Mouse anti-Human PHKB Monoclonal Antibody | anti-PHKB antibody

PHKB (Phosphorylase B Kinase Regulatory Subunit beta, Phosphorylase Kinase Subunit beta, DKFZp781E15103, FLJ41698) (FITC)

Cat. Num. AAA6148827

• Reactivity: Human
• Applications: ELISA, Western Blot
• Purity: Purified by Protein A Affinity Chromatography.
• Synonyms: PHKB; Monoclonal Antibody; PHKB (Phosphorylase B Kinase Regulatory Subunit beta; Phosphorylase Kinase Subunit beta; DKFZp781E15103; FLJ41698) (FITC); anti-PHKB antibody

• Host: Mouse
• Reactivity: Human
• Clonality: Monoclonal
• Isotype: IgG2a,k
• Clone Number: 2E9
• Specificity: Recognizes human PHKB.
• Purity/Purification: Purified by Protein A Affinity Chromatography.
• Form/Format: Supplied as a liquid in PBS, pH 7.2. No preservative added. Labeled with Fluorescein isothiocyanate (FITC).
• Sequence Length: 1093

• Applicable Applications for anti-PHKB antibody: ELISA (EIA), Western Blot (WB)
• Application Notes: Applications are based on unconjugated antibody.
• Immunogen: Partial recombinant corresponding to aa984-1094 from human PHKB (NP_000284) with GST tag. MW of the GST tag alone is 26kD.
• Immunogen Sequence: EDTLGNIDQPQYRQIVVELLMVVSIVLERNPELEFQDKVDLDRLVKEAFNEFQKDQSRLKEIEKQDDMTSFYNTPPLGKRGTCSYLTKAVMNLLLEGEVKPNNDDPCLIS
• Conjugate: FITC
• Preparation and Storage: Store product at 4 degree C if to be used immediately within two weeks. For long-term storage, aliquot to avoid repeated freezing and thawing and store at -20 degree C. Aliquots are stable at -20 degree C for 12 months after receipt. Dilute required amount only prior to immediate use. Further dilutions can be made in assay buffer. Caution: FITC conjugates are sensitive to light. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

Western Blot (WB)

(Western Blot detection against Immunogen (37.84kD).)

Related Product Information for anti-PHKB antibody

Phosphorylase b kinase catalyzes the phosphorylation of serine in certain substrates, including troponin I. The beta chain acts as a regulatory unit and modulates the activity of the holoenzyme in response to phosphorylation.

Product Categories/Family for anti-PHKB antibody

Antibodies; Protein Kinases

NCBI and Uniprot Product Information

• NCBI GI #: 4505783
• NCBI GeneID: 5257
• NCBI Accession #: NP_000284
• NCBI GenBank Nucleotide #: NM_000293
• UniProt Accession #: Q93100
• NCBI Official Full Name: phosphorylase b kinase regulatory subunit beta isoform a
• NCBI Official Synonym Full Names: phosphorylase kinase regulatory subunit beta
• NCBI Official Symbol: PHKB
• NCBI Protein Information: phosphorylase b kinase regulatory subunit beta
• UniProt Protein Name: Phosphorylase b kinase regulatory subunit beta
• Protein Family: Phosphorylase b kinase regulatory
• UniProt Gene Name: PHKB
• UniProt Synonym Gene Names: Phosphorylase kinase subunit beta
• UniProt Entry Name: KPBB_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, encoded by two different genes. The beta subunit is the same in both the muscle and hepatic isoforms, encoded by this gene, which is a member of the phosphorylase b kinase regulatory subunit family. The gamma subunit also includes the skeletal muscle and hepatic isoforms, 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 9B, also known as phosphorylase kinase deficiency of liver and muscle. Alternatively spliced transcript variants encoding different isoforms have been identified in this gene. Two pseudogenes have been found on chromosomes 14 and 20, respectively.[provided by RefSeq, Feb 2010]

Uniprot Description

PHKB: Phosphorylase b kinase catalyzes the phosphorylation of serine in certain substrates, including troponin I. The beta chain acts as a regulatory unit and modulates the activity of the holoenzyme in response to phosphorylation. Defects in PHKB are the cause of glycogen storage disease type 9B (GSD9B); also known as phosphorylase kinase deficiency of liver and muscle (PKD). GSD9B is a metabolic disorder characterized by hepathomegaly, only slightly elevated transaminases and plasma lipids, clinical improvement with increasing age, and remarkably no clinical muscle involvement. Biochemical observations suggest that this mild phenotype is caused by an incomplete holoenzyme that lacks the beta subunit, but that may possess residual activity. Belongs to the phosphorylase b kinase regulatory chain family. 4 isoforms of the human protein are produced by alternative splicing.

Protein type: Protein kinase, regulatory subunit

Chromosomal Location of Human Ortholog: 16q12-q13

Cellular Component: plasma membrane; phosphorylase kinase complex; cytosol

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

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

Disease: Glycogen Storage Disease Ixb