메뉴 건너뛰기




Volumn 24, Issue 20, 2004, Pages 4778-4786

Castration restores function and neurofilament alterations of aged symptomatic males in a transgenic mouse model of spinal and bulbar muscular atrophy

Author keywords

Androgen; Animal model; Motor neuron; Neurodegeneration; Neurofilament; Polyglutamine; Testosterone; Transgenic

Indexed keywords

ANDROGEN RECEPTOR; POLYGLUTAMINE; TESTOSTERONE;

EID: 2442719008     PISSN: 02706474     EISSN: None     Source Type: Journal    
DOI: 10.1523/JNEUROSCI.0808-04.2004     Document Type: Article
Times cited : (191)

References (44)
  • 1
    • 0035862754 scopus 로고    scopus 로고
    • Expression of expanded repeat androgen receptor produces neurologic disease in transgenic mice
    • Abel A, Walcott J, Woods J, Duda J, Merry DE (2001) Expression of expanded repeat androgen receptor produces neurologic disease in transgenic mice. Hum Mol Genet 10:107-116.
    • (2001) Hum Mol Genet , vol.10 , pp. 107-116
    • Abel, A.1    Walcott, J.2    Woods, J.3    Duda, J.4    Merry, D.E.5
  • 2
    • 0034678342 scopus 로고    scopus 로고
    • Cytoplasmic localization and the choice of ligand determine aggregate formation by androgen receptor with amplified polyglutamine stretch
    • Becker M, Elke M, Schneikert J, Krug HF, Cato ACB (2000) Cytoplasmic localization and the choice of ligand determine aggregate formation by androgen receptor with amplified polyglutamine stretch. J Cell Biol 149:255-262.
    • (2000) J Cell Biol , vol.149 , pp. 255-262
    • Becker, M.1    Elke, M.2    Schneikert, J.3    Krug, H.F.4    Cato, A.C.B.5
  • 3
    • 0033499931 scopus 로고    scopus 로고
    • Analysis of the role of heat shock protein (Hsp) molecular chaperones in polyglutamine disease
    • Chai Y, Koppenhafer SL, Bonini NM, Paulson HL (1999a) Analysis of the role of heat shock protein (Hsp) molecular chaperones in polyglutamine disease. J Neurosci 19:10338-10347.
    • (1999) J Neurosci , vol.19 , pp. 10338-10347
    • Chai, Y.1    Koppenhafer, S.L.2    Bonini, N.M.3    Paulson, H.L.4
  • 4
    • 0033030565 scopus 로고    scopus 로고
    • Evidence for proteasome involvement in polyglutamine disease: Localization to nuclear inclusions in SCA3/MJD and suppression of polyglutamine aggregation in vitro
    • Chai Y, Koppenhafer SL, Shoesmith SJ, Perez MK, Paulson HL (1999b) Evidence for proteasome involvement in polyglutamine disease: localization to nuclear inclusions in SCA3/MJD and suppression of polyglutamine aggregation in vitro. Hum Mol Genet 8:673-682.
    • (1999) Hum Mol Genet , vol.8 , pp. 673-682
    • Chai, Y.1    Koppenhafer, S.L.2    Shoesmith, S.J.3    Perez, M.K.4    Paulson, H.L.5
  • 5
    • 0030864463 scopus 로고    scopus 로고
    • Purkinje cell expression of a mutant allele of SCA1 in transgenic mice leads to disparate effects on motor behaviors, followed by a progressive cerebellar dysfunction and histological alterations
    • Clark HB, Burright EN, Yunis WS, Larson S, Wilcox C, Hartman B, Matilla A, Zoghbi HY, Orr HT (1997) Purkinje cell expression of a mutant allele of SCA1 in transgenic mice leads to disparate effects on motor behaviors, followed by a progressive cerebellar dysfunction and histological alterations. J Neurosci 17:7385-7395.
    • (1997) J Neurosci , vol.17 , pp. 7385-7395
