The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
追加情報Dot: Use at an assay dependent dilution.
ELISA: 1/1,000 - 1/3,500. Assayed against 1.0 ug of Fructose-6-Phosphate Kinase
(from Rabbit Muscle) in a standard capture ELISA using ABTS (2,2'-azino-bis-[3-ethylbenthiazoline-6-sulfonic acid]) as a substrate for 30 minutes at room temperature.
IM: Use at an assay dependent dilution.
WB: Use at an assay dependent dilution. Predicted molecular weight: 85 kDa.
Not yet tested in other applications.
Optimal dilutions/concentrations should be determined by the end user.
パスウェイCarbohydrate degradation; glycolysis; D-glyceraldehyde 3-phosphate and glycerone phosphate from D-glucose: step 3/4.
関連疾患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.
配列類似性Belongs to the phosphofructokinase family. Two domains subfamily.