Cln8

Summary

Gene Symbol: Cln8
Description: ceroid-lipofuscinosis, neuronal 8
Alias: mnd, protein CLN8, motor neuron degeneration
Species: mouse
Products:     Cln8

Top Publications

  1. Messer A, Plummer J, MacMillen M, Frankel W. Genetics of primary and timing effects in the mnd mouse. Am J Med Genet. 1995;57:361-4 pubmed
    The mnd mouse shows a spontaneous adult-onset hereditary neurological disease, with motor abnormality by 6 months of age, progressing to severe spastic paralysis and premature death...
  2. Messer A. Mutant mouse models of ALS. Neurobiol Aging. 1994;15:247-8 pubmed
  3. Battaglioli G, Martin D, Plummer J, Messer A. Synaptosomal glutamate uptake declines progressively in the spinal cord of a mutant mouse with motor neuron disease. J Neurochem. 1993;60:1567-9 pubmed
    ..The role of synaptosomal glutamate uptake in the pathogenesis of motor neuron disease was studied in the Mnd mouse...
  4. Chang B, Bronson R, Hawes N, Roderick T, Peng C, Hageman G, et al. Retinal degeneration in motor neuron degeneration: a mouse model of ceroid lipofuscinosis. Invest Ophthalmol Vis Sci. 1994;35:1071-6 pubmed
    To evaluate the retinal degeneration of the motor neuron degeneration (mnd) mouse, and to confirm its inheritance pattern and gene location...
  5. Bennett M, Boriack R, Birch D. In-utero and post-delivery supplementation of motor neuron degeneration mutant mice with polyunsaturated fatty acids does not alter the clinical or pathological course. Neuropediatrics. 1997;28:82-4 pubmed
    We have studied the effects of polyunsaturated fatty acid (PUFA) supplementation in utero and throughout life in mnd mutant mice, a proposed model for juvenile neuronal ceroid lipofuscinosis (CLN-3)...
  6. Callahan L, Wylen E, Messer A, Mazurkiewicz J. Neurofilament distribution is altered in the Mnd (motor neuron degeneration) mouse. J Neuropathol Exp Neurol. 1991;50:491-504 pubmed
    b>Motor neuron degeneration (Mnd) is a genetic neurodegenerative disease of the mouse that is characterized by a progressive increase in motor dysfunction, moving from hind to fore limbs, leading to paralysis...
  7. Cooper J, Messer A, Feng A, Chua Couzens J, Mobley W. Apparent loss and hypertrophy of interneurons in a mouse model of neuronal ceroid lipofuscinosis: evidence for partial response to insulin-like growth factor-1 treatment. J Neurosci. 1999;19:2556-67 pubmed
    ..The mnd/mnd mutant mouse was first characterized as exhibiting adult-onset upper and lower motor neuron degeneration, but closer examination revealed early, widespread pathology similar to that seen in NCL...
  8. Mennini T, Bigini P, Ravizza T, Vezzani A, Calvaresi N, Tortarolo M, et al. Expression of glutamate receptor subtypes in the spinal cord of control and mnd mice, a model of motor neuron disorder. J Neurosci Res. 2002;70:553-60 pubmed
    We studied the expression and distribution of glutamate receptor subtypes in the spinal cord of mnd mice, a model of motor neuron disorders and neuronal ceroid lipofuscinosis, and control mice using immunocytochemistry and in situ ..
  9. Messer A, Plummer J. Accumulating autofluorescent material as a marker for early changes in the spinal cord of the Mnd mouse. Neuromuscul Disord. 1993;3:129-34 pubmed
    The mouse mutant Motor neuron degeneration (Mnd) displays an adult-onset progressive degeneration of upper and lower motor neurons, with mild symptoms recognizable at 6 months, leading to spastic paralysis and premature death at 10-12 ..
  10. Bronson R, Lake B, Cook S, Taylor S, Davisson M. Motor neuron degeneration of mice is a model of neuronal ceroid lipofuscinosis (Batten's disease). Ann Neurol. 1993;33:381-5 pubmed
    Pathological studies of mice homozygous for the motor neuron degeneration (Mnd) mutation show abnormalities similar to those of the human neuronal ceroid lipofuscinoses: sudanophilic, autofluorescent intraneuronal inclusions that are ..