    • Clark, H.B.1    Burright, E.N.2    Yunis, W.S.3    Larson, S.4    Wilcox, C.5    Hartman, B.6    Matilla, A.7    Zoghbi, H.Y.8    Orr, H.T.9
  • 6
    • 0034640011 scopus 로고    scopus 로고
    • Fourteen and counting: Unraveling trinucleotide repeat diseases
    • Cummings C, Zoghbi H (2000) Fourteen and counting: unraveling trinucleotide repeat diseases. Hum Mol Genet 9:909-916.
    • (2000) Hum Mol Genet , vol.9 , pp. 909-916
    • Cummings, C.1    Zoghbi, H.2
  • 7
    • 0031838352 scopus 로고    scopus 로고
    • Chaperone suppression of aggregation and altered subcellular proteasome localization imply protein misfolding in SCA1
    • Cummings CJ, Mancini MA, Antalffy B, DeFranco DB, Orr HT, Zoghbi HY (1998) Chaperone suppression of aggregation and altered subcellular proteasome localization imply protein misfolding in SCA1. Nat Genet 19:148-154.
    • (1998) Nat Genet , vol.19 , pp. 148-154
    • Cummings, C.J.1    Mancini, M.A.2    Antalffy, B.3    DeFranco, D.B.4    Orr, H.T.5    Zoghbi, H.Y.6
  • 8
    • 0037113310 scopus 로고    scopus 로고
    • Ligand-dependent aggregation of polyglutamine-expanded androgen receptor in neuronal cells
    • Darrington RS, Butler R, Leigh PN, McPhaul MJ, Gallo JM (2002) Ligand-dependent aggregation of polyglutamine-expanded androgen receptor in neuronal cells. NeuroReport 13:2117-2120.
    • (2002) NeuroReport , vol.13 , pp. 2117-2120
    • Darrington, R.S.1    Butler, R.2    Leigh, P.N.3    McPhaul, M.J.4    Gallo, J.M.5
  • 9
    • 0034681144 scopus 로고    scopus 로고
    • Regulation of expanded polyglutamine protein aggregation and nuclear localization by the glucocorticoid receptor
    • Diamond MI, Robinson MR, Yamamoto KR (2000) Regulation of expanded polyglutamine protein aggregation and nuclear localization by the glucocorticoid receptor. Proc Natl Acad Sci USA 97:657-661.
    • (2000) Proc Natl Acad Sci USA , vol.97 , pp. 657-661
    • Diamond, M.I.1    Robinson, M.R.2    Yamamoto, K.R.3
  • 11
    • 0034994207 scopus 로고    scopus 로고
    • Early degenerative changes in transgenic mice expressing mutant huntingtin involve dendritic abnormalities but no impairment of mitochondrial energy production
    • Guidetti P, Charles V, Chen EY, Reddy PH, Kordower JH, Whetsell Jr WO, Schwarcz R, Tagle DA (2001) Early degenerative changes in transgenic mice expressing mutant huntingtin involve dendritic abnormalities but no impairment of mitochondrial energy production. Exp Neurol 169:340-350.
    • (2001) Exp Neurol , vol.169 , pp. 340-350
    • Guidetti, P.1    Charles, V.2    Chen, E.Y.3    Reddy, P.H.4    Kordower, J.H.5    Whetsell Jr., W.O.6    Schwarcz, R.7    Tagle, D.A.8
  • 13
    • 0036245221 scopus 로고    scopus 로고
    • Rapid loss and partial recovery of neurofilament immunostaining following focal brain injury in mice
    • Huh JW, Laurer HL, Raghupathi R, Helfaer MA, Saatman KE (2002) Rapid loss and partial recovery of neurofilament immunostaining following focal brain injury in mice. Exp Neurol 175:198-208.
    • (2002) Exp Neurol , vol.175 , pp. 198-208
    • Huh, J.W.1    Laurer, H.L.2    Raghupathi, R.3    Helfaer, M.A.4    Saatman, K.E.5
  • 14
    • 0034793311 scopus 로고    scopus 로고
