Metabolism Antibodies 2

Anti-PHKB Antibody (CAB8015)

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SKU:
CAB8015
Product Type:
Antibody
Applications:
WB
Reactivity:
Human
Reactivity:
Mouse
Host Species:
Rabbit
Isotype:
IgG
Research Area:
Metabolism

Description

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Antibody Name:PHKB Rabbit Polyclonal Antibody
Antibody SKU:CAB8015
Antibody Size:20uL, 50uL, 100uL
Application:WB
Reactivity:Human, Mouse
Host Species:Rabbit
Immunogen:Recombinant fusion protein containing a sequence corresponding to amino acids 854-1093 of human PHKB (NP_000284.1).
Application:WB
Recommended Dilution:WB 1:500 - 1:2000
Reactivity:Human, Mouse
Positive Samples:HepG2, SW620, Jurkat, BT-474
Immunogen:Recombinant fusion protein containing a sequence corresponding to amino acids 854-1093 of human PHKB (NP_000284.1).
Purification Method:Affinity purification
Storage Buffer:Store at -20'C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
Isotype:IgG
Sequence:VIHI GWII SNNP ELFS GMLK IRIG WIIH AMEY ELQI RGGD KPAL DLYQ LSPS EVKQ LLLD ILQP QQNG RCWL NRRQ IDGS LNRT PTGF YDRV WQIL ERTP NGII VAGK HLPQ QPTL SDMT MYEM NFSL LVED TLGN IDQP QYRQ IVVE LLMV VSIV LERN PELE FQDK VDLD RLVK EAFN EFQK DQSR LKEI EKQD DMTS FYNT PPLG KRGT CSYL TKAV MNLL LEGE VKPN NDDP CLIS
Gene ID:5257
Uniprot:Q93100
Cellular Location:Cell membrane, Cytoplasmic side, Lipid-anchor
Calculated MW:123kDa/124kDa
Observed MW:125kDa
Synonyms:PHKB
Background: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.
UniProt Protein Function: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.
UniProt Protein Details:

Protein type:Protein kinase, regulatory subunit

Chromosomal Location of Human Ortholog: 16q12.1

Cellular Component: cytosol; phosphorylase kinase complex; plasma membrane

Molecular Function:calmodulin binding; phosphorylase kinase activity; protein binding

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

Disease: Glycogen Storage Disease Ixb

NCBI Summary: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 Code:Q93100
NCBI GenInfo Identifier:4505783
NCBI Gene ID:5257
NCBI Accession:NP_000284.1
UniProt Secondary Accession:Q93100,Q8N4T5,
UniProt Related Accession:Q93100
Molecular Weight:124 kDa
NCBI Full Name:phosphorylase b kinase regulatory subunit beta isoform a
NCBI 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
UniProt Gene Name:PHKB  
Anti-PHKB Polyclonal Antibody (CAB8015)Western blot analysis of extracts of various cell lines, using PHKB antibody (CAB8015) at 1:1000 dilution. Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (CABS014) at 1:10000 dilution. Lysates/proteins: 25ug per lane. Blocking buffer: 3% nonfat dry milk in TBST. Detection: ECL Basic Kit (RM00020). Exposure time: 5s.
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