Pyruvate Kinase, Liver And RBC (PK)
PKLR; PyK; PK1; PKL; Pyruvate Kinase Isozymes R/L; Glutamic--pyruvic transaminase 1; R-type/L-type pyruvate kinase; Red cell/liver pyruvate kinase
Pyruvate kinase is an enzyme involved in glycolysis. It catalyzes the transfer of a phosphate group from phosphoenolpyruvate (PEP) to ADP, yielding a pyruvate molecule and producing one molecule ATP.
Genetic defects of this enzyme cause the disease known as pyruvate kinase deficiency. In this condition, a lack of pyruvate kinase slows down the process of glycolysis. This effect is especially devastating in cells that lack mitochondria, because these cells must use anaerobic glycolysis as their sole source of energy because the TCA cycle is not available. One example is red blood cells, which in a state of pyruvate kinase deficiency rapidly become deficient in ATP and can undergo hemolysis. Therefore, pyruvate kinase deficiency can cause hemolytic anemia.
Organism species: Homo sapiens (Human)
| PRODUCT TYPE | CATALOG NO. | PRODUCT NAME | APPLICATIONS |
|---|---|---|---|
| Proteins | RPW536Hu01 | Recombinant Pyruvate Kinase, Liver And RBC (PK) | Positive Control; Immunogen; SDS-PAGE; WB. |
| Antibodies | MAW536Hu01 | Monoclonal Antibody to Pyruvate Kinase, Liver And RBC (PK) | WB; IHC; ICC; IP. |
| PAW536Hu01 | Polyclonal Antibody to Pyruvate Kinase, Liver And RBC (PK) | WB; IHC; ICC; IF. | |
| Assay Kits | CLW536Hu01 | CLIA Kit for Pyruvate Kinase, Liver And RBC (PK) | CLIA assay for Antigen Detection |
| ELW536Hu01 | ELISA Kit for Pyruvate Kinase, Liver And RBC (PK) | Enzyme-linked immunosorbent assay for Antigen Detection |
Organism species: Mus musculus (Mouse)
| PRODUCT TYPE | CATALOG NO. | PRODUCT NAME | APPLICATIONS |
|---|---|---|---|
| Proteins | RPW536Mu01 | Recombinant Pyruvate Kinase, Liver And RBC (PK) | Positive Control; Immunogen; SDS-PAGE; WB. |
| Antibodies | MAW536Mu01 | Monoclonal Antibody to Pyruvate Kinase, Liver And RBC (PK) | WB; IHC; ICC; IP. |
| PAW536Mu01 | Polyclonal Antibody to Pyruvate Kinase, Liver And RBC (PK) | WB; IHC; ICC; IF. | |
| Assay Kits | CLW536Mu01 | CLIA Kit for Pyruvate Kinase, Liver And RBC (PK) | CLIA assay for Antigen Detection |
| ELW536Mu01 | ELISA Kit for Pyruvate Kinase, Liver And RBC (PK) | Enzyme-linked immunosorbent assay for Antigen Detection |
Organism species: Rattus norvegicus (Rat)
| PRODUCT TYPE | CATALOG NO. | PRODUCT NAME | APPLICATIONS |
|---|---|---|---|
| Proteins | RPW536Ra01 | Recombinant Pyruvate Kinase, Liver And RBC (PK) | Positive Control; Immunogen; SDS-PAGE; WB. |
| Antibodies | MAW536Ra01 | Monoclonal Antibody to Pyruvate Kinase, Liver And RBC (PK) | WB; IHC; ICC; IP. |
| PAW536Ra01 | Polyclonal Antibody to Pyruvate Kinase, Liver And RBC (PK) | WB; IHC; ICC; IF. | |
| Assay Kits | CLW536Ra01 | CLIA Kit for Pyruvate Kinase, Liver And RBC (PK) | CLIA assay for Antigen Detection |
| ELW536Ra01 | ELISA Kit for Pyruvate Kinase, Liver And RBC (PK) | Enzyme-linked immunosorbent assay for Antigen Detection |
Organism species: Canis familiaris; Canine (Dog)
| PRODUCT TYPE | CATALOG NO. | PRODUCT NAME | APPLICATIONS |
|---|---|---|---|
| Proteins | RPW536Ca01 | Recombinant Pyruvate Kinase, Liver And RBC (PK) | Positive Control; Immunogen; SDS-PAGE; WB. |
| Antibodies | MAW536Ca01 | Monoclonal Antibody to Pyruvate Kinase, Liver And RBC (PK) | WB; IHC; ICC; IP. |
| PAW536Ca01 | Polyclonal Antibody to Pyruvate Kinase, Liver And RBC (PK) | WB; IHC; ICC; IF. | |
