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Rabbit Anti-PFKM/PFK1/PE-Cy5 Conjugated antibody (bs-3982R-PE-Cy5)
~~~促销,代码KT202502A~~~
~~~促销,代码KT202502B~~~
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说 明 书: 100ul  
100ul/2980.00元
大包装/询价
产品编号 bs-3982R-PE-Cy5
英文名称 Rabbit Anti-PFKM/PFK1/PE-Cy5 Conjugated antibody
中文名称 PE-Cy5标记的肌肉型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.  
规格价格 100ul/2980元 购买        大包装/询价
说 明 书 100ul  
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 (predicted: Human, Mouse, Rat, Chicken, Pig, Cow, )
产品应用 IF=1:50-200 
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/ml
免 疫 原 KLH conjugated synthetic peptide derived from human 6 Phosphofructokinase Muscle Type
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:
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.

Database links:

Entrez Gene: 506544 Cow

Entrez Gene: 403849 Dog

Entrez Gene: 100034116 Horse

Entrez Gene: 5213 Human

Entrez Gene: 18642 Mouse

Entrez Gene: 733601 Pig

Entrez Gene: 100345647 Rabbit

Entrez Gene: 65152 Rat

Omim: 610681 Human

SwissProt: Q0IIG5 Cow

SwissProt: P52784 Dog

SwissProt: Q867C9 Horse

SwissProt: P08237 Human

SwissProt: P47857 Mouse

SwissProt: Q2HYU2 Pig

SwissProt: P00511 Rabbit

SwissProt: P47858 Rat

Unigene: 75160 Human

Unigene: 272582 Mouse

Unigene: 11004 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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