Rabbit Anti-PFKM/PFK1/FITC Conjugated antibody

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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;

Cat:

SL3982R-FITC

Species Reactivity：

(predicted: Human,Mouse,Rat,Chicken,Pig,Cow,)

Immunogen：

KLH conjugated synthetic peptide derived from human 6 Phosphofructokinase Muscle Type

Format：

Lyophilized or Liquid

Storage instructions：

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 ant

Buffer：

0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.

Concentration：

1mg/ml

Clonality：

Polyclonal

Isotype：

IgG

Applications：

IF=1:50-200not yet tested in other applications.optimal dilutions/concentrations should be determined by the end user.

Host：

Rabbit

Calculated MW：

86kDa

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Unit:

Price: $596.00

Product PDFs

Datasheet: Down Datasheet

Other Information
Citations
Protein Attributes
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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 SLCterminus) 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: 73721 Pig

Entrez Gene: 100345647 Rabbit

Entrez Gene: 65152 Rat

Omim: 610681 Human

SwissProt: Q0IIG5 Cow

SwissProt: P52784 Dog

SwissProt: Q867C9 Horse