| Assay Kits | CLW536Ca01 | CLIA Kit for Pyruvate Kinase, Liver And RBC (PK) | CLIA assay for Antigen Detection |
| ELW536Ca01 | ELISA Kit for Pyruvate Kinase, Liver And RBC (PK) | Enzyme-linked immunosorbent assay for Antigen Detection |
Organism species: Sus scrofa; Porcine (Pig)
| PRODUCT TYPE | CATALOG NO. | PRODUCT NAME | APPLICATIONS |
|---|---|---|---|
| Proteins | RPW536Po01 | Recombinant Pyruvate Kinase, Liver And RBC (PK) | Positive Control; Immunogen; SDS-PAGE; WB. |
| Antibodies | MAW536Po01 | Monoclonal Antibody to Pyruvate Kinase, Liver And RBC (PK) | WB; IHC; ICC; IP. |
| PAW536Po01 | Polyclonal Antibody to Pyruvate Kinase, Liver And RBC (PK) | WB; IHC; ICC; IF. | |
| Assay Kits | CLW536Po01 | CLIA Kit for Pyruvate Kinase, Liver And RBC (PK) | CLIA assay for Antigen Detection |
| ELW536Po01 | ELISA Kit for Pyruvate Kinase, Liver And RBC (PK) | Enzyme-linked immunosorbent assay for Antigen Detection |
Organism species: Bos taurus; Bovine (Cattle)
| PRODUCT TYPE | CATALOG NO. | PRODUCT NAME | APPLICATIONS |
|---|---|---|---|
| Proteins | RPW536Bo01 | Recombinant Pyruvate Kinase, Liver And RBC (PK) | Positive Control; Immunogen; SDS-PAGE; WB. |
| Antibodies | MAW536Bo01 | Monoclonal Antibody to Pyruvate Kinase, Liver And RBC (PK) | WB; IHC; ICC; IP. |
| PAW536Bo01 | Polyclonal Antibody to Pyruvate Kinase, Liver And RBC (PK) | WB; IHC; ICC; IF. | |
| Assay Kits | CLW536Bo01 | CLIA Kit for Pyruvate Kinase, Liver And RBC (PK) | CLIA assay for Antigen Detection |
| ELW536Bo01 | ELISA Kit for Pyruvate Kinase, Liver And RBC (PK) | Enzyme-linked immunosorbent assay for Antigen Detection |
Organism species: Oryctolagus cuniculus (Rabbit)
| PRODUCT TYPE | CATALOG NO. | PRODUCT NAME | APPLICATIONS |
|---|---|---|---|
| Proteins | RPW536Rb01 | Recombinant Pyruvate Kinase, Liver And RBC (PK) | Positive Control; Immunogen; SDS-PAGE; WB. |
| Antibodies | MAW536Rb01 | Monoclonal Antibody to Pyruvate Kinase, Liver And RBC (PK) | WB; IHC; ICC; IP. |
| PAW536Rb01 | Polyclonal Antibody to Pyruvate Kinase, Liver And RBC (PK) | WB; IHC; ICC; IF. | |
| Assay Kits | CLW536Rb01 | CLIA Kit for Pyruvate Kinase, Liver And RBC (PK) | CLIA assay for Antigen Detection |
| ELW536Rb01 | ELISA Kit for Pyruvate Kinase, Liver And RBC (PK) | Enzyme-linked immunosorbent assay for Antigen Detection |
Organism species: Rhesus monkey (Simian)
| PRODUCT TYPE | CATALOG NO. | PRODUCT NAME | APPLICATIONS |
|---|---|---|---|
| Proteins | RPW536Mo01 | Recombinant Pyruvate Kinase, Liver And RBC (PK) | Positive Control; Immunogen; SDS-PAGE; WB. |
| Antibodies | MAW536Mo01 | Monoclonal Antibody to Pyruvate Kinase, Liver And RBC (PK) | WB; IHC; ICC; IP. |
| PAW536Mo01 | Polyclonal Antibody to Pyruvate Kinase, Liver And RBC (PK) | WB; IHC; ICC; IF. | |
| Assay Kits | CLW536Mo01 | CLIA Kit for Pyruvate Kinase, Liver And RBC (PK) | CLIA assay for Antigen Detection |
| ELW536Mo01 | ELISA Kit for Pyruvate Kinase, Liver And RBC (PK) | Enzyme-linked immunosorbent assay for Antigen Detection |
Reference
"cDNA cloning of human R-type pyruvate kinase and identification of a single amino acid substitution (Thr384-->Met) affecting enzymatic stability in a pyruvate kinase variant (PK Tokyo) associated with hereditary hemolytic anemia."
Proc. Natl. Acad. Sci. U.S.A. 88:8218-8221(1991) [PubMed] [Europe PMC] [Abstract]
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"Molecular basis of impaired pyruvate kinase isozyme conversion in erythroid cells: a single amino acid substitution near the active site and decreased mRNA content of the R-type PK."