    • Clinical features and skewed X-chromosome inactivation in female carriers of X-linked recessive spinal and bulbar muscular atrophy
    • Ishihara H, Kanda F, Nishio H, Sumino K, Chihara K (2001) Clinical features and skewed X-chromosome inactivation in female carriers of X-linked recessive spinal and bulbar muscular atrophy. J Neurol 248:856-860.
    • (2001) J Neurol , vol.248 , pp. 856-860
    • Ishihara, H.1    Kanda, F.2    Nishio, H.3    Sumino, K.4    Chihara, K.5
  • 15
    • 0033977488 scopus 로고    scopus 로고
    • C-terminal phosphorylation of the high molecular weight neurofilament subunit correlates with decreased neurofilament axonal transport velocity
    • Jung C, Yabe JT, Shea TB (2000) C-terminal phosphorylation of the high molecular weight neurofilament subunit correlates with decreased neurofilament axonal transport velocity. Brain Res 856:12-19.
    • (2000) Brain Res , vol.856 , pp. 12-19
    • Jung, C.1    Yabe, J.T.2    Shea, T.B.3
  • 16
    • 18644379256 scopus 로고    scopus 로고
    • Testosterone reduction prevents phenotypic expression in a transgenic mouse model of spinal and bulbar muscular atrophy
    • Katsuno M, Adachi H, Kume A, Li M, Nakagomi Y, Niwa H, Sang C, Kobayashi Y, Doyu M, Sobue G (2002) Testosterone reduction prevents phenotypic expression in a transgenic mouse model of spinal and bulbar muscular atrophy. Neuron 35:843-854.
    • (2002) Neuron , vol.35 , pp. 843-854
    • Katsuno, M.1    Adachi, H.2    Kume, A.3    Li, M.4    Nakagomi, Y.5    Niwa, H.6    Sang, C.7    Kobayashi, Y.8    Doyu, M.9    Sobue, G.10
  • 17
    • 0038714285 scopus 로고    scopus 로고
    • Leuprorelin rescues polyglutamine-dependent phenotypes in a transgenic mouse model of spinal and bulbar muscular atrophy
    • Katsuno M, Adachi H, Doyu M, Minamiyama M, Sang C, Kobayashi Y, Inukai A, Sobue G (2003) Leuprorelin rescues polyglutamine-dependent phenotypes in a transgenic mouse model of spinal and bulbar muscular atrophy. Nat Med 9:768-773.
    • (2003) Nat Med , vol.9 , pp. 768-773
    • Katsuno, M.1    Adachi, H.2    Doyu, M.3    Minamiyama, M.4    Sang, C.5    Kobayashi, Y.6    Inukai, A.7    Sobue, G.8
  • 18
    • 0026595896 scopus 로고
    • Androgen receptor phosphorylation, turnover, nuclear transport and transcriptional activation: Specificity for steroids and antihormones
    • Kemppainen JA, Lane MV, Sar M, Wilson EM (1992) Androgen receptor phosphorylation, turnover, nuclear transport and transcriptional activation: specificity for steroids and antihormones. J Biol Chem 267:968-974.
    • (1992) J Biol Chem , vol.267 , pp. 968-974
    • Kemppainen, J.A.1    Lane, M.V.2    Sar, M.3    Wilson, E.M.4
  • 19
    • 0014310582 scopus 로고
    • Progressive proximal spinal and bulbar muscular atrophy of late onset: A sex-linked recessive trait
    • Kennedy WR, Alter M, Sung JH (1968) Progressive proximal spinal and bulbar muscular atrophy of late onset: a sex-linked recessive trait. Neurology 18:671-680.
    • (1968) Neurology , vol.18 , pp. 671-680
    • Kennedy, W.R.1    Alter, M.2    Sung, J.H.3
  • 21
    • 0025800526 scopus 로고
    • Androgen receptor gene mutations in X-linked spinal and bulbar muscular atrophy
    • La Spada AR, Wilson EM, Lubahn DB, Harding AE, Fischbeck KH (1991) Androgen receptor gene mutations in X-linked spinal and bulbar muscular atrophy. Nature 353:77-79.
