Pathogenesis of axonal dystrophy and demyelination in αA-crystallin-expressing transgenic mice
We recently described a transgenic mouse strain overexpressing hamster αA‐crystallin, a small heat shock protein, under direction of the hamster vimentin promoter. As a result myelin was degraded and axonal dystrophy in both central nervous system (especially spinal cord) and peripheral nervous syst...
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| Published in | International journal of experimental pathology Vol. 84; no. 2; pp. 91 - 99 |
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| Main Authors | , , , , |
| Format | Journal Article |
| Language | English |
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Oxford, UK
Blackwell Science Ltd
01.04.2003
Blackwell Science Blackwell Science Inc |
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| Abstract | We recently described a transgenic mouse strain overexpressing hamster αA‐crystallin, a small heat shock protein, under direction of the hamster vimentin promoter. As a result myelin was degraded and axonal dystrophy in both central nervous system (especially spinal cord) and peripheral nervous system occurred. Homozygous transgenic mice developed hind limb paralysis after 8 weeks of age and displayed progressive loss of myelin and axonal dystrophy in both the central and peripheral nervous system with ongoing age. Pathologically the phenotype resembled, to a certain extent, neuroaxonal dystrophy. The biochemical findings presented in this paper (activity of the enzymes superoxide dismutase, catalase and transglutamase, myelin protein zero expression levels and blood sugar levels) confirm this pathology and exclude other putative pathologies like Amyothrophic Lateral Sclerosis and Hereditary Motor and Sensory Neuropathy. Consequently, an excessive cytoplasmic accumulation of the transgenic protein or a disturbance of the normal metabolism are considered to cause the observed neuropathology. Therefore, extra‐ocular αA‐crystallin‐expressing transgenic mice may serve as a useful animal model to study neuroaxonal dystrophy. |
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| AbstractList | We recently described a transgenic mouse strain overexpressing hamster αA-crystallin, a small heat shock protein, under direction of the hamster vimentin promoter. As a result myelin was degraded and axonal dystrophy in both central nervous system (especially spinal cord) and peripheral nervous system occurred. Homozygous transgenic mice developed hind limb paralysis after 8 weeks of age and displayed progressive loss of myelin and axonal dystrophy in both the central and peripheral nervous system with ongoing age. Pathologically the phenotype resembled, to a certain extent, neuroaxonal dystrophy. The biochemical findings presented in this paper (activity of the enzymes superoxide dismutase, catalase and transglutamase, myelin protein zero expression levels and blood sugar levels) confirm this pathology and exclude other putative pathologies like Amyothrophic Lateral Sclerosis and Hereditary Motor and Sensory Neuropathy. Consequently, an excessive cytoplasmic accumulation of the transgenic protein or a disturbance of the normal metabolism are considered to cause the observed neuropathology. Therefore, extra-ocular αA-crystallin-expressing transgenic mice may serve as a useful animal model to study neuroaxonal dystrophy. |
| Author | Sweers, M.A.M. Merkx, G.F.M. Bloemendal, H. Lammens, M. Van Rijk, A.F. |
