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Gene Review

ANK2  -  ankyrin 2, neuronal

Homo sapiens

Synonyms: ANK-2, Ankyrin-2, Ankyrin-B, Brain ankyrin, LQT4, ...
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Disease relevance of ANK2

  • Genotype-negative LQTS patients with a single ANK2 variant displayed nonexertional syncope, U waves, sinus bradycardia, and extracardiac findings [1].
  • Among these LQT models, the LQT3 and LQT4 mice exhibit spontaneous or exercise-induced life-threatening arrhythmias characteristics of long-QT patients [2].
  • Targeted mutational analysis of ankyrin-B in 541 consecutive, unrelated patients referred for long QT syndrome genetic testing and 200 healthy subjects [1].
  • Molecular cloning, expression, and localization of E1, an Onchocerca volvulus antigen with similarity to brain ankyrin [3].
  • RESULTS: In non-treated hearts subjected to ischemia (control hearts), reperfusion for 5 min severely reduced Na(+)/K(+)-ATPase activity and dissociated alpha(1) and alpha(2) subunits of Na(+)/K(+)-ATPase from the membrane-cytoskeleton complex in parallel with proteolysis of alpha-fodrin and ankyrin-B and calpain activation [4].

High impact information on ANK2

  • This report describes initial characterization of a 440-kD isoform of brain ankyrin (ankyrinB) representing an alternatively spliced mRNA product of the gene encoding the major isoform of ankyrin in adult human brain (Otto, E., M. Kunimoto, T. McLaughlin, V. Bennett, J. Cell Biology. 114:241-253) [5].
  • In contrast, COOH-terminal sequences of brain ankyrin 1 and 2 are distinct from one another and from human erythrocyte ankyrins, and thus are candidates to mediate protein interactions that distinguish these isoforms [6].
  • Moreover, an antibody raised against the conserved NH2-terminal domain of brain ankyrin cross-reacts with a single Mr = 220 kD polypeptide in adult human brain [6].
  • The brain ankyrin 2 cDNA sequence includes a stop codon and encodes a polypeptide with a predicted molecular mass of 202 kD, which is similar to the Mr of the major form of ankyrin in adult bovine brain membranes [6].
  • These results strongly suggest that the amino acid sequence of brain ankyrin 2 determined in this report represents the complete coding sequence of the major form of ankyrin in adult human brain [6].

Biological context of ANK2


Anatomical context of ANK2


Associations of ANK2 with chemical compounds

  • Sig-1R agonists such as pregnenolone sulfate and cocaine caused the dissociation of an ankyrin B isoform (ANK 220) from IP(3)R-3 [11].
  • In this study, we systematically generated alanine mutants of clusters of charged residues in the spectrin-binding domains of both ankyrin-B and -G [8].
  • Furthermore, conversion of amino acids EED(1597) to AAA(1597) leads to a loss of function in the localization of inositol 1,4,5-trisphosphate receptors in ankyrin-B mutant cardiomyocytes [13].
  • RESULTS: We identified a T to A transition mutation at position 4,603 in exon 40, resulting in the substitution of arginine for a tryptophan at amino acid residue 1,535 (W1535R) in the regulatory domain of 220-kD ankyrin-B, which is a highly conserved domain shared by different species [14].

Other interactions of ANK2


Analytical, diagnostic and therapeutic context of ANK2

  • Here we identify an intramolecular interaction between the C-terminal domain and the membrane-binding domain of ankyrin-B using pure proteins in solution and the yeast two-hybrid assay [13].
  • Physical properties of the ankyrin-B C-terminal domain determined by circular dichroism spectroscopy and hydrodynamic parameters reveal it is unstructured and highly extended in solution [13].