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"Mutations in pyruvate kinase."
Hum. Mutat. 7:1-6(1996) [PubMed] [Europe PMC] [Abstract]
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Blood Cells Mol. Dis. 22:85-89(1996) [PubMed] [Europe PMC] [Abstract]
"Hematologically important mutations: red cell pyruvate kinase (1st update)."
Blood Cells Mol. Dis. 22:259-264(1996) [PubMed] [Europe PMC] [Abstract]
"Hematologically important mutations: red cell pyruvate kinase (2nd update)."
Blood Cells Mol. Dis. 24:273-279(1998) [PubMed] [Europe PMC] [Abstract]
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Blood Cells Mol. Dis. 26:47-53(2000) [PubMed] [Europe PMC] [Abstract]
"Initial characterization of the human central proteome."
BMC Syst. Biol. 5:17-17(2011) [PubMed] [Europe PMC] [Abstract]
"An enzyme assisted RP-RPLC approach for in-depth analysis of human liver phosphoproteome."
J. Proteomics 96:253-262(2014) [PubMed] [Europe PMC] [Abstract]
"Structure and function of human erythrocyte pyruvate kinase. Molecular basis of nonspherocytic hemolytic anemia."
J. Biol. Chem. 277:23807-23814(2002) [PubMed] [Europe PMC] [Abstract]
"Point mutations in the L-type pyruvate kinase gene of two children with hemolytic anemia caused by pyruvate kinase deficiency."
Blood 77:1871-1875(1991) [PubMed] [Europe PMC] [Abstract]
"Identical point mutations of the R-type pyruvate kinase (PK) cDNA found in unrelated PK variants associated with hereditary hemolytic anemia."
Blood 79:1347-1350(1992) [PubMed] [Europe PMC] [Abstract]
"Low substrate affinity of pyruvate kinase variant (PK Sapporo) caused by a single amino acid substitution (426 Arg-->Gln) associated with hereditary hemolytic anemia."
Blood 81:2439-2441(1993) [PubMed] [Europe PMC] [Abstract]
"Analysis of pyruvate kinase-deficiency mutations that produce nonspherocytic hemolytic anemia."
Proc. Natl. Acad. Sci. U.S.A. 90:4324-4327(1993) [PubMed] [Europe PMC] [Abstract]
"Molecular abnormality of erythrocyte pyruvate kinase deficiency in the Amish."
Blood 83:2311-2316(1994) [PubMed] [Europe PMC] [Abstract]
"Mutations in the pyruvate kinase L gene in patients with hereditary hemolytic anemia."
Blood 83:2817-2822(1994) [PubMed] [Europe PMC] [Abstract]
"Molecular study of pyruvate kinase deficient patients with hereditary nonspherocytic hemolytic anemia."
J. Clin. Invest. 95:1702-1709(1995) [PubMed] [Europe PMC] [Abstract]
"Study of the molecular defects in pyruvate kinase (PK) deficient patients affected by hereditary nonspherocytic hemolytic anemia (HNHA)."
J. Invest. Med. 43:341A-341A(1995)
"G-to-T transition at cDNA nt 110 (K37Q) in the PKLR (pyruvate kinase) gene is the molecular basis of a case of hereditary increase of red blood cell ATP."
Hum. Mutat. 9:282-285(1997) [PubMed] [Europe PMC] [Abstract]
"Molecular characterization of the PK-LR gene in pyruvate kinase deficient Spanish patients."
Br. J. Haematol. 103:377-382(1998) [PubMed] [Europe PMC] [Abstract]
"A new sickle cell disease phenotype associating Hb S trait, severe pyruvate kinase deficiency (PK Conakry), and an alpha-2 globin gene variant (Hb Conakry)."
Br. J. Haematol. 103:950-956(1998) [PubMed] [Europe PMC] [Abstract]
"Novel mutations and structural implications in R-type pyruvate kinase-deficient patients from Southern Italy."
Hum. Mutat. 11:127-134(1998) [PubMed] [Europe PMC] [Abstract]
"Molecular characterization of the PK-LR gene in sixteen pyruvate kinase-deficient patients."
Br. J. Haematol. 113:43-48(2001) [PubMed] [Europe PMC] [Abstract]
"Fifteen novel mutations in PKLR associated with pyruvate kinase (PK) deficiency: structural implications of amino acid substitutions in PK."
Hum. Mutat. 30:446-453(2009) [PubMed] [Europe PMC] [Abstract]
"Exome sequencing and unrelated findings in the context of complex disease research: ethical and clinical implications."
Discov. Med. 12:41-55(2011) [PubMed] [Europe PMC] [Abstract]