| Author_xml | – sequence: 1 givenname: A.F. surname: Van Rijk fullname: Van Rijk, A.F. organization: Department of Biochemistry, University of Nijmegen, 6500 HB Nijmegen, the Netherlands – sequence: 2 givenname: M.A.M. surname: Sweers fullname: Sweers, M.A.M. organization: Department of Human Genetics and – sequence: 3 givenname: G.F.M. surname: Merkx fullname: Merkx, G.F.M. organization: Department of Pathology and Neurology, University Medical Center 6500 HB Nijmegen, the Netherlands – sequence: 4 givenname: M. surname: Lammens fullname: Lammens, M. organization: Department of Biochemistry, University of Nijmegen, 6500 HB Nijmegen, the Netherlands – sequence: 5 givenname: H. surname: Bloemendal fullname: Bloemendal, H. email: H.Bloemendal@ncmls.kun.nl organization: Department of Biochemistry, University of Nijmegen, 6500 HB Nijmegen, the Netherlands |
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| Cites_doi | 10.1016/0092-8674(92)90591-Y 10.1523/JNEUROSCI.16-20-06386.1996 10.1093/genetics/110.4.723 10.1007/BF01790973 10.5414/CPP38094 10.1016/0165-4608(94)90082-5 10.1038/356241a0 10.1046/j.1365-2613.2000.00161.x 10.1074/jbc.271.30.17903 10.1007/BF01235758 10.1016/0003-2697(71)90370-8 10.1007/BF00995383 10.1002/ana.410180302 10.1016/S0021-9258(19)50881-X 10.1016/S0006-291X(89)80215-3 10.2131/jts.25.137 10.1111/j.1471-4159.1992.tb10051.x 10.1016/0896-6273(95)90259-7 10.1126/science.877544 10.1016/S0378-1119(98)00103-6 10.1016/0888-7543(91)90370-T 10.1073/pnas.95.9.5161 10.1248/bpb.26.313 10.1056/NEJM198906293202606 10.1016/S0022-510X(00)00321-X 10.1097/00005072-199812000-00010 10.1038/ng0993-35 10.1016/0022-510X(95)00124-K 10.1046/j.1471-4159.1995.64031216.x 10.1093/nar/3.9.2303 10.1023/A:1022442904179 10.1111/j.1399-3011.1969.tb01623.x 10.1515/bchm3.1989.370.2.661 |
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| Keywords | Immunohistochemistry Animal model Nervous system diseases Biochemical analysis Rodentia Transgenic animal Neuroaxonal dystrophy Cerebral disorder Genetic disease Pathology Vertebrata Experimental disease Mammalia Crystallin Mouse Demyelination Animal Central nervous system disease Degenerative disease Molecular biology Immunofluorescence |
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| References | Fujita K., Shibayama K., Yamauchi M., Kato T., Ando M., Takahashi H., Iritani K., Yoshimoto N. & Nagata Y. (1998) Alteration of enzymatic activities implicating neuronal degeneration in the spinal of the motor neuron degeneration mouse during postnatal development. Neurochem. Res. 23, 557-562. Schindler D., Bishop D.F., Wolfe D.E., Wang A.M., Egge H., Lemieux R.U. & Desnick R.J. (1989) Neuroaxonal dystrophy due to lysosomal α-N-acetylgalactosaminidase deficiency. N. Engl. J. Med. 320, 1735-1740. Tandan R. & Bradley W.G. (1985) Amyotrophic lateral sclerosis: Part 1. Clinical features, pathology, and ethical issues in management. Ann. Neurol. 18, 271-280. Ikegami H., Tabata H., Matsuzawa T. & Suzuki H. (2000) The Exacerbating effect of insulin-induced hypoglycemia on spontaneous peripheral neuropathy in aged B6C3F1 mice. J. Toxicol. Sci. 25, 137-142. Schwarz H.J., Chang Y.S., Hennemann H., Dahl E., Lalley P.A. & Willecke K. (1992) Chromosomal assignment of mouse connexin genes, coding for gap junctional proteins, by somatic cell hybridization. Somat. Cell. Mol. Genet. 18, 205-206. Fesus L., Thomazy V. & Falus A. (1988) Reaching for function of tissue transglutaminase; its possible involvement in biochemical pathway of programmed cell death. Adv. Exp. Biol. 231, 119-139. De Rijk E.P.C.T., Van Rijk A.F., Van Esch E., De Jong W.W., Wesseling P. & Bloemendal H. (2000) Demyelination and axonal dystrophy in alpha A-crystallin transgenic mice. Int. J. Exp. Pathol. 