产品货号 :  mlR3982

英文名称 :  PFKM/PFK1

中文名称 :  肌肉型6磷酸果糖激酶/磷酸果糖激酶1抗体

别    名 :  Fructose 6 Phosphate Kinase; 6 Phosphofructokinase Muscle Type; GSD7; PFKA; PFK-A; 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; PFKAM_HUMAN; 6-phosphofructokinase, muscle type; PFK1; PFK-1; ATP-PFK; PPP1R122.  

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=1:500-2000 ELISA=1:500-1000 IHC-P=1:400-800 IHC-F=1:400-800 IF=1:100-500 （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

细胞定位 :  细胞浆

性    状 :  Lyophilized or Liquid

浓    度 :  1mg/ml

免 疫 原 :  KLH conjugated synthetic peptide derived from human 6 Phosphofructokinase Muscle Type:701-780/780

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.

PubMed :  PubMed

产品介绍background:

Three phosphofructokinase isozymes exist in humans: muscle, liver and platelet. These isozymes function as subunits of the mammalian tetramer phosphofructokinase, which catalyzes the phosphorylation of fructose-6-phosphate to fructose-1,6-bisphosphate. Tetramer composition varies depending on tissue type. This gene encodes the muscle-type isozyme. Mutations in this gene have been associated with glycogen storage disease type VII, also known as Tarui disease. Alternatively spliced transcript variants have been described.[provided by RefSeq, Nov 2009]

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:

Homo- and heterotetramers. Muscle is M4, liver is L4, and red cell is M3L, M2L2, or ML3. Interacts (via C-terminus) with HK1 (via N-terminal spermatogenic cell-specific region)

Subcellular Location:

Cytoplasm.

Post-translational modifications:

GlcNAcylation decreases enzyme activity.

DISEASE:

Glycogen storage disease 7 (GSD7) [MIM:232800]: A metabolic disorder characterized by exercise intolerance with associated nausea and vomiting, muscle cramping, exertional myopathy and compensated hemolysis. Short bursts of intense activity are particularly difficult. Severe muscle cramps and myoglobinuria develop after vigorous exercise.

Similarity:

Belongs to the phosphofructokinase type A (PFKA) family. ATP-dependent PFK group I subfamily. Eukaryotic two domain clade 'E' sub-subfamily.

SWISS:

P08237

Gene ID:

5213

Important Note:

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