{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,2]],"date-time":"2026-06-02T12:58:55Z","timestamp":1780405135018,"version":"3.54.1"},"reference-count":33,"publisher":"Wiley","issue":"9","license":[{"start":{"date-parts":[[2017,6,19]],"date-time":"2017-06-19T00:00:00Z","timestamp":1497830400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Protein Science"],"published-print":{"date-parts":[[2017,9]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>WBSCR16 (Williams\u2010Beuren Syndrome Chromosomal Region 16) gene is located in a large deletion region of Williams\u2010Beuren syndrome (WBS), which is a neurodevelopmental disorder. Although the relationship between WBSCR16 and WBS remains unclear, it has been reported that WBSCR16 is a member of a functional module that regulates mitochondrial 16S rRNA abundance and intra\u2010mitochondrial translation. WBSCR16 has RCC1 (Regulator of Chromosome Condensation 1)\u2010like amino acid sequence repeats but the function of WBSCR16 appears to be different from that of other RCC1 superfamily members. Here, we demonstrate that WBSCR16 localizes to mitochondria in HeLa cells, and report the crystal structure of WBSCR16 determined to 2.0 \u00c5 resolution using multi\u2010wavelength anomalous diffraction. WBSCR16 adopts the seven\u2010bladed \u03b2\u2010propeller fold characteristic of RCC1\u2010like proteins. A comparison of the WBSCR16 structure with that of RCC1 and other RCC1\u2010like proteins reveals that, although many of the residues buried in the core of the \u03b2\u2010propeller are highly conserved, the surface residues are poorly conserved and conformationally divergent.<\/jats:p>","DOI":"10.1002\/pro.3210","type":"journal-article","created":{"date-parts":[[2017,6,13]],"date-time":"2017-06-13T01:02:57Z","timestamp":1497315777000},"page":"1870-1877","source":"Crossref","is-referenced-by-count":8,"title":["Crystal structure of human WBSCR16, an RCC1\u2010like protein in mitochondria"],"prefix":"10.1002","volume":"26","author":[{"given":"Masako","family":"Koyama","sequence":"first","affiliation":[{"name":"Division of Biological Science Nagoya University Furo\u2010cho Chikusa\u2010ku Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Taeko","family":"Sasaki","sequence":"additional","affiliation":[{"name":"Division of Biological Science Nagoya University Furo\u2010cho Chikusa\u2010ku Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Narie","family":"Sasaki","sequence":"additional","affiliation":[{"name":"Division of Biological Science Nagoya University Furo\u2010cho Chikusa\u2010ku Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yoshiyuki","family":"Matsuura","sequence":"additional","affiliation":[{"name":"Division of Biological Science Nagoya University Furo\u2010cho Chikusa\u2010ku Japan"},{"name":"Structural Biology Research Center, Graduate School of Science, Nagoya University Furo\u2010cho Chikusa\u2010ku Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2017,6,19]]},"reference":[{"key":"e_1_2_6_2_1","doi-asserted-by":"publisher","DOI":"10.1146\/annurev-biochem-060815-014334"},{"key":"e_1_2_6_3_1","doi-asserted-by":"publisher","DOI":"10.1146\/annurev-biochem-060815-014343"},{"key":"e_1_2_6_4_1","doi-asserted-by":"publisher","DOI":"10.1080\/15384101.2015.1053672"},{"key":"e_1_2_6_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.cmet.2016.08.017"},{"key":"e_1_2_6_6_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00439-002-0710-x"},{"key":"e_1_2_6_7_1","first-page":"799","article-title":"Williams\u2010Beuren syndrome: Determination of deletion size using quantitative