Detail Information

Publications60

  1. Messer A, Plummer J, MacMillen M, Frankel W. Genetics of primary and timing effects in the mnd mouse. Am J Med Genet. 1995;57:361-4 pubmed
    The mnd mouse shows a spontaneous adult-onset hereditary neurological disease, with motor abnormality by 6 months of age, progressing to severe spastic paralysis and premature death...
  2. Messer A. Mutant mouse models of ALS. Neurobiol Aging. 1994;15:247-8 pubmed
  3. Battaglioli G, Martin D, Plummer J, Messer A. Synaptosomal glutamate uptake declines progressively in the spinal cord of a mutant mouse with motor neuron disease. J Neurochem. 1993;60:1567-9 pubmed
    ..The role of synaptosomal glutamate uptake in the pathogenesis of motor neuron disease was studied in the Mnd mouse...
  4. Chang B, Bronson R, Hawes N, Roderick T, Peng C, Hageman G, et al. Retinal degeneration in motor neuron degeneration: a mouse model of ceroid lipofuscinosis. Invest Ophthalmol Vis Sci. 1994;35:1071-6 pubmed
    To evaluate the retinal degeneration of the motor neuron degeneration (mnd) mouse, and to confirm its inheritance pattern and gene location...
  5. Bennett M, Boriack R, Birch D. In-utero and post-delivery supplementation of motor neuron degeneration mutant mice with polyunsaturated fatty acids does not alter the clinical or pathological course. Neuropediatrics. 1997;28:82-4 pubmed
    We have studied the effects of polyunsaturated fatty acid (PUFA) supplementation in utero and throughout life in mnd mutant mice, a proposed model for juvenile neuronal ceroid lipofuscinosis (CLN-3)...
  6. Callahan L, Wylen E, Messer A, Mazurkiewicz J. Neurofilament distribution is altered in the Mnd (motor neuron degeneration) mouse. J Neuropathol Exp Neurol. 1991;50:491-504 pubmed
    b>Motor neuron degeneration (Mnd) is a genetic neurodegenerative disease of the mouse that is characterized by a progressive increase in motor dysfunction, moving from hind to fore limbs, leading to paralysis...
  7. Cooper J, Messer A, Feng A, Chua Couzens J, Mobley W. Apparent loss and hypertrophy of interneurons in a mouse model of neuronal ceroid lipofuscinosis: evidence for partial response to insulin-like growth factor-1 treatment. J Neurosci. 1999;19:2556-67 pubmed
    ..The mnd/mnd mutant mouse was first characterized as exhibiting adult-onset upper and lower motor neuron degeneration, but closer examination revealed early, widespread pathology similar to that seen in NCL...
  8. Mennini T, Bigini P, Ravizza T, Vezzani A, Calvaresi N, Tortarolo M, et al. Expression of glutamate receptor subtypes in the spinal cord of control and mnd mice, a model of motor neuron disorder. J Neurosci Res. 2002;70:553-60 pubmed
    We studied the expression and distribution of glutamate receptor subtypes in the spinal cord of mnd mice, a model of motor neuron disorders and neuronal ceroid lipofuscinosis, and control mice using immunocytochemistry and in situ ..
  9. Messer A, Plummer J. Accumulating autofluorescent material as a marker for early changes in the spinal cord of the Mnd mouse. Neuromuscul Disord. 1993;3:129-34 pubmed
    The mouse mutant Motor neuron degeneration (Mnd) displays an adult-onset progressive degeneration of upper and lower motor neurons, with mild symptoms recognizable at 6 months, leading to spastic paralysis and premature death at 10-12 ..
  10. Bronson R, Lake B, Cook S, Taylor S, Davisson M. Motor neuron degeneration of mice is a model of neuronal ceroid lipofuscinosis (Batten's disease). Ann Neurol. 1993;33:381-5 pubmed
    Pathological studies of mice homozygous for the motor neuron degeneration (Mnd) mutation show abnormalities similar to those of the human neuronal ceroid lipofuscinoses: sudanophilic, autofluorescent intraneuronal inclusions that are ..
  11. Winter E, Ponting C. TRAM, LAG1 and CLN8: members of a novel family of lipid-sensing domains?. Trends Biochem Sci. 2002;27:381-3 pubmed
    ..The family includes the protein product of CLN8, a gene mutated in progressive epilepsy with mental retardation...