    • (1991) Nature , vol.353 , pp. 77-79
    • La Spada, A.R.1    Wilson, E.M.2    Lubahn, D.B.3    Harding, A.E.4    Fischbeck, K.H.5
  • 24
    • 0041632303 scopus 로고    scopus 로고
    • Lack of huntingtin-associated protein-1 causes neuronal death resembling hypothalamic degeneration in Huntington's disease
    • Li SH, Yu ZX, Li CL, Nguyen HP, Zhou YX, Deng C, Li XJ (2003) Lack of huntingtin-associated protein-1 causes neuronal death resembling hypothalamic degeneration in Huntington's disease. J Neurosci 23:6956-6964.
    • (2003) J Neurosci , vol.23 , pp. 6956-6964
    • Li, S.H.1    Yu, Z.X.2    Li, C.L.3    Nguyen, H.P.4    Zhou, Y.X.5    Deng, C.6    Li, X.J.7
  • 26
    • 0031948607 scopus 로고    scopus 로고
    • Cleavage, aggregation and toxicity of the expanded androgen receptor in spinal and bulbar muscular atrophy
    • Merry DE, Kobayashi Y, Bailey CK, Taye AA, Fischbeck KH (1998) Cleavage, aggregation and toxicity of the expanded androgen receptor in spinal and bulbar muscular atrophy. Hum Mol Genet 7:693-701.
    • (1998) Hum Mol Genet , vol.7 , pp. 693-701
    • Merry, D.E.1    Kobayashi, Y.2    Bailey, C.K.3    Taye, A.A.4    Fischbeck, K.H.5
  • 27
    • 0024284028 scopus 로고
    • A simple salting out procedure for extracting DNA from human nucleated cells
    • Miller SA, Dykes DD, Polesky HF (1988) A simple salting out procedure for extracting DNA from human nucleated cells. Nucleic Acids Res 16:1215.
    • (1988) Nucleic Acids Res , vol.16 , pp. 1215
    • Miller, S.A.1    Dykes, D.D.2    Polesky, H.F.3
  • 29
    • 0036716277 scopus 로고    scopus 로고
    • Androgen receptor with elongated polyglutamine tract forms aggregates that alter axonal trafficking and mitochondrial distribution in motor neuronal processes
    • Piccioni F, Pinton P, Simeoni S, Pozzi P, Fascio U, Vismara G, Martini L, Rizzuto R, Poletti A (2002) Androgen receptor with elongated polyglutamine tract forms aggregates that alter axonal trafficking and mitochondrial distribution in motor neuronal processes. FASEB J 16:1418-1420.
    • (2002) FASEB J , vol.16 , pp. 1418-1420
    • Piccioni, F.1    Pinton, P.2    Simeoni, S.3    Pozzi, P.4    Fascio, U.5    Vismara, G.6    Martini, L.7    Rizzuto, R.8    Poletti, A.9
  • 33
    • 0029018031 scopus 로고
    • Neurofilament-associated protein phosphatase 2A: Its possible role in preserving neurofilaments in filamentous states
    • Saito T, Shima H, Osawa Y, Nagao M, Hemmings BA, Kishimoto T, Hisanaga S (1995) Neurofilament-associated protein phosphatase 2A: its possible role in preserving neurofilaments in filamentous states. Biochemistry 34:7376-7384.
    • (1995) Biochemistry , vol.34 , pp. 7376-7384
    • Saito, T.1    Shima, H.2    Osawa, Y.3    Nagao, M.4    Hemmings, B.A.5    Kishimoto, T.6    Hisanaga, S.7
  • 34
    • 0017685107 scopus 로고
    • Androgen concentration in motor neurons of cranial nerves and spinal cord
    • Sar M, Stumpf WE (1977) Androgen concentration in motor neurons of cranial nerves and spinal cord. Science 19:77-79.
    • (1977) Science , vol.19 , pp. 77-79
    • Sar, M.1    Stumpf, W.E.2
  • 35
  • 36
    • 0032945938 scopus 로고    scopus 로고