real\u2010time PCR","volume":"18","author":"Schubert C","year":"2006","journal-title":"Int J Mol Med"},{"key":"e_1_2_6_8_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.bbamcr.2008.03.015"},{"key":"e_1_2_6_9_1","doi-asserted-by":"publisher","DOI":"10.1038\/354080a0"},{"key":"e_1_2_6_10_1","doi-asserted-by":"publisher","DOI":"10.1083\/jcb.109.4.1389"},{"key":"e_1_2_6_11_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0960-9822(02)01076-X"},{"key":"e_1_2_6_12_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0960-9822(02)00970-3"},{"key":"e_1_2_6_13_1","doi-asserted-by":"publisher","DOI":"10.1038\/nprot.2007.131"},{"key":"e_1_2_6_14_1","doi-asserted-by":"publisher","DOI":"10.1038\/32204"},{"key":"e_1_2_6_15_1","doi-asserted-by":"publisher","DOI":"10.1093\/nar\/gkq366"},{"key":"e_1_2_6_16_1","doi-asserted-by":"publisher","DOI":"10.1007\/s13238-011-1018-1"},{"key":"e_1_2_6_17_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.94.1.65"},{"key":"e_1_2_6_18_1","doi-asserted-by":"publisher","DOI":"10.1093\/nar\/gku316"},{"key":"e_1_2_6_19_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0092-8674(01)00315-4"},{"key":"e_1_2_6_20_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jsb.2010.11.016"},{"key":"e_1_2_6_21_1","doi-asserted-by":"publisher","DOI":"10.1038\/embor.2013.37"},{"key":"e_1_2_6_22_1","doi-asserted-by":"publisher","DOI":"10.1093\/nar\/gkq399"},{"key":"e_1_2_6_23_1","doi-asserted-by":"publisher","DOI":"10.1006\/jmbi.1993.1626"},{"key":"e_1_2_6_24_1","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M607279200"},{"key":"e_1_2_6_25_1","doi-asserted-by":"publisher","DOI":"10.1038\/nature03144"},{"key":"e_1_2_6_26_1","doi-asserted-by":"publisher","DOI":"10.1006\/jmbi.1993.1012"},{"key":"e_1_2_6_27_1","doi-asserted-by":"publisher","DOI":"10.1107\/S0907444910045749"},{"key":"e_1_2_6_28_1","first-page":"331","article-title":"Recent advances in the CRANK software suite for experimental phasing","volume":"67","author":"Pannu NS","year":"2011","journal-title":"Acta Cryst"},{"key":"e_1_2_6_29_1","first-page":"30","article-title":"Methods used in the structure determination of bovine mitochondrial F1 ATPase","volume":"52","author":"Abrahams JP","year":"1996","journal-title":"Acta Cryst"},{"key":"e_1_2_6_30_1","doi-asserted-by":"publisher","DOI":"10.1107\/S0907444906022116"},{"key":"e_1_2_6_31_1","doi-asserted-by":"publisher","DOI":"10.1107\/S0907444904019158"},{"key":"e_1_2_6_32_1","doi-asserted-by":"publisher","DOI":"10.1107\/S0907444909052925"},{"key":"e_1_2_6_33_1","doi-asserted-by":"publisher","DOI":"10.1107\/S0907444909042073"},{"key":"e_1_2_6_34_1","first-page":"386","article-title":"Presenting your structures: The CCP4mg molecular\u2010graphics software","volume":"67","author":"McNicholas S","year":"2011","journal-title":"Acta Cryst"}],"container-title":["Protein Science"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Fpro.3210","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/pro.3210","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,14]],"date-time":"2023-09-14T16:44:32Z","timestamp":1694709872000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/pro.3210"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2017,6,19]]},"references-count":33,"journal-issue":{"issue":"9","published-print":{"date-parts":[[2017,9]]}},"alternative-id":["10.1002\/pro.3210"],"URL":"https:\/\/doi.org\/10.1002\/pro.3210","archive":["Portico"],"relation":{},"ISSN":["0961-8368","1469-896X"],"issn-type":[{"value":"0961-8368","type":"print"},{"value":"1469-896X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2017,6,19]]}}}