ankyrin-2; brain ankyrin; ankyrin-B; non-erythroid ankyrin (ANK2)

Function: - attaches integral membrane proteins to cytoskeleton - binds to cytoskeletal proteins - required for coordinate assembly of Na⁺/Ca⁺² exchanger, Na⁺/K⁺ ATPase & insP3 receptor at sarcoplasmic reticulum sites in cardiomyocytes - required for the coordinated expression of the Na⁺/K⁺ ATPase, Na⁺/Ca⁺² exchanger & beta-2-spectrin (SPTBN1) in the inner segment of rod photoreceptors - required for expression & targeting of SPTBN1 in neonatal cardiomyocytes & for the regulation of neonatal cardiomyocyte contraction rate - phosphorylated at multiple sites by different protein kinases & each phosphorylation event regulates the protein's structure & function (putative) - interacts with RHBG & SPTBN1 Structure: - contains 24 ANK repeats - contains 1 death domain - contains 1 ZU5 domain Compartment: - cytoplasm, cytoskeleton, membrane - cytoplasm, myofibril, sarcomere, M-band (putative) - apical cell membrane (putative) - expressed at the apical membrane of airway lung epithelial cells - localized to the plasma membrane of the inner segments of photoreceptors in retina - colocalizes with SPTBN1 in a distict intracellular compartment of neonatal cardiomyocytes Alternative splicing: named isoforms=6 Expression: - present in plasma membrane of neurons as well as glial cells throughout the brain - expressed in fetal brain & in temporal cortex of adult brain - also expressed in the inner segments of rod photoreceptors in retina Pathology: - defects in ANK2 are the cause of long QT syndrome type 4

Specific

ankyrin-B1 ankyrin-B2

Properties

SIZE: entity length = 3924 aa MW = 430 kD COMPARTMENT: cytoplasm MOTIF: ankyrin repeat NAME: ankyrin repeat SITE: 30-62 MOTIF: Ser phosphorylation site {S31} Ser phosphorylation site {S34} ankyrin repeat NAME: ankyrin repeat SITE: 63-92 ankyrin repeat NAME: ankyrin repeat SITE: 96-125 ankyrin repeat NAME: ankyrin repeat SITE: 129-158 ankyrin repeat NAME: ankyrin repeat SITE: 162-191 ankyrin repeat NAME: ankyrin repeat SITE: 193-220 ankyrin repeat NAME: ankyrin repeat SITE: 232-261 ankyrin repeat NAME: ankyrin repeat SITE: 265-294 ankyrin repeat NAME: ankyrin repeat SITE: 298-327 ankyrin repeat NAME: ankyrin repeat SITE: 331-360 ankyrin repeat NAME: ankyrin repeat SITE: 364-393 MOTIF: Tyr phosphorylation site {Y378} ankyrin repeat NAME: ankyrin repeat SITE: 397-426 ankyrin repeat NAME: ankyrin repeat SITE: 430-459 ankyrin repeat NAME: ankyrin repeat SITE: 463-492 ankyrin repeat NAME: ankyrin repeat SITE: 496-525 ankyrin repeat NAME: ankyrin repeat SITE: 529-558 MOTIF: Tyr phosphorylation site {Y531} ankyrin repeat NAME: ankyrin repeat SITE: 562-591 ankyrin repeat NAME: ankyrin repeat SITE: 595-624 ankyrin repeat NAME: ankyrin repeat SITE: 628-657 ankyrin repeat NAME: ankyrin repeat SITE: 661-690 ankyrin repeat NAME: ankyrin repeat SITE: 694-723 ankyrin repeat NAME: ankyrin repeat SITE: 727-756 ankyrin repeat NAME: ankyrin repeat SITE: 760-789 ankyrin repeat NAME: ankyrin repeat SITE: 793-822 Ser phosphorylation site {S911} SPTBN1 interaction {966-1125} MOTIF: ZU5 {966-1073} Tyr phosphorylation site {Y1349} Ser phosphorylation site {S1428} Ser phosphorylation site {S1475} Thr phosphorylation site {T1490} Repeat-rich region {1773-1950} MOTIF: Repeat {1773-1784} Repeat {1785-1796} Repeat {1797-1808} Repeat {1809-1820} Repeat {1821-1832} Repeat {1833-1844} Repeat {1845-1856} consensus repeat {1857-1867} Repeat {1868-1879} Repeat {1880-1891} consensus repeat {1892-1902} Repeat {1903-1914} Repeat {1915-1926} Repeat {1927-1938} Repeat {1939-1950} Thr phosphorylation site {T2550} Thr phosphorylation site {T3060} Ser phosphorylation site {S3240} Ser phosphorylation site {S3329} Ser phosphorylation site {S3357} Ser phosphorylation site {S3376} Tyr phosphorylation site {Y3377} death domain NAME: death domain SITE: 3536-3620 Ser phosphorylation site {S3702} Thr phosphorylation site {T3743} Ser phosphorylation site {S3769} Thr phosphorylation site {T3770} Ser phosphorylation site {S3790} Thr phosphorylation site {T3811}

Database Correlations

OMIM correlations MORBIDMAP 106410 UniProt Q01484 PFAM correlations Entrez Gene 287