Drug – bio-affecting and body treating compositions – Designated organic active ingredient containing – Peptide containing doai
Reexamination Certificate
2006-09-11
2010-06-01
Kemmerer, Elizabeth C. (Department: 1649)
Drug, bio-affecting and body treating compositions
Designated organic active ingredient containing
Peptide containing doai
C530S313000
Reexamination Certificate
active
07727952
ABSTRACT:
It is intended to provide a model animal faithfully reproducing the pathogenic conditions of spinal and bulbar muscular atrophy, a method of screening a remedy for polyglutamine disease using the same, and a remedy for spinal and bulbar muscular atrophy. Namely, a nonhuman animal having the following characteristics (1) to (5) in its conditions or pathological findings: (1) showing progressive myoatrophy; (2) showing lowering in muscular power; (3) in immunostaining with the use of an anti-polyglutamine antibody, showing nuclear diffuse staining and nuclear inclusions; (4) in immunostaining with the use of an anti-androgen receptor antibody, showing nuclear diffuse staining and nuclear inclusions; and (5) showing a neurogenic change. A remedy for polyglutamine disease is screened by administering a test substance to this nonhuman animal and examining changes in its conditions or pathological findings. A remedy for spinal and bulbar muscular atrophy is prepared by using as the active ingredient a compound having an effect of inhibiting the secretion of testosterone.
REFERENCES:
patent: 5733565 (1998-03-01), Moo-Young et al.
patent: 6096764 (2000-08-01), Bryant et al.
patent: 6506410 (2003-01-01), Park et al.
patent: 2004/0087557 (2004-05-01), Steiner et al.
M. Katsuno, et al.; “Testosterone reduction prevents phenotypic expression in a transgenic mouse model of spinal and bulbar muscular atrophy;”Neuron; vol. 35; No. 5; Aug. 2002; pp. 843-854.
H. Adachi, et al.; “Transgenic mouse model of spinal and bulbar muscular atrophy;”Society for Neuroscience Abstract Viewer and Itinerary Planner;2002; Abstract, No. 597.15.
R.S. Darrington, et al; “Protective effects of estrogens on polyglutamine-expanded androgen receptor aggregation in mice;”Neurosci. Lett.;No. 1; p. 350; Oct. 2003; pp. 37-40.
M. Katsuno, et al; Leuprorelin rescues polyglutamine-dependent phenotypes in a transgenic mouse model of spinal and bulbar muscular atrophy;Nat.Med.; vol. 9; No. 6; 2003; p. 768-773.
A. Abel, et al; “Expression of expanded repeat androgen receptor produces neurologic disease in trans genic mice;”Hum. Mol. Genet.; vol. 10; No. 2; Jan. 2001; pp. 107-116.
A.Abel et al., “Expression of expanded repeat androgen receptor produces neurologic disease in transgenic mice,”Human Molecular Genetics,vol. 10, No. 2, 2001, pp. 107-116.
H. Adachi et al., “Transgenic mice with an expanded CAG repeat controlled by the human AR promoter show polyglutamine nuclear inclusions and neuronal dysfunction without neuronal cell death,”Human Molecular Genetics,vol. 10, No. 10,2001, pp. 1039-1048.
O. A Andreassen et al., “Creatine Increases Survival and Delays Motor Symptoms in a Transgenic Animal Model of Huntington's Disease,”Neurobiology of Disease8,2001, pp. 479-491.
P. M. Bingham et al., “Stability of an expanded trinucleotide repeat in the androgen receptor gene in transgenic mice,”Nature Genetics,vol. 9, Feb. 1995, pp. 191-196.
N. L. Chamberlain et al., “The length and location of CAG trinucleotide repeats in the androgen receptor N-terminal domain affect transactivation function,”Nucleic Acids Research,vol. 22, No. 15, 1994, pp. 3181-3186.
C. J. Cummings et al., “Chaperone suppression of aggregation and altered subcellular proteasome localization imply protein misfolding in SCA 1,”Nature Genetics,vol. 19, Jun. 1998, pp. 148-154.
C. J. Cummings et al., “Mutation of the E6-AP Ubiquitin Ligase Reduces Nuclear Inclusion Frequency While Accelerating Polyglutamine-Induced Pathology inSCA 1Mice,”Neuron, vol. 24, Dec. 1999, pp. 879-892.
C. J. Cummings et al., “Over-expression of inducible HSP70 chaperone suppresses neuropathology and improves motor function inSCA 1mice,”Human Molecular Genetics,vol. 10, No. 14, 2001, pp. 1511-1518.
A. Danek et al., “Decrease in androgen binding and effect of androgen treatment in a case of X-linked bulbospinal neuronopathy,”Clin Investig, 72, 1994, pp. 892-897.
