The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
Application notesDot: 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.
PathwayCarbohydrate degradation; glycolysis; D-glyceraldehyde 3-phosphate and glycerone phosphate from D-glucose: step 3/4.
Involvement in diseaseDefects 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.
Sequence similaritiesBelongs to the phosphofructokinase family. Two domains subfamily.