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
| 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: | PHKA2Â Â |
| NCBI Official Synonym Symbols: | PHK; PYK; XLG; PYKL; XLG2; GSD9AÂ Â |
| 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: | PHKA2Â Â |
| UniProt Entry Name: | KPB2_HUMAN |
 | 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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