Rabbit PHKA2 Polyclonal Antibody | anti-PHKA2 antibody

PHKA2 Polyclonal Antibody

Cat. Num. AAA2520323

• Gene Names: PHKA2; PHK; PYK; XLG; PYKL; XLG2; GSD9A
• Reactivity: Human, Mouse, Rat
• Applications: ELISA, Immunohistochemistry
• Purity: Antigen affinity purification
• Synonyms: PHKA2; Polyclonal Antibody; PHKA2 Polyclonal Antibody; PHK; PYK; XLG; PYKL; XLG2; GSD9A; anti-PHKA2 antibody

• Host: Rabbit
• Reactivity: Human, Mouse, Rat
• Clonality: Polyclonal
• Isotype: IgG
• Purity/Purification: Antigen affinity purification
• Concentration: 1.5mg/mL (varies by lot)
• Sequence Length: 1235

• Applicable Applications for anti-PHKA2 antibody: ELISA (EIA), Immunohistochemistry (IHC)
• Application Notes: IHC: 1:25-1:100
• Immunogen: Synthetic peptide of human PHKA2
• Buffer: PBS with 0.05% sodium azide, 50% glycerol, pH7.3
• Preparation and Storage: Store at -20 degree C (regular) and -80 degree C (long term). Avoid freeze / thaw cycles.

Immunohistochemistry (IHC)

(Immunohistochemistry of paraffin-embedded Human prostate cancer tissue using PHKA2 Polyclonal Antibody at dilution 1:30)

Immunohistochemistry (IHC)

(Immunohistochemistry of paraffin-embedded Human brain tissue using PHKA2 Polyclonal Antibody at dilution 1:30)

Related Product Information for anti-PHKA2 antibody

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.

NCBI and Uniprot Product Information

• NCBI GI #: 4505781
• NCBI GeneID: 5256
• NCBI Accession #: NP_000283.1
• NCBI GenBank Nucleotide #: NM_000292.2
• Molecular Weight: 138,408 Da
• NCBI Official Full Name: phosphorylase b kinase regulatory subunit alpha, liver isoform
• NCBI Official Synonym Full Names: phosphorylase kinase, alpha 2 (liver)
• NCBI Official Symbol: PHKA2
• NCBI Official Synonym Symbols: PHK; PYK; XLG; PYKL; XLG2; GSD9A
• NCBI Protein Information: phosphorylase b kinase regulatory subunit alpha, liver isoform; phosphorylase b kinase regulatory subunit alpha liver isoform; phosphorylase kinase alpha L subunit; phosphorylase kinase alpha-subunit
• UniProt Protein Name: Phosphorylase b kinase regulatory subunit alpha, liver isoform
• Protein Family: Phosphorylase b kinase regulatory
• UniProt Gene Name: PHKA2
• UniProt Synonym Gene Names: PHKLA; PYK; Phosphorylase kinase alpha L subunit
• UniProt Entry Name: KPB2_HUMAN

NCBI Description

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]

Uniprot Description

PHKA2: Phosphorylase b kinase catalyzes the phosphorylation of serine in certain substrates, including troponin I. The alpha chain may bind calmodulin. Defects in PHKA2 are the cause of glycogen storage disease type 9A (GSD9A); 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 with no phosphorylase kinase activity in liver or erythrocytes, and type 2 or variant type with no phosphorylase kinase activity in liver, but normal activity in erythrocytes. Unlike other glycogenosis diseases, glycogen storage disease type 9A is generally a benign condition. Patients improve with age and are often asymptomatic as adults. Accurate diagnosis is therefore also of prognostic interest. Belongs to the phosphorylase b kinase regulatory chain family.

Protein type: Protein kinase, regulatory subunit

Chromosomal Location of Human Ortholog: Xp22.2-p22.1

Cellular Component: plasma membrane; phosphorylase kinase complex; cytosol

Molecular Function: calmodulin binding; phosphorylase kinase activity; hydrolase activity, hydrolyzing O-glycosyl compounds

Biological Process: generation of precursor metabolites and energy; glycogen catabolic process; carbohydrate metabolic process; protein modification process; glucose metabolic process; pathogenesis; protein amino acid phosphorylation

Disease: Glycogen Storage Disease Ixa1