{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,9,17]],"date-time":"2025-09-17T14:45:07Z","timestamp":1758120307459},"reference-count":60,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2001,2,27]],"date-time":"2001-02-27T00:00:00Z","timestamp":983232000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J of Comparative Neurology"],"published-print":{"date-parts":[[2001,4,2]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>We have investigated the fate of different neurotrophin\u2010responsive subpopulations of dorsal root ganglion neurons in <jats:italic>dystonia musculorum<\/jats:italic> (<jats:italic>dt<\/jats:italic>) mice. These mice have a null mutation in the cytoskeletal linker protein, dystonin. Dystonin is expressed by all sensory neurons and cross links actin filaments, intermediate filaments, and microtubules. The <jats:italic>dt<\/jats:italic> mice undergo massive sensory neurodegeneration postnatally and die at around 4 weeks of age. We assessed the surviving and degenerating neuronal populations by comparing the dorsal root ganglion (DRG) neurons and central and peripheral projections in <jats:italic>dt<\/jats:italic> mice and wildtype mice. Large, neurofilament\u2010H\u2010positive neurons, many of which are muscle afferents and are neurotrophin\u20103 (NT\u20103)\u2010responsive, were severely decreased in number in <jats:italic>dt<\/jats:italic> DRGs. The loss of muscle afferents was correlated with a degeneration of muscle spindles in skeletal muscle. Nerve growth factor (NGF)\u2010responsive populations, which were visualized using calcitonin gene\u2010related peptide and p75, appeared qualitatively normal in the lumbar spinal cord, DRG, and hindlimb skin. In contrast, glial cell line\u2010derived neurotrophic factor (GDNF)\u2010responsive populations, which were visualized using the isolectin B\u20104 and thiamine monophosphatase, were severely diminished in the lumbar spinal cord, DRG, and hindlimb skin. Analysis of NT\u20103, NGF, and GDNF mRNA levels using semiquantitative reverse transcriptase\u2010polymerase chain reaction revealed normal trophin synthesis in the peripheral targets of <jats:italic>dt<\/jats:italic> mice, arguing against decreased trophic synthesis as a possible cause of neuronal degeneration. Thus, the absence of dystonin results in the selective survival of NGF\u2010responsive neurons and the postnatal degeneration of many NT\u20103\u2010 and GDNF\u2010responsive neurons. Our results reveal that the loss of this ubiquitously expressed cytoskeletal linker has diverse effects on sensory subpopulations. Moreover, we show that dystonin is critical for the maintenance of certain DRG neurons, and its function may be related to neurotrophic support. J. Comp. Neurol. 432:155\u2013168, 2001. \u00a9 2001 Wiley\u2010Liss, Inc.<\/jats:p>","DOI":"10.1002\/cne.1094","type":"journal-article","created":{"date-parts":[[2002,8,25]],"date-time":"2002-08-25T18:04:33Z","timestamp":1030298673000},"page":"155-168","source":"Crossref","is-referenced-by-count":24,"title":["Glial cell line\u2010derived neurotrophic factor\u2010responsive and neurotrophin\u20103\u2010responsive neurons require the cytoskeletal linker protein dystonin for postnatal survival"],"prefix":"10.1002","volume":"432","author":[{"given":"Julie A.","family":"Carlsten","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Rashmi","family":"Kothary","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Douglas E.","family":"Wright","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2001,2,27]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"publisher","DOI":"10.1006\/mcne.1999.0754"},{"key":"e_1_2_7_3_1","doi-asserted-by":"publisher","DOI":"10.1006\/exnr.2000.7547"},{"key":"e_1_2_7_4_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1750-3639.1999.tb00234.x"},{"key":"e_1_2_7_5_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1460-9568.1995.tb01143.x"},{"key":"e_1_2_7_6_1","doi-asserted-by":"crossref","first-page":"148","DOI":"10.1016\/0955-0674(95)80022-0","article-title":"Neurotrophic factors and their receptors","volume":"2","author":"Barbacid M","year":"1995","journal-title":"Curr Opin Cell Biol"},{"key":"e_1_2_7_7_1","doi-asserted-by":"publisher","DOI":"10.1523\/JNEUROSCI.18-08-03059.1998"},{"key":"e_1_2_7_8_1","doi-asserted-by":"publisher","DOI":"10.1002\/(SICI)1520-6408(1998)22:2<160::AID-DVG5>3.0.CO;2-4"},{"key":"e_1_2_7_9_1","doi-asserted-by":"publisher","DOI":"10.1139\/o95-066"},{"key":"e_1_2_7_10_1","doi-asserted-by":"publisher","DOI":"10.1006\/mcne.1995.0003"},{"key":"e_1_2_7_11_1","doi-asserted-by":"publisher","DOI":"10.1523\/JNEUROSCI.17-18-07007.1997"},{"key":"e_1_2_7_12_1","doi-asserted-by":"publisher","DOI":"10.1038\/ng0795-301"},{"key":"e_1_2_7_13_1","doi-asserted-by":"publisher","DOI":"10.1016\/0306-4522(90)90017-X"},{"key":"e_1_2_7_14_1","doi-asserted-by":"publisher","DOI":"10.1038\/242"},{"key":"e_1_2_7_15_1","doi-asserted-by":"publisher","DOI":"10.1016\/0306-4522(94)90578-9"},{"key":"e_1_2_7_16_1","doi-asserted-by":"publisher","DOI":"10.1002\/(SICI)1096-9861(19990913)412:1<186::AID-CNE14>3.0.CO;2-H"},{"key":"e_1_2_7_17_1","doi-asserted-by":"publisher","DOI":"10.1006\/dbio.1997.8567"},{"key":"e_1_2_7_18_1","first-page":"7","article-title":"Dystonia musculorum. 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