81, 271-282. Blin N. & Stafford D.W. (1976) A general method for isolation of high molecular weight DNA from eukaryotes. Nucl Acid Res. 3, 2303-2308. Przedborski S., Jackson-Lewis V., Kostic V., Carlson E., Epstein C.J. & Cadet J.L. (1992) Superoxide dismutase, catalase, and glutathione peroxidase activities in copper/zinc-superoxide dismutase transgenic mice. J. Neurochem. 58, 1760-1767. Beers R.F. & Sizer I.W. (1952) A spectrophotometric method for measuring the breakdown of hydrogen peroxide by catalase. J. Biol. Chem. 195, 133-140. Wong P.C., Pardo C.A., Borchelt D.R., Lee M.K., Copeland N.G., Jenkins N.A., Sisodia S.S., Cleveland D.W. & Price D.L. (1995) An adverse property of a familial ALS-linked SOD1 mutation causes motor neuron disease characterized by vacuolar degeneration of mitochondria. Neuron 14, 1105-1116. Smit J.J.M., Baas F., Hoogendijk J.E., Jansen G.H., Van Der Valk M.A., Schinkel A.H., Berns A.J.M., Acton D., Nooter K., Burger H., Smith S.J. & Borst P. (1996) Peripheral neuropathy in mice transgenic for a human MDR3 P-glycoprotein mini-gene. J. Neurosci. 16, 6386-6393. Bloemendal H. (1977) The vertebrate eye lens. Science 197, 127-138. Van Den Heuvel-Eiberink M.M., Sonneveld P. & Pieters R. (2000) The prognostic significance of membrane transport-associated multidrug resistance (MDR) proteins in leukemia. Int. J. Clin. Pharmacol. Ther. 38, 94-110. Schoenmakers J.G.G., Matze R., Van Poppel M. & Bloemendal H. (1969) Isolation of non-identical polypeptide chains of α-crystallin. Intern. J. Prot. Res. 1, 19-27. Gsell W., Conrad R., Hickethier M., Sofic E., Frolich L., Wichart I., Jellinger K., Moll G., Ransmayr G., Beckmann H. & Riederer P. (1995) Decreased catalase activity but unchanged superoxide dismutase activity in brains of patients with dementia of Alzheimer type. J. Neurochem. 64, 1216-1223. Skow L.C. & Donner M.E. (1985) The locus encoding alpha A-crystallin is closely linked to H-2K on mouse chromosome 17. Genetics 110, 723-732. Suijkerbuijk R.F., Sinke R.J., Weghuis D.E., Roque L., Forus A., Stellink F., Siepman A., Van De Kaa C., Soares J. & Geurts van Kessel A.H. (1994) Amplification of chromosome subregion 12p11.2-p12.1 in a metastasis of an i(12p) -negative seminoma: Relationship to progression? Cancer. Genet. Cytogenet. 78, 145-152. Giese K.P., Martini R., Lemke G., Soriano P. & Schachner M. (1992) Mouse P0 gene disruption leads to hypomyelination, abnormal expression of recognition molecules, and degeneration of myelin and axons. Cell 71, 565-576. Kato S., Hayashi H., Nakashima K., Nanba E., Katao M., Hirano A., Nakano I., Asayama K. & Ohama E. (1997) Pathological characterization of astrocytic hyaline inclusions in familial amyotrophic lateral sclerosis. Am. J. Pathol. 151, 611-620. Suter U., Welcher A.A., Ozcelik T., Snipes G.J., Kosaras B., Francke U., Billings-Gagliardi S., Sidman R.L. & Shooter E.M. (1992) Trembler mouse carries a point mutation in a myelin gene. Nature 356, 241-244. Herbarth B., Pingault V., Bondurand N., Kuhlbrodt K., Hermans-Borgmeyer I., Puliti A., Lemort N., Goossens M. & Wegner M. (1998) Mutation of Sry-related Sox10 gene in dominant megacolon, a mouse model for human Hirschsprung disease. Proc. Natl. Acad. Sci. USA 95, 5161-5165. Linden H.U., Klein R.A., Egge H., Peter-Katalinic J., Dabrowski J. & Schindler D. (1989) Isolation and structural characterization of sialic-acid containing glycopeptides of the O-glycosidic type from the urine of two patients with a hereditary deficiency in α-N-acetylgalactosaminidase activity. Biol. Chem. Hoppe-Seyler 370, 661-672. Dahl E., Mantey D., Chen Y., Schwarz H.J., Chang Y.S., Lalley P.A., Nicholson B.J. & Willecke K. (1996) Molecular cloning and functional expression of mouse connexin-30, a gap junction gene highly expressed in adult brain and skin. J. Biol. Chem. 271, 17903-17910. Schmidt R.E., Dorsey D.A., Beaudet L.N., Plurad S.B., Williamson J.R. & Ido Y. (1998) Effect of sorbitol dehydrogenase inhibition on experimental diabetic autonomic neuropathy. J. Neuropathol. Exp. Neurol. 