ID NOVA2_HUMAN STANDARD; PRT; 492 AA. AC Q9UNW9; O43267; Q9UEA1; DT 04-AUG-2003, integrated into UniProtKB/Swiss-Prot. DT 01-MAY-2000, sequence version 1. DT 19-SEP-2006, entry version 36. DE RNA-binding protein Nova-2 (Neuro-oncological ventral antigen 2) DE (Astrocytic NOVA1-like RNA-binding protein). GN Name=NOVA2; Synonyms=ANOVA, NOVA3; OS Homo sapiens (Human). OC Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi; OC Mammalia; Eutheria; Euarchontoglires; Primates; Haplorrhini; OC Catarrhini; Hominidae; Homo. OX NCBI_TaxID=9606; RN [1] RP NUCLEOTIDE SEQUENCE [MRNA]. RC TISSUE=Brain; RX MEDLINE=20197319; PubMed=10735272; DOI=10.1007/s100480050005; RA Ueki K., Ramaswamy S., Billings S.J., Mohrenweiser H.W., Louis D.N.; RT "ANOVA, a putative astrocytic RNA binding protein gene that maps to RT chromosome 19q13.3."; RL Neurogenetics 1:31-36(1997). RN [2] RP NUCLEOTIDE SEQUENCE [MRNA]. RX MEDLINE=99007301; PubMed=9789075; DOI=10.1073/pnas.95.22.13254; RA Yang Y.Y., Yin G.L., Darnell R.B.; RT "The neuronal RNA-binding protein Nova-2 is implicated as the RT autoantigen targeted in POMA patients with dementia."; RL Proc. Natl. Acad. Sci. U.S.A. 95:13254-13259(1998). RN [3] RP NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA]. RX PubMed=15057824; DOI=10.1038/nature02399; RA Grimwood J., Gordon L.A., Olsen A.S., Terry A., Schmutz J., RA Lamerdin J.E., Hellsten U., Goodstein D., Couronne O., Tran-Gyamfi M., RA Aerts A., Altherr M., Ashworth L., Bajorek E., Black S., Branscomb E., RA Caenepeel S., Carrano A.V., Caoile C., Chan Y.M., Christensen M., RA Cleland C.A., Copeland A., Dalin E., Dehal P., Denys M., Detter J.C., RA Escobar J., Flowers D., Fotopulos D., Garcia C., Georgescu A.M., RA Glavina T., Gomez M., Gonzales E., Groza M., Hammon N., Hawkins T., RA Haydu L., Ho I., Huang W., Israni S., Jett J., Kadner K., Kimball H., RA Kobayashi A., Larionov V., Leem S.-H., Lopez F., Lou Y., Lowry S., RA Malfatti S., Martinez D., McCready P.M., Medina C., Morgan J., RA Nelson K., Nolan M., Ovcharenko I., Pitluck S., Pollard M., RA Popkie A.P., Predki P., Quan G., Ramirez L., Rash S., Retterer J., RA Rodriguez A., Rogers S., Salamov A., Salazar A., She X., Smith D., RA Slezak T., Solovyev V., Thayer N., Tice H., Tsai M., Ustaszewska A., RA Vo N., Wagner M., Wheeler J., Wu K., Xie G., Yang J., Dubchak I., RA Furey T.S., DeJong P., Dickson M., Gordon D., Eichler E.E., RA Pennacchio L.A., Richardson P., Stubbs L., Rokhsar D.S., Myers R.M., RA Rubin E.M., Lucas S.M.; RT "The DNA sequence and biology of human chromosome 19."; RL Nature 428:529-535(2004). RN [4] RP X-RAY CRYSTALLOGRAPHY (2.0 ANGSTROMS) OF 405-480. RX MEDLINE=99148126; PubMed=10368286; DOI=10.1016/S0969-2126(99)80025-2; RA Lewis H.A., Chen H., Edo C., Buckanovich R.J., Yang Y.Y.-L., RA Musunuru K., Zhong R., Darnell R.B., Burley S.K.; RT "Crystal structures of Nova-1 and Nova-2 K-homology RNA-binding RT domains."; RL Structure 7:191-203(1999). RN [5] RP X-RAY CRYSTALLOGRAPHY (2.4 ANGSTROMS) OF 405-491 IN COMPLEX WITH RNA. RX MEDLINE=20139984; PubMed=10676814; DOI=10.1016/S0092-8674(00)80668-6; RA Lewis H.A., Musunuru K., Jensen K.B., Edo C., Chen H., Darnell R.B., RA Burley S.K.; RT "Sequence-specific RNA binding by a Nova KH domain: implications for RT paraneoplastic disease and the fragile X syndrome."; RL Cell 100:323-332(2000). CC -!