  12. Messer A, Flaherty L. Autosomal dominance in a late-onset motor neuron disease in the mouse. J Neurogenet. 1986;3:345-55 pubmed
    ..In outcrosses to wild-type, symptoms have been observed in all obligate heterozygotes, with a similar age range for onset to that of homozygotes. We have designated this autosomal dominant disorder Motor neuron degeneration (Mnd).
  13. Ranta S, Zhang Y, Ross B, Lonka L, Takkunen E, Messer A, et al. The neuronal ceroid lipofuscinoses in human EPMR and mnd mutant mice are associated with mutations in CLN8. Nat Genet. 1999;23:233-6 pubmed
    ..CLN8, conservation of the codon harbouring the human mutation and is localized to the same region as the motor neuron degeneration mouse, mnd, a naturally occurring mouse NCL (ref. 4)...
  14. Mazurkiewicz J, Callahan L, Swash M, Martin J, Messer A. Cytoplasmic inclusions in spinal neurons of the motor neuron degeneration (Mnd) mouse. I. Light microscopic analysis. J Neurol Sci. 1993;116:59-66 pubmed
    The motor neuron degeneration (Mnd) mutation in the mouse is a late onset, autosomal dominant, neurodegenerative disease in which ventral horn neurons have been shown to contain numerous, large cytoplasmic inclusions...
  15. Lonka L, Salonen T, Siintola E, Kopra O, Lehesjoki A, Jalanko A. Localization of wild-type and mutant neuronal ceroid lipofuscinosis CLN8 proteins in non-neuronal and neuronal cells. J Neurosci Res. 2004;76:862-71 pubmed
    ..Mutations in the CLN8 gene underlie Northern epilepsy (progressive epilepsy with mental retardation [EPMR], OMIM 600143) and a subset of ..
  16. Messer A, Plummer J, Wong V, LaVail M. Retinal degeneration in motor neuron degeneration (mnd) mutant mice. Exp Eye Res. 1993;57:637-41 pubmed
  17. Mennini T, Bastone A, Crespi D, Comoletti D, Manzoni C. Spinal cord GLT-1 glutamate transporter and blood glutamic acid alterations in motor neuron degeneration (Mnd) mice. J Neurol Sci. 1998;157:31-6 pubmed
    This study characterizes for the first time neurochemical mechanisms in Mnd mice, initially described as a model of motor neuron disease and more recently proposed as a model for neuronal ceroid lipofuscinosis...
  18. Bihl F, Lariviere L, Qureshi S, Flaherty L, Malo D. LPS-hyporesponsiveness of mnd mice is associated with a mutation in Toll-like receptor 4. Genes Immun. 2001;2:56-9 pubmed
    ..In this paper, we report that C57BL/6.KB2-mnd mice, a model of neuronal ceroid lipofuscinosis, do not respond to LPS...
  19. Elliott J. Experimental models of amyotrophic lateral sclerosis. Neurobiol Dis. 1999;6:310-20 pubmed
    ..Recently, however, a variety of newer model systems have been developed and utilized to investigate motor neuron degeneration as related to ALS. For example, in this issue, Corse et al...
  20. Bolivar V, Scott Ganus J, Messer A. The development of behavioral abnormalities in the motor neuron degeneration (mnd) mouse. Brain Res. 2002;937:74-82 pubmed
    The motor neuron degeneration (mnd) mouse, which has widespread abnormal accumulating lipoprotein and neuronal degeneration, has a mutation in CLN8, the gene for human progressive epilepsy with mental retardation (EPMR)...
  21. Tanner A, Dice J. Batten disease fibroblasts in culture accumulate mitochondrial ATP synthase subunit 9. Cell Biol Int. 1995;19:71-5 pubmed
    ..However, the exact growth state of the human fibroblasts was critical, and this factor probably accounts for discrepencies in the literature. ..
  22. Li J, Nixon R, Messer A, Berman S, Bursztajn S. Altered gene expression for calpain/calpastatin system in motor neuron degeneration (Mnd) mutant mouse brain and spinal cord. Brain Res Mol Brain Res. 1998;53:174-86 pubmed
    ..the in situ mRNA expression of calpain I and II and their endogenous inhibitor, calpastatin, in the motor neuron degeneration (Mnd) mutant mouse, which exhibits progressive dysfunction of the spinal cord and brain...