57, 1175-1189. Beauchamp C. & Fridovich I. (1971) Superoxide dismutase: improved assay and an assay applicable to acrylamide gels. Anal Biochem. 44, 276-287. Fujita K., Ando M., Yamauchi M., Nagata Y. & Honda M. (1995) Alteration of transglutaminase activity in rat and human spinal cord after neuronal degeneration. Neurochem. Res. 20, 1195-1201. Aizawa H., Kimura T., Hashimoto K., Yahara O., Okamoto K. & Kikuchi K. (2000) Basophilic cytoplasmic inclusions in a case of sporadic juvenile amyotrophiclateral sclerosis. J. Neurol. Sci. 176, 109-113. Wolfe D.E., Schindler D. & Desnick R.J. (1995) Neuroaxonal dystrophy in infantile α-N-acetylgalactosaminidase deficiency. J. Neurol. Sci. 132, 44-56. Kulkens T., Bolhuis P.A., Wolterman R.A., Kemp S., Te Nijenhuis S., Valentij L.J., Hensels G.W., Jennekens F.G.I., De Visser M., Hoogendijk J.E. & Baas F. (1993) Deletion of the serine 34 codon from the major peripheral myelin protein P0 gene in Charcot-Marie Tooth disease type 1B. Nat Genet 5, 35-38. You K.H., Hsieh C.L., Hayes C., Stahl N., Francke U. & Popko B. (1991) DNA sequence, genomic organization, and chromosomal localization of the mouse peripheral myelin protein zero gene: identification of polymorphic alleles. Genomics 9, 751-757. Herrmann T., Schindler D., Tabe H., Onodera O., Igarashi S., Polack A., Zehnpfennig D. & Shoji Tsuji (1998) Molecular cloning, structural organization, sequence, chromosomal assignment,and expression of the mouse alpha-N-acetylgalactosaminidase gene. Gene 211, 205-214. Hoffmann J. (1889) Ueber progressive neurotische Muskelatrophie. Arch. Psychiat Nervenkr 20, 660-713. Bhat S.P. & Nagineni C.N. (1989) αB Subunit of lens-specific protein alpha-crystallin is present in other ocular and non-ocular tissues. Biophys. Res. Commun. 158, 319-325. Tsutsumi T., Kobayashi S., Liu Y.Y. & Kontani H. (2003) Anti-hyperglycemic Effect of Fangchinoline Isolated from Stephania Tetrandra Radix in Streptozotocin-Diabetic Mice. Biol. Pharm. Bull. 26, 313-317. 1971; 44 1952; 195 2000; 25 1989; 158 1997; 151 1995; 14 1976; 3 1992; 18 2000; 176 1992; 58 1992 1995; 132 1996; 16 1998; 211 1991; 9 1998; 23 1992; 71 1993; 5 1995; 20 1995; 64 1985; 18 2000; 38 1969; 1 1992; 356 1889; 20 1989; 320 1986; 49 1994; 78 1996; 271 1989; 370 2000; 81 2003; 26 1988; 231 1982 1998; 95 1977; 197 1989 1985; 110 1998; 57 Kuypers H.G.J.M. (e_1_2_6_23_1) 1982 e_1_2_6_30_1 Kato S. (e_1_2_6_21_1) 1997; 151 Desnick R.J. (e_1_2_6_9_1) 1989 e_1_2_6_19_1 Skow L.C. (e_1_2_6_32_1) 1985; 110 e_1_2_6_13_1 e_1_2_6_36_1 e_1_2_6_14_1 e_1_2_6_35_1 e_1_2_6_34_1 e_1_2_6_12_1 e_1_2_6_17_1 e_1_2_6_18_1 e_1_2_6_39_1 e_1_2_6_15_1 e_1_2_6_38_1 e_1_2_6_16_1 e_1_2_6_37_1 e_1_2_6_20_1 e_1_2_6_40_1 Fesus L. (e_1_2_6_11_1) 1988; 231 e_1_2_6_8_1 Smit J.J.M. (e_1_2_6_33_1) 1996; 16 e_1_2_6_5_1 e_1_2_6_4_1 e_1_2_6_7_1 e_1_2_6_6_1 e_1_2_6_25_1 e_1_2_6_24_1 e_1_2_6_3_1 e_1_2_6_2_1 Dyck P.J. (e_1_2_6_10_1) 1992 e_1_2_6_22_1 e_1_2_6_29_1 e_1_2_6_28_1 e_1_2_6_27_1 Seitelberger F. (e_1_2_6_31_1) 1986 e_1_2_6_26_1 |
