase Muscle Type; GSD7; PFKA; PFK-A; PFKL; PFKM; PFKP; PFKX; Phosphofructo 1 Kinase Isozyme A; Phosphofructo-1-kinase isozyme A; Phosphofructokinase 1; Phosphofructokinase M; Phosphofructokinase-M; Phosphofructokinase, muscle; Phosphofructokinase, muscle type; Phosphofructokinase, polypeptide X; Phosphohexokinase; K6PF_HUMAN; 6-phosphofructokinase, muscle type.    

说 明 书    0.1ml  0.2ml      

研究领域    肿瘤  细胞生物  免疫学  激酶和磷酸酶      

抗体来源    Rabbit    

克隆类型    Polyclonal    

交叉反应    Human, Mouse, Rat, Chicken, Pig, Cow,     

产品应用    WB=1:100-500 ELISA=1:500-1000 IP=1:20-100 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:100-500 6磷酸果糖激酶抗体（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.    

分 子 量    86kDa    

性    状    Lyophilized or Liquid    

浓    度    1mg/1ml    

免 疫 原    KLH conjugated synthetic peptide derived from human Fructose 6 Phosphate Kinase    

亚    型    IgG    

纯化方法    affinity purified by Protein A    

储 存 液    0.01M PBS, pH 7.4 with 10 mg/ml BSA and 0.1% Sodium azide    

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.    

6磷酸果糖激酶抗体产品介绍    background:

PFKL is the major form in liver and kidney while only M type PFK isozyme is expressed in mature muscle; therefore, muscle contains only homotetramers of M subunits. Erythrocytes contain both L and M subunits, and these randomly tetramerize to form M4, L4, and 3 additional hybrid forms of the holoenzyme.

Function:
Catalyzes the third step of glycolysis, the phosphorylation of fructose-6-phosphate (F6P) by ATP to generate fructose-1,6-bisphosphate (FBP) and ADP.

Subunit:
Tetramer. Muscle is M4, liver is L4, and red cell is M3L, M2L2, or ML3.

Post-translational modifications:
GlcNAcylation decreases enzyme activity (By similarity).

DISEASE:
Defects in PFKM are the cause of glycogen storage disease type 7 (GSD7) [MIM:232800]; also known as Tarui disease. GSD7 is an autosomal recessive disorder characterized by exercise intolerance with associated nausea and vomiting. Short bursts of intense activity are particularly difficult. Severe muscle cramps and myoglobinuria develop after vigorous exercise. Most patients obtain a 'second wind' when the onset of exercise is followed by a brief rest period. In time patients adjust their activity level and are well compensated.

Similarity:
Belongs to the phosphofructokinase family. Two domains subfamily.

Database links:
UniProtKB/Swiss-Prot: P08237.2

Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.    

6磷酸果糖激酶抗体

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