  23. Bakalian A, Kopmels B, Messer A, Fradelizi D, Delhaye Bouchaud N, Wollman E, et al. Peripheral macrophage abnormalities in mutant mice with spinocerebellar degeneration. Res Immunol. 1992;143:129-39 pubmed
    ..phenomenon was found, but to a lesser extent, in the spinal mutants dystonia musculorum, wobbler and motor neuron degeneration. On the contrary, no hyperexpression of IL1 beta mRNA was found in non-genetic models of neuronal ..
  24. Plummer J, Peterson A, Messer A. Accelerated and widespread neuronal loss occurs in motor neuron degeneration (mnd) mice expressing a neurofilament-disrupting transgene. Mol Cell Neurosci. 1995;6:532-43 pubmed
    ..ubiquitous accumulation of autofluorescent lipopigment, with retinal degeneration and late-onset motor neuron degeneration. The neurofilament H-beta-galactosidase fusion protein causes endogenous neurofilament subunits to ..
  25. Fujita K, Shibayama K, Yamauchi M, Kato T, Ando M, Takahashi H, et al. Alteration of enzymatic activities implicating neuronal degeneration in the spinal cord of the motor neuron degeneration mouse during postnatal development. Neurochem Res. 1998;23:557-62 pubmed
    ..CO), superoxide dismutase (SOD), and transglutaminase (TG) in spinal cord of an animal model of ALS, motor neuron degeneration (Mnd) mouse, a mutant that exhibits progressive degeneration of lower spinal neurons during ..
  26. Galizzi G, Russo D, Deidda I, Cascio C, Passantino R, Guarneri R, et al. Different early ER-stress responses in the CLN8(mnd) mouse model of neuronal ceroid lipofuscinosis. Neurosci Lett. 2011;488:258-62 pubmed publisher
    ..In the motor neuron degeneration (mnd) mouse model of the CLN8-LINCL (CLN8(mnd)), we carried out an analysis of ER stress-related ..
  27. Horvath D, Watson J, Travis G. Probable exclusion of the cortexin-encoding gene as a candidate for mouse neurological mutants: nervous, tottering and motor neuron degeneration. Gene. 1996;171:305-6 pubmed
    ..for the mouse neurological mutants: nervous (nr), tottering (tg) plus tottering-leaner (tgla), and motor neuron degeneration (mnd) by Northern blot analysis of brain poly(A)+ RNA and direct polymerase chain reaction (PCR) ..
  28. Bigini P, Milanese M, Gardoni F, Longhi A, Bonifacino T, Barbera S, et al. Increased [³H]D-aspartate release and changes in glutamate receptor expression in the hippocampus of the mnd mouse. J Neurosci Res. 2012;90:1148-58 pubmed publisher
    ..The mnd mouse, carrying a mutation in the Cln8 gene, has been proposed as a model of epilepsy with mental retardation (EPMR, ornorthern epilepsy)...
  29. Hawes N, Smith R, Chang B, Davisson M, Heckenlively J, John S. Mouse fundus photography and angiography: a catalogue of normal and mutant phenotypes. Mol Vis. 1999;5:22 pubmed
    ..images for C57BL/6J, BALB/cByJ, retinal degeneration 1 (rd1), Rd2, rd3, rd7, achondroplasia, nervous, motor neuron degeneration, Purkinje cell degeneration, kidney and retinal defects, optic nerve coloboma 1, and two apparently ..
  30. Kuronen M, Hermansson M, Manninen O, Zech I, Talvitie M, Laitinen T, et al. Galactolipid deficiency in the early pathogenesis of neuronal ceroid lipofuscinosis model Cln8mnd : implications to delayed myelination and oligodendrocyte maturation. Neuropathol Appl Neurobiol. 2012;38:471-86 pubmed publisher
    b>CLN8 deficiency underlies one of a group of devastating childhood neurodegenerative disorders, the neuronal ceroid lipofuscinoses. The function of the CLN8 protein is currently unknown, but a role in lipid metabolism has been proposed...
  31. Kuronen M, Lehesjoki A, Jalanko A, Cooper J, Kopra O. Selective spatiotemporal patterns of glial activation and neuron loss in the sensory thalamocortical pathways of neuronal ceroid lipofuscinosis 8 mice. Neurobiol Dis. 2012;47:444-57 pubmed publisher
    ..Here, we have characterized the timing and regional-specificity of the pathological events of CLN8 disease utilizing the Cln8 deficient mouse model, Cln8(mnd)...
