Metabolism Antibodies 3

Anti-PHKA2 Antibody (CAB9636)

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SKU:
CAB9636
Product Type:
Antibody
Applications:
WB
Reactivity:
Human
Host Species:
Rabbit
Isotype:
IgG
Research Area:
Metabolism

Description

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Antibody Name:Anti-PHKA2 Antibody
Antibody SKU:CAB9636
Antibody Size:20uL, 50uL, 100uL
Application:WB
Reactivity:Human
Host Species:Rabbit
Immunogen:Recombinant fusion protein containing a sequence corresponding to amino acids 620-780 of human PHKA2 (NP_000283.1).
Application:WB
Recommended Dilution:WB 1:200 - 1:2000
Reactivity:Human
Positive Samples:HepG2, HL-60, MCF7, HT-29, HeLa, U-87MG
Immunogen:Recombinant fusion protein containing a sequence corresponding to amino acids 620-780 of human PHKA2 (NP_000283.1).
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:DPDC DEKL FDNA SEGT FSPD SDSD LVGY LEDT CNQE SQDE LDHY INHL LQST SLRS YLPP LCKN TEDR HVFS AIHS TRDI LSVM AKAK GLEV PFVP MTLP TKVL SAHR KSLN LVDS PQPL LEKV PESD FQWP RDDH GDVD CEKL VEQL KDCS NLQD QADI L
Gene ID:5256
Uniprot:P46019
Cellular Location:Cell membrane, Cytoplasmic side, Lipid-anchor
Calculated MW:138kDa
Observed MW:138kDa
Synonyms:PHKA2, GSD9A, PHK, PYK, PYKL, XLG, XLG2
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, 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.
UniProt Protein Function: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.
UniProt Protein Details:

Protein type:Protein kinase, regulatory subunit

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

Cellular Component: cytosol

Biological Process: carbohydrate metabolic process; generation of precursor metabolites and energy; glycogen catabolic process; protein modification process

Disease: Glycogen Storage Disease Ixa1

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, 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 Code:P46019
NCBI GenInfo Identifier:1170685
NCBI Gene ID:5256
NCBI Accession:P46019.1
UniProt Secondary Accession:P46019,Q6LAJ5, Q7Z6W0, Q96CR3, Q9UDA1, A8K1T1,
UniProt Related Accession:P46019
Molecular Weight:138,408 Da
NCBI Full Name:Phosphorylase b kinase regulatory subunit alpha, liver isoform
NCBI Synonym Full Names:phosphorylase kinase regulatory subunit alpha 2
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
UniProt Protein Name:Phosphorylase b kinase regulatory subunit alpha, liver isoform
Protein Family:Phosphorylase b kinase regulatory
UniProt Gene Name:PHKA2  
UniProt Entry Name:KPB2_HUMAN
Anti-PHKA2 Polyclonal Antibody (CAB9636)Western blot analysis of extracts of various cell lines, using PHKA2 antibody (CAB9636) 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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