| References_xml | – volume: 49 start-page: 391 year: 1986 end-page: 415 – volume: 78 start-page: 145 year: 1994 end-page: 152 article-title: Amplification of chromosome subregion 12p11.2‐p12.1 in a metastasis of an i(12p) ‐negative seminoma: Relationship to progression? publication-title: Cancer. Genet. Cytogenet. – volume: 25 start-page: 137 year: 2000 end-page: 142 article-title: The Exacerbating effect of insulin‐induced hypoglycemia on spontaneous peripheral neuropathy in aged B6C3F1 mice publication-title: J. Toxicol. Sci. – volume: 320 start-page: 1735 year: 1989 end-page: 1740 article-title: Neuroaxonal dystrophy due to lysosomal α‐N‐acetylgalactosaminidase deficiency publication-title: N. Engl. J. Med. – volume: 14 start-page: 1105 year: 1995 end-page: 1116 article-title: An adverse property of a familial ALS‐linked SOD1 mutation causes motor neuron disease characterized by vacuolar degeneration of mitochondria publication-title: Neuron – start-page: 1094 year: 1992 end-page: 1136 – volume: 16 start-page: 6386 year: 1996 end-page: 6393 article-title: Peripheral neuropathy in mice transgenic for a human MDR3 P‐glycoprotein mini‐gene publication-title: J. Neurosci. – volume: 95 start-page: 5161 year: 1998 end-page: 5165 article-title: Mutation of Sry‐related Sox10 gene in dominant megacolon, a mouse model for human Hirschsprung disease publication-title: Proc. Natl. Acad. Sci. USA – volume: 58 start-page: 1760 year: 1992 end-page: 1767 article-title: Superoxide dismutase, catalase, and glutathione peroxidase activities in copper/zinc‐superoxide dismutase transgenic mice publication-title: J. Neurochem. – volume: 158 start-page: 319 year: 1989 end-page: 325 article-title: αB Subunit of lens‐specific protein alpha‐crystallin is present in other ocular and non‐ocular tissues publication-title: Biophys. Res. Commun. – volume: 132 start-page: 44 year: 1995 end-page: 56 article-title: Neuroaxonal dystrophy in infantile α‐N‐acetylgalactosaminidase deficiency publication-title: J. Neurol. Sci. – volume: 71 start-page: 565 year: 1992 end-page: 576 article-title: Mouse P0 gene disruption leads to hypomyelination, abnormal expression of recognition molecules, and degeneration of myelin and axons publication-title: Cell – volume: 9 start-page: 751 year: 1991 end-page: 757 article-title: DNA sequence, genomic organization, and chromosomal localization of the mouse peripheral myelin protein zero gene: identification of polymorphic alleles publication-title: Genomics – volume: 64 start-page: 1216 year: 1995 end-page: 1223 article-title: Decreased catalase activity but unchanged superoxide dismutase activity in brains of patients with dementia of Alzheimer type publication-title: J. Neurochem. – volume: 356 start-page: 241 year: 1992 end-page: 244 article-title: Trembler mouse carries a point mutation in a myelin gene publication-title: Nature – start-page: 381 year: 1982 end-page: 403 – volume: 18 start-page: 271 year: 1985 end-page: 280 article-title: Amyotrophic lateral sclerosis: Part 1. Clinical features, pathology, and ethical issues in management publication-title: Ann. Neurol. – volume: 110 start-page: 723 year: 1985 end-page: 732 article-title: The locus encoding alpha A‐crystallin is closely linked to H‐2K on mouse chromosome 17 publication-title: Genetics – volume: 370 start-page: 661 year: 1989 end-page: 672 article-title: Isolation and structural characterization of sialic‐acid containing glycopeptides of the ‐glycosidic type from the urine of two patients with a hereditary deficiency in α‐N‐acetylgalactosaminidase activity publication-title: Biol. Chem. Hoppe-Seyler – volume: 81 start-page: 271 year: 2000 end-page: 282 article-title: Demyelination and axonal dystrophy in alpha A‐crystallin transgenic mice publication-title: Int. J. Exp. Pathol. – volume: 20 start-page: 1195 year: 1995 end-page: 1201 article-title: Alteration of transglutaminase activity in rat and