  32. Cho S, Gao N, Pearce D, Lehrman M, Hofmann S. Characterization of lipid-linked oligosaccharide accumulation in mouse models of Batten disease. Glycobiology. 2005;15:637-48 pubmed
    ..and found striking lipid-linked oligosaccharide (LLO) accumulation in NCL mouse models (especially CLN1, CLN6, and CLN8 knockout or mutant mice) but not in several other lysosomal storage disorders affecting the brain...
  33. Mennini T, Cagnotto A, Carvelli L, Comoletti D, Manzoni C, Muzio V, et al. Biochemical and pharmacological evidence of a functional role of AMPA receptors in motor neuron dysfunction in mnd mice. Eur J Neurosci. 1999;11:1705-10 pubmed
    ..rs)-alpha-amino-3-hydroxy-5-methyl-4-isoxazole propionic acid (AMPA) receptor antagonist, in the spinal cord of mnd mice. NBQX (8 mg/kg daily i.p...
  34. Seigel G, Wagner J, Wronska A, Campbell L, Ju W, Zhong N. Progression of early postnatal retinal pathology in a mouse model of neuronal ceroid lipofuscinosis. Eye (Lond). 2005;19:1306-12 pubmed
    ..well as potential therapeutic responses, we followed the course of postnatal retinal pathology in tissues from the CLN8 (mnd) mouse model of NCL...
  35. Frey D, Schneider C, Xu L, Borg J, Spooren W, Caroni P. Early and selective loss of neuromuscular synapse subtypes with low sprouting competence in motoneuron diseases. J Neurosci. 2000;20:2534-42 pubmed
  36. Boyce S, Webb J, Carlson E, Rupniak N, Hill R, Martin J. Onset and progression of motor deficits in motor neuron degeneration (mnd) mice are unaltered by the glycine/NMDA receptor antagonist L-701,324 or the MAO-B inhibitor R(-)-deprenyl. Exp Neurol. 1999;155:49-58 pubmed
    ..oxidase B inhibitor (r)-deprenyl on the onset and rate of progression of neurological impairment in the motor neuron degeneration (mnd) mouse, a murine model of neurodegeneration...
  37. Messer A, Plummer J, Maskin P, Coffin J, Frankel W. Mapping of the motor neuron degeneration (Mnd) gene, a mouse model of amyotrophic lateral sclerosis (ALS). Genomics. 1992;13:797-802 pubmed
    The motor neuron degeneration mutation (Mnd) causes a late-onset, progressive degeneration of upper and lower motor neurons in mice...
  38. Messer A, Manley K, Plummer J. An early-onset congenic strain of the motor neuron degeneration (mnd) mouse. Mol Genet Metab. 1999;66:393-7 pubmed
    The mouse mutant motor neuron degeneration (mnd/mnd) has been proposed as a model of neuronal ceroid lipofuscinosis (NCL) on the basis of widespread abnormal accumulating lipopigment and neuronal and retinal degeneration...
  39. Oh C, Murray B, Bhattacharya N, Holland D, Tatton W. (-)-Deprenyl alters the survival of adult murine facial motoneurons after axotomy: increases in vulnerable C57BL strain but decreases in motor neuron degeneration mutants. J Neurosci Res. 1994;38:64-74 pubmed
    ..three different strains of adult mice--A/J, C57BL/6J, and a congenic substrain of the C57BL/6J mice, the C57BL/Mnd mutants--underwent unilateral facial nerve transection...
  40. Lyly A, von Schantz C, Heine C, Schmiedt M, Sipilä T, Jalanko A, et al. Novel interactions of CLN5 support molecular networking between Neuronal Ceroid Lipofuscinosis proteins. BMC Cell Biol. 2009;10:83 pubmed publisher
    ..We found that CLN5 interacts with several other NCL proteins namely, CLN1/PPT1, CLN2/TPP1, CLN3, CLN6 and CLN8. Furthermore, analysis of the intracellular targeting of CLN5 together with the interacting NCL proteins revealed ..
