BD-PT6905

KPB2 Rabbit Polyclonal Antibody

常规抗体

PHKA2 PHKLA PYK

KPB2

WB

Human,Mouse

1 mg/ml

Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% New type preservative N.

5256

https://www.uniprot.org/uniprot/5256

P46019

https://www.uniprot.org/uniprotkb/P46019/entry

110094

https://www.uniprot.org/uniprot/110094

Q8BWJ3

https://www.uniprot.org/uniprotkb/Q8BWJ3

Synthesized peptide derived from human KPB2 AA range: 911-961

This antibody detects endogenous levels of KPB2 at Human/Mouse

WB 1:500-2000;IHC-p 1:50-300

136kD

-20°C/1 year

Polyclonal, Rabbit,IgG

Phosphorylase kinase is a polymer of 16 subunits, four each of alpha, beta, gamma and delta. The alpha subunit includes the skeletal muscle and hepatic isoforms, and the hepatic isoform is encoded by this gene. The beta subunit is the same in both the muscle and hepatic isoforms, and encoded by one gene. The gamma subunit also includes the skeletal muscle and hepatic isoforms, which are encoded by two different genes. The delta subunit is a calmodulin and can be encoded by three different genes. The gamma subunits contain the active site of the enzyme, whereas the alpha and beta subunits have regulatory functions controlled by phosphorylation. The delta subunit mediates the dependence of the enzyme on calcium concentration. Mutations in this gene cause glycogen storage disease type 9A, also known as X-linked liver glycogenosis. Alternatively spliced transcript variants have been reported, but the full-length nature of these variants has not been determined.[provided by RefSeq, Feb 2010],

Predominantly expressed in liver and other non-muscle tissues.

Cell membrane ; Lipid-anchor ; Cytoplasmic side .

disease:Defects in PHKA2 are the cause of glycogen storage disease type 9A (GSD9A) [MIM:306000]; also known as X-linked liver glycogenosis (XLG). GSD9A is a metabolic disorder resulting in a mild glycogenosis with clinical symptoms that include hepatomegaly, growth retardation, muscle weakness, elevation of glutamate-pyruvate transaminase and glutamate-oxaloacetate transaminase, hypercholesterolemia, hypertriglyceridemia, and fasting hyperketosis. Two subtypes are known: type 1 or classic type, and type 2 or variant type. The variant type is characterized mainly by enlarged liver and growth retardation; patients do not show in vitro enzymatic deficiency of phosphorylase kinase. Unlike other glycogenosis diseases, GSD9A is generally a benign condition. Patients improve with age and are often asymptomatic as adults. Accurate diagnosis is therefore also of prognostic interest.,enzyme regulation:By phosphorylation of various serine residues and by calcium.,function:Phosphorylase b kinase catalyzes the phosphorylation of serine in certain substrates, including troponin I. The alpha chain may bind calmodulin.,pathway:Glycan biosynthesis; glycogen metabolism.,similarity:Belongs to the phosphorylase b kinase regulatory chain family.,subunit:Polymer of 16 chains, four each of alpha, beta, gamma, and delta. Alpha and beta are regulatory chains, gamma is the catalytic chain, and delta is calmodulin.,tissue specificity:Predominantly expressed in liver and other non-muscle tissues.,

现货

The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.