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PHKB, Polyclonal Antibody

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產(chǎn)品名稱: PHKB, Polyclonal Antibody
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PHKB, Polyclonal Antibody


PHKB, Polyclonal Antibody  的詳細(xì)介紹
Product Name

PHKB, Polyclonal Antibody

Popular Item
Full Product Name

PHKB Polyclonal Antibody

Product Gene Name

anti-PHKB antibody

[Similar Products]
Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
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OMIM
172490
3D Structure
ModBase 3D Structure for Q93100
Clonality
Polyclonal
Isotype
IgG
Host
Rabbit
Species Reactivity
Human, Mouse
Purity/Purification
Affinity Purification
Immunogen
Recombinant Protein
Immunogen
Recombinant protein of human PHKB
Preparation and Storage
Store at -20 degree C. Avoid freeze / thaw cycles.
Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
Other Notes
Small volumes of anti-PHKB antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.
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Related Product Information for
anti-PHKB 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, 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.
Applications Tested/Suitable for anti-PHKB antibody
Western Blot (WB)
Application Notes for anti-PHKB antibody
WB 1:500 - 1:2000

Western Blot (WB) of anti-PHKB antibody
Western blot analysis of extracts of various cell lines, using PHKB antibody at 1:1000 dilution.
Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (MBS128200) at 1:10000 dilution.
Lysates/proteins: 25ug per lane.
Blocking buffer: 3% nonfat dry milk in TBST.
Detection: ECL Basic Kit.
Exposure time: 5s.
anti-PHKB antibody Western Blot (WB) (WB) image
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NCBI/Uniprot data below describe general gene information for PHKB. It may not necessarily be applicable to this product.
NCBI GI #
4505783
NCBI GeneID
5257
NCBI Accession #
NP_000284.1 [Other Products]
NCBI GenBank Nucleotide #
NM_000293.2 [Other Products]
UniProt Primary Accession #
Q93100 [Other Products]
UniProt Secondary Accession #
Q8N4T5[Other Products]
UniProt Related Accession #
Q93100[Other Products]
Molecular Weight
125kDa
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NCBI Official Full Name
phosphorylase b kinase regulatory subunit beta isoform a
NCBI Official Synonym Full Names
phosphorylase kinase, beta
NCBI Official Symbol
PHKB??[Similar Products]
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??[Similar Products]
UniProt Synonym Gene Names
Phosphorylase kinase subunit beta??[Similar Products]
UniProt Entry Name
KPBB_HUMAN
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NCBI Summary for PHKB
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]
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UniProt Comments for PHKB
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
Research Articles on PHKB
1. Analysis showed that the glycogen phosphorylase kinase beta-subunit (PHKB) interacted with the C-terminal region of KIAA1199 protein.
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Precautions
All of MyBioSource's Products are for scientific laboratory research purposes and are not for diagnostic, therapeutics, prophylactic or in vivo use. Through your purchase, you expressly represent and warrant to MyBioSource that you will properly test and use any Products purchased from MyBioSource in accordance with industry standards. MyBioSource and its authorized distributors reserve the right to refuse to process any order where we reasonably believe that the intended use will fall outside of our acceptable guidelines.
Disclaimer
While every efforts were made to ensure the accuracy of the information provided in this datasheet, MyBioSource will not be liable for any omissions or errors contained herein. MyBioSource reserves the right to make changes to this datasheet at any time without prior notice.

It is the responsibility of the customer to report product performance issues to MyBioSource within 30 days of receipt of the product. Please visit our Terms & Conditions page for more information.
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