{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,30]],"date-time":"2026-09-30T16:19:49Z","timestamp":1790785189787,"version":"4.1.0"},"reference-count":67,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2005,11,1]],"date-time":"2005-11-01T00:00:00Z","timestamp":1130803200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Glia"],"published-print":{"date-parts":[[2006,2]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>The <jats:italic>shaking pup<\/jats:italic> (<jats:italic>shp<\/jats:italic>) is a canine mutation that affects the myelin protein proteolipid protein (PLP) and its smaller and less abundant isoform, DM20, with proline replacing histidine<jats:sup>36<\/jats:sup>, resulting in a severe myelin deficiency in the central nervous system. We present evidence that the mutation leads to disrupted trafficking of the <jats:italic>shp<\/jats:italic> PLP\/DM20 within oligodendrocytes. Immunohistochemical studies revealed significantly reduced levels of PLP\/DM20 and other major myelin components such as myelin basic protein (MBP), myelin associated glycoprotein (MAG), and 2\u2032,3\u2032\u2010cyclic nucleotide 3\u2032\u2010phosphodiesterase (CNP) in <jats:italic>shp<\/jats:italic> myelin. The distribution of <jats:italic>shp<\/jats:italic> PLP\/DM20 proteins were altered and mostly retained in perinuclear cytoplasm and proximal processes, which co\u2010localized with distended rough endoplasmic reticulum (RER) within oligodendrocytes. No abnormal accumulation of MAG, MBP, or CNP in the cell body was found. These results suggest that mutated PLP\/DM20 in the <jats:italic>shp<\/jats:italic> could be selectively retained in RER, causing disruption of their translocation to the periphery to myelinate axons. \u00a9 2005 Wiley\u2010Liss, Inc.<\/jats:p>","DOI":"10.1002\/glia.20279","type":"journal-article","created":{"date-parts":[[2005,11,1]],"date-time":"2005-11-01T22:16:58Z","timestamp":1130883418000},"page":"257-265","source":"Crossref","is-referenced-by-count":3,"title":["His36Pro point\u2010mutated proteolipid protein retained in the endoplasmic reticulum of oligodendrocytes in the <i>Shaking pup<\/i>"],"prefix":"10.1002","volume":"53","author":[{"given":"Jonathan","family":"Song","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Brian D.","family":"Goetz","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ian 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