    • Polyglutamine-expanded androgen receptors form aggregates that sequester heat shock proteins, proteasome components and SRC-1, and are suppressed by the HDJ-2 chaperone
    • Stenoien DL, Cummings CJ, Adams HP, Mancini MG, Patel K, DeMartino GN, Marcelli M, Weigel NL, Mancini MA (1999) Polyglutamine-expanded androgen receptors form aggregates that sequester heat shock proteins, proteasome components and SRC-1, and are suppressed by the HDJ-2 chaperone. Hum Mol Genet 8:731-741.
    • (1999) Hum Mol Genet , vol.8 , pp. 731-741
    • Stenoien, D.L.1    Cummings, C.J.2    Adams, H.P.3    Mancini, M.G.4    Patel, K.5    DeMartino, G.N.6    Marcelli, M.7    Weigel, N.L.8    Mancini, M.A.9
  • 39
    • 0029052277 scopus 로고
    • Overexpression of the human NFM subunit in transgenic mice modifies the level of endogenous NFL and the phosphorylation state of NFH subunits
    • Tu PH, Elder G, Lazzarini RA, Nelson D, Trojanowski JQ, Lee VM (1995) Overexpression of the human NFM subunit in transgenic mice modifies the level of endogenous NFL and the phosphorylation state of NFH subunits. J Cell Biol 129:1629-1640.
    • (1995) J Cell Biol , vol.129 , pp. 1629-1640
    • Tu, P.H.1    Elder, G.2    Lazzarini, R.A.3    Nelson, D.4    Trojanowski, J.Q.5    Lee, V.M.6
  • 40
    • 0037184933 scopus 로고    scopus 로고
    • Ligand promotes intranuclear inclusions in a novel cell model of spinal and bulbar muscular atrophy
    • Walcott JL, Merry DE (2002) Ligand promotes intranuclear inclusions in a novel cell model of spinal and bulbar muscular atrophy. J Biol Chem 277:50855-50859.
    • (2002) J Biol Chem , vol.277 , pp. 50855-50859
    • Walcott, J.L.1    Merry, D.E.2
  • 41
    • 0033366384 scopus 로고    scopus 로고
    • Slowing of axonal transport is a very early event in the toxicity of ALS-linked SOD1 mutants to motor neurons
    • Williamson TL, Cleveland DW (1999) Slowing of axonal transport is a very early event in the toxicity of ALS-linked SOD1 mutants to motor neurons. Nat Neurosci 2:50-56.
    • (1999) Nat Neurosci , vol.2 , pp. 50-56
    • Williamson, T.L.1    Cleveland, D.W.2
  • 42
    • 0034737299 scopus 로고    scopus 로고
    • Reversal of neuropathology and motor dysfunction in a conditional model of Huntington's disease
    • Yamamoto A, Lucas JL, Hen R (2000) Reversal of neuropathology and motor dysfunction in a conditional model of Huntington's disease. Cell 101:57-66.
    • (2000) Cell , vol.101 , pp. 57-66
    • Yamamoto, A.1    Lucas, J.L.2    Hen, R.3
  • 43
    • 0037444445 scopus 로고    scopus 로고
    • Mutant huntingtin causes context-dependent neurodegeneration in mice with Huntington's disease
    • Yu ZX, Li SH, Evans J, Pillarisetti A, Li H, Li XJ (2003) Mutant huntingtin causes context-dependent neurodegeneration in mice with Huntington's disease. J Neurosci 23:2193-2202.
    • (2003) J Neurosci , vol.23 , pp. 2193-2202
    • Yu, Z.X.1    Li, S.H.2    Evans, J.3    Pillarisetti, A.4    Li, H.5    Li, X.J.6
  • 44
    • 0034094873 scopus 로고    scopus 로고
    • Glutamine repeats and neurodegeneration
    • Zoghbi HY, Orr HT (2000) Glutamine repeats and neurodegeneration. Annu Rev Neurosci 23:217-247.
    • (2000) Annu Rev Neurosci , vol.23 , pp. 217-247
    • Zoghbi, H.Y.1    Orr, H.T.2


* 이 정보는 Elsevier사의 SCOPUS DB에서 KISTI가 분석하여 추출한 것입니다.