  1. Targeted mutational analysis of ankyrin-B in 541 consecutive, unrelated patients referred for long QT syndrome genetic testing and 200 healthy subjects. Sherman, J., Tester, D.J., Ackerman, M.J. Heart rhythm : the official journal of the Heart Rhythm Society. (2005) [Pubmed]
  2. Cardiac channelopathies: from men to mice. Charpentier, F., Demolombe, S., Escande, D. Ann. Med. (2004) [Pubmed]
  3. Molecular cloning, expression, and localization of E1, an Onchocerca volvulus antigen with similarity to brain ankyrin. Erttmann, K.D., Büttner, D.W., Gallin, M.Y. J. Biol. Chem. (1996) [Pubmed]
  4. Ischemic preconditioning prevents calpain-mediated impairment of Na(+)/K(+)-ATPase activity during early reperfusion. Inserte, J., Garcia-Dorado, D., Hernando, V., Barba, I., Soler-Soler, J. Cardiovasc. Res. (2006) [Pubmed]
  5. A new 440-kD isoform is the major ankyrin in neonatal rat brain. Kunimoto, M., Otto, E., Bennett, V. J. Cell Biol. (1991) [Pubmed]
  6. Isolation and characterization of cDNAs encoding human brain ankyrins reveal a family of alternatively spliced genes. Otto, E., Kunimoto, M., McLaughlin, T., Bennett, V. J. Cell Biol. (1991) [Pubmed]
  7. Isolation and chromosomal localization of a novel nonerythroid ankyrin gene. Tse, W.T., Menninger, J.C., Yang-Feng, T.L., Francke, U., Sahr, K.E., Lux, S.E., Ward, D.C., Forget, B.G. Genomics (1991) [Pubmed]
  8. Ankyrin-G and beta2-spectrin collaborate in biogenesis of lateral membrane of human bronchial epithelial cells. Kizhatil, K., Yoon, W., Mohler, P.J., Davis, L.H., Hoffman, J.A., Bennett, V. J. Biol. Chem. (2007) [Pubmed]
  9. Ankyrin and synapsin: spectrin-binding proteins associated with brain membranes. Bennett, V., Baines, A.J., Davis, J.Q. J. Cell. Biochem. (1985) [Pubmed]
  10. Vaccinia virus K1L protein supports viral replication in human and rabbit cells through a cell-type-specific set of its ankyrin repeat residues that are distinct from its binding site for ACAP2. Meng, X., Xiang, Y. Virology (2006) [Pubmed]
  11. Regulating ankyrin dynamics: Roles of sigma-1 receptors. Hayashi, T., Su, T.P. Proc. Natl. Acad. Sci. U.S.A. (2001) [Pubmed]
  12. Diversity in membrane binding sites of ankyrins. Brain ankyrin, erythrocyte ankyrin, and processed erythrocyte ankyrin associate with distinct sites in kidney microsomes. Davis, J., Davis, L., Bennett, V. J. Biol. Chem. (1989) [Pubmed]
  13. Isoform specificity of ankyrin-B: a site in the divergent C-terminal domain is required for intramolecular association. Abdi, K.M., Mohler, P.J., Davis, J.Q., Bennett, V. J. Biol. Chem. (2006) [Pubmed]
  14. Analysis of ankyrin-B gene mutations in patients with long QT syndrome. Zhou, X., Shimizu, M., Konno, T., Ino, H., Fujino, N., Uchiyama, K., Mabuchi, T., Kaneda, T., Fujita, T., Masuda, E., Kato, H., Funada, A., Mabuchi, H. Nan fang yi ke da xue xue bao = Journal of Southern Medical University. (2006) [Pubmed]
  15. Lateral membrane biogenesis in human bronchial epithelial cells requires 190-kDa ankyrin-G. Kizhatil, K., Bennett, V. J. Biol. Chem. (2004) [Pubmed]
  16. G protein-induced trafficking of voltage-dependent calcium channels. Tombler, E., Cabanilla, N.J., Carman, P., Permaul, N., Hall, J.J., Richman, R.W., Lee, J., Rodriguez, J., Felsenfeld, D.P., Hennigan, R.F., Diversé-Pierluissi, M.A. J. Biol. Chem. (2006) [Pubmed]
  17. MAP kinase pathway-dependent phosphorylation of the L1-CAM ankyrin binding site regulates neuronal growth. Whittard, J.D., Sakurai, T., Cassella, M.R., Gazdoiu, M., Felsenfeld, D.P. Mol. Biol. Cell (2006) [Pubmed]
  18. Major quantitative trait locus for resting heart rate maps to a region on chromosome 4. Martin, L.J., Comuzzie, A.G., Sonnenberg, G.E., Myklebust, J., James, R., Marks, J., Blangero, J., Kissebah, A.H. Hypertension (2004) [Pubmed]
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