human spinal cord after neuronal degeneration publication-title: Neurochem. Res. – volume: 231 start-page: 119 year: 1988 end-page: 139 article-title: Reaching for function of tissue transglutaminase; its possible involvement in biochemical pathway of programmed cell death publication-title: Adv. Exp. Biol. – volume: 23 start-page: 557 year: 1998 end-page: 562 article-title: Alteration of enzymatic activities implicating neuronal degeneration in the spinal of the motor neuron degeneration mouse during postnatal development publication-title: Neurochem. Res. – start-page: 1751 year: 1989 end-page: 1798 – volume: 20 start-page: 660 year: 1889 end-page: 713 article-title: Ueber progressive neurotische Muskelatrophie publication-title: Arch. Psychiat Nervenkr – volume: 211 start-page: 205 year: 1998 end-page: 214 article-title: Molecular cloning, structural organization, sequence, chromosomal assignment,and expression of the mouse alpha‐N‐acetylgalactosaminidase gene publication-title: Gene – volume: 1 start-page: 19 year: 1969 end-page: 27 article-title: Isolation of non‐identical polypeptide chains of α–crystallin publication-title: Intern. J. Prot. Res. – volume: 176 start-page: 109 year: 2000 end-page: 113 article-title: Basophilic cytoplasmic inclusions in a case of sporadic juvenile amyotrophiclateral sclerosis publication-title: J. Neurol. Sci. – volume: 197 start-page: 127 year: 1977 end-page: 138 article-title: The vertebrate eye lens publication-title: Science – volume: 38 start-page: 94 year: 2000 end-page: 110 article-title: The prognostic significance of membrane transport‐associated multidrug resistance (MDR) proteins in leukemia publication-title: Int. J. Clin. Pharmacol. Ther. – volume: 18 start-page: 205 year: 1992 end-page: 206 article-title: Chromosomal assignment of mouse connexin genes, coding for gap junctional proteins, by somatic cell hybridization publication-title: Somat. Cell. Mol. Genet. – volume: 3 start-page: 2303 year: 1976 end-page: 2308 article-title: A general method for isolation of high molecular weight DNA from eukaryotes publication-title: Nucl Acid Res. – volume: 271 start-page: 17903 year: 1996 end-page: 17910 article-title: Molecular cloning and functional expression of mouse connexin‐30, a gap junction gene highly expressed in adult brain and skin publication-title: J. Biol. Chem. – volume: 57 start-page: 1175 year: 1998 end-page: 1189 article-title: Effect of sorbitol dehydrogenase inhibition on experimental diabetic autonomic neuropathy publication-title: J. Neuropathol. Exp. Neurol. – volume: 44 start-page: 276 year: 1971 end-page: 287 article-title: Superoxide dismutase: improved assay and an assay applicable to acrylamide gels publication-title: Anal Biochem. – volume: 195 start-page: 133 year: 1952 end-page: 140 article-title: A spectrophotometric method for measuring the breakdown of hydrogen peroxide by catalase publication-title: J. Biol. Chem. – volume: 5 start-page: 35 year: 1993 end-page: 38 article-title: Deletion of the serine 34 codon from the major peripheral myelin protein P0 gene in Charcot‐Marie Tooth disease type 1B publication-title: Nat Genet – volume: 151 start-page: 611 year: 1997 end-page: 620 article-title: Pathological characterization of astrocytic hyaline inclusions in familial amyotrophic lateral sclerosis publication-title: Am. J. Pathol. – volume: 26 start-page: 313 year: 2003 end-page: 317 article-title: Anti‐hyperglycemic Effect of Fangchinoline Isolated from Stephania Tetrandra Radix in Streptozotocin‐Diabetic Mice publication-title: Biol. Pharm. Bull. – ident: e_1_2_6_14_1 doi: 10.1016/0092-8674(92)90591-Y – volume: 16 