M. Doyu et al., “Severity of X-Linked Recessive Bulbospinal Neuronopathy Correlates with Size of the Tandem CAG Repeat in Androgen Receptor Gene,”Annals of Neurology, vol. 32, No. 5, Nov. 1992, pp. 707-710.
M. Duyao et al, “Trinucleotide repeat length instability and age of onset in Huntington's disease,”Nature Genetics, vol. 4, Aug. 1993, pp. 387-392.
J. N. Goldenberg et al, “Testosterone therapy and the pathogenesis of Kennedy's disease (X-linked bulbospinal muscular atrophy),”Journal of the Neurological Sciences135, 1996, pp. 158-161.
C.-A. Gutekunst et al., “Nuclear and Neuropil Aggregates in Huntington's Disease: Relationship to Neuropathology,”Journal of Neuroscience19(7), Apr. 1999, pp. 2522-2534.
M. C. Hall et al., “Prospective Determination of the Hormonal Response After Cessation of Luteinizing Hormone-Releasing Hormone Agonist Treatment in Patients With Prostate Cancer,”Urology53(5), 1999, pp. 898-902.
V. Heiser et al., “Inhibition of Huntingtin Fibrillogenesis by Specific Antibodies and Small Molecules: Implications for Huntington's Disease Therapy,”Proceedings of the National Academy of Sciences of the United States of America, vol. 97, No. 12, Jun. 2000, pp. 6739-6744.
D. P. Huynh et al., “Nuclear localization or inclusion body formation of ataxin-2 are not necessary for SCA2 pathogenesis in mouse or human,”Nature Genetics, vol. 26, Sep. 2000, pp. 44-50.
S. Igarashi et al., “Strong correlation between the number of CAG repeats in androgen receptor genes and the clinical onset of features of spinal and bulbar muscular atrophy,”Neurology42, Dec. 1992, pp. 2300-2302.
S. Igarashi et al., “Suppression of aggregate formation and apoptosis by transglutaminase inhibitors in cells expressing truncated DRPLA protein with an expanded polyglutamine stretch,”Nature Genetics,vol. 18, Feb. 1998, pp. 111-117.
M. V. Karpuj et al., “Prolonged survival and decreased abnormal movements in transgenic model of Huntington disease, with administration of the transglutaminase inhibitor cystamine,”Nature Medicine,vol. 8, No. 2, Feb. 2002, pp. 143-149.
P. Kazemi-Esfarjani et al., “Evidence for a repressive function of the long polyglutamine tract in the human androgen receptor: possible pathogenetic relevance for the (CAG)n-expanded neuronopathies,”Human Molecular Genetics, vol. 4, No. 4, 1995, pp. 523-527.
J. A. Kemppainen et al, “Androgen Receptor Phosphorylation, Turnover, Nuclear Transport, and Transcriptional Activation,”Journal of Biological Chemistry, vol. 267, No. 2, Jan. 1992, pp. 968-974.
W. R. Kennedy et al., “Progressive proximal spinal and bulbar muscular atrophy of late onset: A sex-linked recessive trait,”Neurology, vol. 18, Jul. 1968, pp. 671-680.
I. A. Klement et al., “Ataxin-1 Nuclear Localization and Aggregation: Role in Polyglutamine-induced Disease inSCA1Transgenic Mice,”Cell,Vo. 95, Oct. 1998, pp. 41-53.
Y. Kobayashi et al., “Caspase-3 Cleaves the Expanded Androgen Receptor Protein of Spinal and Bulbar Muscular Atrophy in a Polyglutamine Repeat Length-Dependent Manner,”Biochemical and Biophysical Research Communications252, 1998, pp. 145-150.
Y. Kobayashi et al., “Chaperones Hsp70 and Hsp40 Suppress Aggregate Formation and Apoptosis in Cultured Neuronal Cells Expressing Truncated Androgen Receptor Protein with Expanded Polyglutamine Tract,”Journal of Biological Chemistry,vol. 275, No. 12, Mar. 2000, pp. 8772-8778.
A. R. La Spada et al., “Androgen receptor gene mutations in X-linked spinal and bulbar muscular atrophy,”Naturevol. 352, Jul. 1991, pp. 77-79.
A. R. La Spada et al., “Meiotic stability and genotype-phenotype correlation of the trinucleotide repeat in X-linked spinal and bulbar muscular atrophy,”Nature Genetics,vol. 2, Dec. 1992, pp. 301-304.
A. R. La Spada et al., “Androgen receptor
Adachi Hiroaki
Katsuno Masahisa
Sobue Gen
Ballard Kimberly A.
Edwards Angell Palmer & & Dodge LLP
Kemmerer Elizabeth C.
Nagoya Industrial Science Research Institute
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