  41. Pardo C, Rabin B, Palmer D, Price D. Accumulation of the adenosine triphosphate synthase subunit C in the mnd mutant mouse. A model for neuronal ceroid lipofuscinosis. Am J Pathol. 1994;144:829-35 pubmed
    The motor neuron degeneration (mnd) mutant mouse, initially described as an autosomal semidominant model of motor neuron disease, is characterized by progressive loss of motor activities and the accumulation of lipofuscin-like material ..
  42. Mennini T, Bigini P, Cagnotto A, Carvelli L, Di Nunno P, Fumagalli E, et al. Glial activation and TNFR-I upregulation precedes motor dysfunction in the spinal cord of mnd mice. Cytokine. 2004;25:127-35 pubmed
    Mice homozygous for the spontaneous motor neuron degeneration mutation (mnd) show at the age of 8 months a marked impairment of the motor function and accumulation of lipofuscin granules in the cytoplasm of almost all neurons of the ..
  43. Palmer D, Barry L, Tyynelä J, Cooper J. NCL disease mechanisms. Biochim Biophys Acta. 2013;1832:1882-93 pubmed publisher
    ..This article is part of a Special Issue entitled: The Neuronal Ceroid Lipofuscinoses or Batten Disease. ..
  44. Kolikova J, Afzalov R, Surin A, Lehesjoki A, Khiroug L. Deficient mitochondrial Ca(2+) buffering in the Cln8(mnd) mouse model of neuronal ceroid lipofuscinosis. Cell Calcium. 2011;50:491-501 pubmed publisher
    ..fluorescence imaging and caged Ca(2+) photolysis, we evaluated the mechanisms of neuronal Ca(2+) clearance in Cln8(mnd) mice, a model of the human NCL caused by mutations in the CLN8 gene...
  45. Vance J, Stone S, Faust J. Abnormalities in mitochondria-associated membranes and phospholipid biosynthetic enzymes in the mnd/mnd mouse model of neuronal ceroid lipofuscinosis. Biochim Biophys Acta. 1997;1344:286-99 pubmed
    ..Biol. Chem. 270, 11190-11198). We have now examined further the role of MAM in lipid metabolism using the mnd/mnd mouse, a model for the human degenerative disease neuronal ceroid lipofuscinosis...
  46. Chang B, Hawes N, Hurd R, Davisson M, Nusinowitz S, Heckenlively J. Retinal degeneration mutants in the mouse. Vision Res. 2002;42:517-25 pubmed
    ..pcd); nervous (nr); retinal degeneration slow (rds, now Prph(Rd2)); retinal degeneration 3 (rd3); motor neuron degeneration (mnd); retinal degeneration 4 (Rd4); retinal degeneration 5 (rd5, now tub); vitiligo (vit, now Mitf(mi-..
  47. Ghezzi P, Bernardini R, Giuffrida R, Bellomo M, Manzoni C, Comoletti D, et al. Tumor necrosis factor is increased in the spinal cord of an animal model of motor neuron degeneration. Eur Cytokine Netw. 1998;9:139-44 pubmed
    ..encephalomyelitis, and can be neurotoxic, we studied TNF production in a proposed animal model of ALS, the mnd mouse. These mice develop symptoms (progressive weakness of the limbs) as late as at 7 months of age...
  48. Fujita K, Yamauchi M, Matsui T, Titani K, Takahashi H, Kato T, et al. Increase of glial fibrillary acidic protein fragments in the spinal cord of motor neuron degeneration mutant mouse. Brain Res. 1998;785:31-40 pubmed
    We analyzed protein fractions extracted from the spinal cord of the motor neuron degeneration (Mnd) mouse, a mutant that exhibits progressive degeneration of lower spinal motor neurons, by one- and two-dimensional polyacrylamide gel ..
  49. Griffin J, Muller D, Woograsingh R, Jowatt V, Hindmarsh A, Nicholson J, et al. Vitamin E deficiency and metabolic deficits in neuronal ceroid lipofuscinosis described by bioinformatics. Physiol Genomics. 2002;11:195-203 pubmed
    The mnd mouse, a model of neuronal ceroid lipofusinosis (NCL), has a profound vitamin E deficiency in sera and brain, associated with cerebral deterioration characteristic of NCL...
  50. Encinas J, Lees M, Sobel R, Symonowicz C, Weiner H, Seidman C, et al. Identification of genetic loci associated with paralysis, inflammation and weight loss in mouse experimental autoimmune encephalomyelitis. Int Immunol. 2001;13:257-64 pubmed
    ..2 x 10(-6)) and the incidence of CNS inflammation (P = 7.0 x 10(-5)) in EAE. ..