- FUNCTION: May regulate RNA splicing or metabolism in a specific CC subset of developing neurons (By similarity). Binds single strand CC RNA. CC -!- SUBUNIT: Interacts with PTBP2 (By similarity). CC -!- SUBCELLULAR LOCATION: Nucleus (By similarity). CC -!- TISSUE SPECIFICITY: Brain. Expression restricted to astrocytes. CC -!- DOMAIN: The third KH domain (KH3) recognizes specifically 5'-UCAY- CC 3'. CC -!- DISEASE: Autoantigen in the paraneoplastic opsoclonus myoclonus CC ataxia (POMA), a paraneoplastic neurological syndrome/disorder CC (PNS/D) associated with breast cancer, fallopian cancer, and SCLCa CC and characterized primarily by loss of inhibitory control of motor CC neurons in the spinal cord and brainstem. Recognized by the IgG CC autoantibody ANNA-2 (also called anti-Ri). CC -!- SIMILARITY: Contains 3 KH domains. CC --------------------------------------------------------------------------- CC Copyrighted by the UniProt Consortium, see https://www.uniprot.org/terms CC Distributed under the Creative Commons Attribution (CC BY 4.0) License CC --------------------------------------------------------------------------- DR EMBL; U70477; AAB88661.1; ALT_INIT; mRNA. DR EMBL; AF083898; AAC72355.1; -; mRNA. DR EMBL; AC006540; AAD13116.1; -; Genomic_DNA. DR UniGene; Hs.375439; -. DR PDB; 1DTJ; X-ray; A/B/C/D=406-480. DR PDB; 1EC6; X-ray; A/B=406-491. DR Ensembl; ENSG00000104967; Homo sapiens. DR KEGG; hsa:4858; -. DR HGNC; HGNC:7887; NOVA2. DR MIM; 601991; gene. DR ArrayExpress; Q9UNW9; -. DR GO; GO:0003723; F:RNA binding; NAS. DR InterPro; IPR004087; KH. DR InterPro; IPR004088; KH_type_1. DR Pfam; PF00013; KH_1; 3. DR SMART; SM00322; KH; 3. DR PROSITE; PS50084; KH_TYPE_1; 3. KW 3D-structure; Nuclear protein; Repeat; RNA-binding. FT CHAIN 1 492 RNA-binding protein Nova-2. FT /FTId=PRO_0000050119. FT DOMAIN 32 99 KH 1. FT DOMAIN 130 196 KH 2. FT DOMAIN 406 473 KH 3. FT MOTIF 10 26 Bipartite nuclear localization signal FT (Potential). FT COMPBIAS 232 405 Ala-rich. FT COMPBIAS 332 393 Gly-rich. FT CONFLICT 247 247 A -> R (in Ref. 1). FT CONFLICT 265 267 PAA -> TAT (in Ref. 1). FT STRAND 407 414 FT TURN 415 417 FT HELIX 418 422 FT STRAND 424 424 FT TURN 425 426 FT HELIX 427 436 FT STRAND 439 442 FT TURN 445 446 FT STRAND 449 449 FT TURN 450 451 FT STRAND 454 462 FT HELIX 463 476 FT STRAND 487 487 SQ SEQUENCE 492 AA; 49009 MW; 41B63EAF6899256B CRC64; MEPEAPDSRK RPLETPPEVV CTKRSNTGEE GEYFLKVLIP SYAAGSIIGK GGQTIVQLQK ETGATIKLSK SKDFYPGTTE RVCLVQGTAE ALNAVHSFIA EKVREIPQAM TKPEVVNILQ PQTTMNPDRA KQAKLIVPNS TAGLIIGKGG ATVKAVMEQS GAWVQLSQKP EGINLQERVV TVSGEPEQVH KAVSAIVQKV QEDPQSSSCL NISYANVAGP VANSNPTGSP YASPADVLPA AAAASAAAAS GLLGPAGLAG VGAFPAALPA FSGTDLLAIS TALNTLASYG YNTNSLGLGL NSAAASGVLA AVAAGANPAA AAAANLLASY AGEAGAGPAG GAAPPPPPPP GALGSFALAA AANGYLGAGA GGGAGGGGGP LVAAAAAAGA AGGFLTAEKL AAESAKELVE IAVPENLVGA ILGKGGKTLV EYQELTGARI QISKKGEFLP GTRNRRVTIT GSPAATQAAQ YLISQRVTYE QGVRASNPQK VG //