start-page: 6386 year: 1996 ident: e_1_2_6_33_1 article-title: Peripheral neuropathy in mice transgenic for a human MDR3 P‐glycoprotein mini‐gene publication-title: J. Neurosci. doi: 10.1523/JNEUROSCI.16-20-06386.1996 contributor: fullname: Smit J.J.M. – volume: 110 start-page: 723 year: 1985 ident: e_1_2_6_32_1 article-title: The locus encoding alpha A‐crystallin is closely linked to H‐2K on mouse chromosome 17 publication-title: Genetics doi: 10.1093/genetics/110.4.723 contributor: fullname: Skow L.C. – start-page: 1751 volume-title: The Metabolic Basis of Inherited Disease year: 1989 ident: e_1_2_6_9_1 contributor: fullname: Desnick R.J. – ident: e_1_2_6_19_1 doi: 10.1007/BF01790973 – ident: e_1_2_6_18_1 doi: 10.5414/CPP38094 – ident: e_1_2_6_34_1 doi: 10.1016/0165-4608(94)90082-5 – ident: e_1_2_6_35_1 doi: 10.1038/356241a0 – ident: e_1_2_6_26_1 doi: 10.1046/j.1365-2613.2000.00161.x – ident: e_1_2_6_8_1 doi: 10.1074/jbc.271.30.17903 – ident: e_1_2_6_30_1 doi: 10.1007/BF01235758 – ident: e_1_2_6_3_1 doi: 10.1016/0003-2697(71)90370-8 – start-page: 1094 volume-title: Hereditary, Motor and Sensory Neuropathies. year: 1992 ident: e_1_2_6_10_1 contributor: fullname: Dyck P.J. – ident: e_1_2_6_12_1 doi: 10.1007/BF00995383 – ident: e_1_2_6_36_1 doi: 10.1002/ana.410180302 – ident: e_1_2_6_4_1 doi: 10.1016/S0021-9258(19)50881-X – ident: e_1_2_6_5_1 doi: 10.1016/S0006-291X(89)80215-3 – volume: 231 start-page: 119 year: 1988 ident: e_1_2_6_11_1 article-title: Reaching for function of tissue transglutaminase; its possible involvement in biochemical pathway of programmed cell death publication-title: Adv. Exp. Biol. contributor: fullname: Fesus L. – ident: e_1_2_6_20_1 doi: 10.2131/jts.25.137 – ident: e_1_2_6_25_1 doi: 10.1111/j.1471-4159.1992.tb10051.x – ident: e_1_2_6_39_1 doi: 10.1016/0896-6273(95)90259-7 – ident: e_1_2_6_7_1 doi: 10.1126/science.877544 – ident: e_1_2_6_17_1 doi: 10.1016/S0378-1119(98)00103-6 – ident: e_1_2_6_40_1 doi: 10.1016/0888-7543(91)90370-T – start-page: 381 volume-title: Progress in Brain Research: Anatomy of the Descending Pathways to the Spinal Cord year: 1982 ident: e_1_2_6_23_1 contributor: fullname: Kuypers H.G.J.M. – start-page: 391 volume-title: Handbook of Clinical Neurology year: 1986 ident: e_1_2_6_31_1 contributor: fullname: Seitelberger F. – ident: e_1_2_6_16_1 doi: 10.1073/pnas.95.9.5161 – ident: e_1_2_6_37_1 doi: 10.1248/bpb.26.313 – ident: e_1_2_6_27_1 doi: 10.1056/NEJM198906293202606 – ident: e_1_2_6_2_1 doi: 10.1016/S0022-510X(00)00321-X – ident: e_1_2_6_28_1 doi: 10.1097/00005072-199812000-00010 – ident: e_1_2_6_22_1 doi: 10.1038/ng0993-35 – ident: e_1_2_6_38_1 doi: 10.1016/0022-510X(95)00124-K – ident: e_1_2_6_15_1 doi: 10.1046/j.1471-4159.1995.64031216.x – ident: e_1_2_6_6_1 doi: 10.1093/nar/3.9.2303 – ident: e_1_2_6_13_1 doi: 10.1023/A:1022442904179 – ident: e_1_2_6_29_1 doi: 10.1111/j.1399-3011.1969.tb01623.x – volume: 151 start-page: 611 year: 1997 ident: e_1_2_6_21_1 article-title: Pathological characterization of astrocytic hyaline inclusions in familial amyotrophic lateral sclerosis publication-title: Am. J. Pathol. contributor: fullname: Kato S. – ident: e_1_2_6_24_1 doi: 10.1515/bchm3.1989.370.2.661 |
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| SubjectTerms | Biological and medical sciences Degenerative and inherited degenerative diseases of the nervous system. Leukodystrophies. Prion diseases Medical sciences Neurology Original |
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| Title | Pathogenesis of axonal dystrophy and demyelination in αA-crystallin-expressing transgenic mice |
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