  51. Bermingham N, Martin J, Fisher E. The mouse lysosomal membrane protein 1 gene as a candidate for the motorneuron degeneration (mnd) locus. Genomics. 1996;32:266-71 pubmed
    The motorneuron degeneration (mnd) mutation causes one of the few late-onset progressive neurodegenerations in mice; therefore, the mnd mouse is a valuable paradigm for studying neurodegenerative biology...
  52. Guarneri R, Russo D, Cascio C, D Agostino S, Galizzi G, Bigini P, et al. Retinal oxidation, apoptosis and age- and sex-differences in the mnd mutant mouse, a model of neuronal ceroid lipofuscinosis. Brain Res. 2004;1014:209-20 pubmed
    ..We here used the mutant motor neuron degeneration (mnd) mouse, a late-infantile NCL variant, to investigate the retinal oxidative state and apoptotic cell ..
  53. Wendt K, Lei B, Schachtman T, Tullis G, Ibe M, Katz M. Behavioral assessment in mouse models of neuronal ceroid lipofuscinosis using a light-cued T-maze. Behav Brain Res. 2005;161:175-82 pubmed
    ..Murine models for the neuronal ceroid lipofuscinoses include the well-characterized motor neuron degeneration (mnd/mnd) model for one variant of late infantile NCL (CLN8), and the more recently generated models for ..
  54. Holmes F, Haynes L. Superactivation of transglutaminase type 2 without change in enzyme level occurs during progressive neurodegeneration in the mnd mouse mutant. Neurosci Lett. 1996;213:185-8 pubmed
    We have investigated the activity of the Ca(2+)-dependent apoptosis-related transglutaminase type 2 in the mnd/mnd mouse mutant...
  55. Green S, Tolwani R. Animal models for motor neuron disease. Lab Anim Sci. 1999;49:480-7 pubmed
    ..Animal models of motor neuron disease (MND) have significantly contributed to the remarkable recent progress in understanding the cause, genetic factors, and ..
  56. Porter J, Messer A, Peterson A. The motor neuron degeneration (mnd) gene acts intrinsically in motor neurons and peripheral fibroblasts. Mol Cell Neurosci. 1997;9:185-93 pubmed
    In motor neuron degeneration (mnd/mnd) mice, multiple cell types develop cytopathology and motor neurons degenerate prematurely...
  57. Faust J, Rodman J, Daniel P, Dice J, Bronson R. Two related proteolipids and dolichol-linked oligosaccharides accumulate in motor neuron degeneration mice (mnd/mnd), a model for neuronal ceroid lipofuscinosis. J Biol Chem. 1994;269:10150-5 pubmed
    ..study, we show that two biochemical markers of neuronal ceroid lipofuscinoses (NCLs) are present in a mutant mouse (mnd/mnd) that exhibits symptoms of the disease...
  58. Bertamini M, Marzani B, Guarneri R, Guarneri P, Bigini P, Mennini T, et al. Mitochondrial oxidative metabolism in motor neuron degeneration (mnd) mouse central nervous system. Eur J Neurosci. 2002;16:2291-6 pubmed
    The mnd mouse spontaneously develops slowly evolving motoneuron pathology leading to progressive motor impairment...
  59. Lonka L, Aalto A, Kopra O, Kuronen M, Kokaia Z, Saarma M, et al. The neuronal ceroid lipofuscinosis Cln8 gene expression is developmentally regulated in mouse brain and up-regulated in the hippocampal kindling model of epilepsy. BMC Neurosci. 2005;6:27 pubmed
    ..phenotype is characterized by epilepsy, progressive psychomotor deterioration and visual loss, while motor neuron degeneration (mnd) mice with a Cln8 mutation show progressive motor neuron dysfunction and retinal degeneration...
  60. Mazurkiewicz J. Ubiquitin deposits are present in spinal motor neurons in all stages of the disease in the motor neuron degeneration (Mnd) mutant of the mouse. Neurosci Lett. 1991;128:182-6 pubmed
    ..antibodies to ubiquitin were used in an immunocytochemical analysis of spinal cord from the Mnd (motor neuron degeneration) mouse, an animal model for motor neuron disease...