PFKM Rabbit Polyclonal Antibody
Konjugasyon: Unconjugated
Rabbit polyclonal Antibody
Uygulama
Reaktivite
Human,Mouse,Rat
Gen Adı
PFKM
Saklama
Aliquot and store at -20°C (valid for 12 months). Avoid freeze/thaw cycles.
Özet
| Ürün Adı | PFKM Rabbit Polyclonal Antibody |
| Açıklama | Rabbit polyclonal Antibody |
| Konak | Rabbit |
| Reaktivite | Human,Mouse,Rat |
| Konjugasyon | Unconjugated |
| Modifikasyon | Unmodified |
| İzotip | IgG |
| Klonalite | Polyclonal |
| Form | Liquid |
| Konsantrasyon | Unconjugated |
| Saklama | Aliquot and store at -20°C (valid for 12 months). Avoid freeze/thaw cycles. |
| Nakliye | Ice bags. |
| Tampon | Liquid in PBS containing 50% glycerol, 0.5% protective protein and 0.02% New type preservative N. |
| Saflaştırma | Affinity purification |
Antijen Bilgisi
| Gen Adı | PFKM |
| Alternatif İsimler | PFKM; PFKX; 6-phosphofructokinase; muscle type; Phosphofructo-1-kinase isozyme A; PFK-A; Phosphofructokinase-M; Phosphofructokinase 1; Phosphohexokinase |
| Gen Kimliği | 5213 |
| SwissProt Kimliği | P08237 |
| İmmünojen | The antiserum was produced against synthesized peptide derived from human PFK-1. AA range:320-369 |
Uygulama
| Uygulama | WB,IHC,ICC/IF,ELISA |
| Seyreltme Oranı | WB 1:500-1:2000,IHC 1:100-1:300,ICC/IF 1:50-1:200,ELISA 1:5000-1:20000 |
| Moleküler Ağırlık | 85kDa |
Araştırma Alanı
| Glycolysis / Gluconeogenesis;Pentose phosphate pathway;Fructose and mannose metabolism;Galactose metabolism; |
Arka Plan
| 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],catalytic activity:ATP + D-fructose 6-phosphate = ADP + D-fructose 1,6-bisphosphate.,cofactor:Magnesium.,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.,enzyme regulation:Allosteric enzyme activated by ADP, AMP, or fructose bisphosphate and inhibited by ATP or citrate.,miscellaneous:In human PFK exists as a system of 3 types of subunits, PFKM (muscle), PFKL (liver) and PFKP (platelet) isoenzymes.,pathway:Carbohydrate degradation; glycolysis; D-glyceraldehyde 3-phosphate and glycerone phosphate from D-glucose: step 3/4.,similarity:Belongs to the phosphofructokinase family. Two domains subfamily.,subunit:Tetramer. Muscle is M4, liver is L4, and red cell is M3L, M2L2, or ML3., |
