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Therefore, in addition to elucidating the mechanisms responsible for polyQ-mediated pathology, it is also important to understand the normal function of Htt protein for both basic biology and for HD.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      To systematically search for a mouse Htt function, we took advantage of the\n                      <jats:italic>Hdh<\/jats:italic>\n                      +\/- and\n                      <jats:italic>Hdh<\/jats:italic>\n                      -floxed mice and generated four mouse embryonic fibroblast (MEF) cells lines which contain a single copy of the\n                      <jats:italic>Hdh<\/jats:italic>\n                      gene (\n                      <jats:italic>Hdh<\/jats:italic>\n                      -HET) and four MEF lines in which the\n                      <jats:italic>Hdh<\/jats:italic>\n                      gene was deleted (\n                      <jats:italic>Hdh<\/jats:italic>\n                      -KO). The function of Htt in calcium (Ca\n                      <jats:sup>2+<\/jats:sup>\n                      ) signaling was analyzed in Ca\n                      <jats:sup>2+<\/jats:sup>\n                      imaging experiments with generated cell lines. We found that the cytoplasmic Ca\n                      <jats:sup>2+<\/jats:sup>\n                      spikes resulting from the activation of inositol 1,4,5-trisphosphate receptor (InsP\n                      <jats:sub>3<\/jats:sub>\n                      R) and the ensuing mitochondrial Ca\n                      <jats:sup>2+<\/jats:sup>\n                      signals were suppressed in the\n                      <jats:italic>Hdh<\/jats:italic>\n                      -KO cells when compared to\n                      <jats:italic>Hdh<\/jats:italic>\n                      -HET cells. Furthermore, in experiments with permeabilized cells we found that the InsP\n                      <jats:sub>3<\/jats:sub>\n                      -sensitivity of Ca\n                      <jats:sup>2+<\/jats:sup>\n                      mobilization from endoplasmic reticulum was reduced in\n                      <jats:italic>Hdh<\/jats:italic>\n                      -KO cells. These results indicated that Htt plays an important role in modulating InsP\n                      <jats:sub>3<\/jats:sub>\n                      R-mediated Ca\n                      <jats:sup>2+<\/jats:sup>\n                      signaling. To further evaluate function of Htt, we performed genome-wide transcription profiling of generated\n                      <jats:italic>Hdh<\/jats:italic>\n                      -HET and\n                      <jats:italic>Hdh<\/jats:italic>\n                      -KO cells by microarray. Our results revealed that 106 unique transcripts were downregulated by more than two-fold with p &lt; 0.05 and 173 unique transcripts were upregulated at least two-fold with p &lt; 0.05 in\n                      <jats:italic>Hdh<\/jats:italic>\n                      -KO cells when compared to\n                      <jats:italic>Hdh<\/jats:italic>\n                      -HET cells. The microarray results were confirmed by quantitative real-time PCR for a number of affected transcripts. Several signaling pathways affected by\n                      <jats:italic>Hdh<\/jats:italic>\n                      gene deletion were identified from annotation of the microarray results.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>\n                      Functional analysis of generated Htt-null MEF cells revealed that Htt plays a direct role in Ca\n                      <jats:sup>2+<\/jats:sup>\n                      signaling by modulating InsP\n                      <jats:sub>3<\/jats:sub>\n                      R sensitivity to InsP\n                      <jats:sub>3<\/jats:sub>\n                      . The genome-wide transcriptional profiling of Htt-null cells yielded novel and unique information about the normal function of Htt in cells, which may contribute to our understanding and treatment of HD.\n                    <\/jats:p>\n                  <\/jats:sec>","DOI":"10.1186\/1471-2202-9-38","type":"journal-article","created":{"date-parts":[[2008,4,16]],"date-time":"2008-04-16T02:13:30Z","timestamp":1208312010000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":31,"title":["Elucidating a normal function of huntingtin by functional and microarray analysis of huntingtin-null mouse embryonic fibroblasts"],"prefix":"10.1186","volume":"9","author":[{"given":"Hua","family":"Zhang","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sudipto","family":"Das","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Quan-Zhen","family":"Li","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ioannis","family":"Dragatsis","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Joyce","family":"Repa","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Scott","family":"Zeitlin","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gy\u00f6rgy","family":"Hajn\u00f3czky","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ilya","family":"Bezprozvanny","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2008,4,15]]},"reference":[{"key":"721_CR1","doi-asserted-by":"crossref","first-page":"pe48","DOI":"10.1126\/stke.2003.207.pe48","volume":"2003","author":"ME MacDonald","year":"2003","unstructured":"MacDonald ME: Huntingtin: alive and well and working in middle management. 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