Description

The genotypes of the animals provided may not reflect those discussed in the strain description or the mating scheme utilized by The Jackson Laboratory prior to cryopreservation. Please inquire for possible genotypes for this specific strain.

Strain Information

Type Congenic; Mutant Strain; Additional information on Genetically Engineered and Mutant Mice. Visit our online Nomenclature tutorial. Additional information on Congenic nomenclature. Species laboratory mouse Background Strain AKR/J Donor Strain C57BL/6.KB2/Rn Generation N13F4p (27-JUN-04) Generation Definitions

Appearance
albino
Related Genotype: a/a Tyr^c/Tyr^c

Control Information

	Control
	Heterozygote from the colony
Considerations for Choosing Controls

Related Strains

Strains carrying   Cln8^mnd allele

001612

B6.KB2-Cln8mnd/MsrJ

View Strains carrying   Cln8^mnd     (1 strain)

Strains carrying other alleles of Cln8

001612

B6.KB2-Cln8mnd/MsrJ

View Strains carrying other alleles of Cln8     (1 strain)

Phenotype

Phenotype Information

View Related Disease (OMIM) Terms

Related Disease (OMIM) Terms provided by MGI

Ceroid Lipofuscinosis, Neuronal, 8; CLN8 - Models with phenotypic similarity to human disease where etiologies involve orthologs.1

1 Human genes are associated with this disease. Orthologs of those genes appear in the mouse genotype(s).

View Mammalian Phenotype Terms

Mammalian Phenotype Terms provided by MGI

      assigned by genotype

Cln8^mnd/Cln8^mnd

        AK.B6(Cg)-Cln8^mnd

• mortality/aging
• premature death
  • die by 5.5 months of age   (MGI Ref ID J:56219)
• nervous system phenotype
• abnormal motor neuron morphology
  • motor neuron disease is accelerated even more than in the mixed AKR/J and B6.KB2 background, with symptoms appearing by 4 months and death by 5.5 months of age   (MGI Ref ID J:56219)

The following phenotype information may relate to a genetic background differing from this JAX^® Mice strain.

Cln8^mnd/Cln8⁺

        involves: B6.KB2 * C57BL/6Fla

• nervous system phenotype
• abnormal motor neuron morphology
  • exhibit neurological disease, with onset of mild symptoms at an age (5 months) similar to homozygotes   (MGI Ref ID J:8492)
  • slower emergence of more severe motor neuron disease symptoms than in homozygotes   (MGI Ref ID J:8492)

Cln8^mnd/Cln8^mnd

        B6.KB2/Rn-Cln8^mnd

• mortality/aging
• premature death   (MGI Ref ID J:56219)
  • most die by 9-14 months of age   (MGI Ref ID J:8492)
• behavior/neurological phenotype
• abnormal motor capabilities/coordination/movement
  • hindlimbs are unable to grasp the bars of wire cagetop when attempting to walk over it   (MGI Ref ID J:8492)
• • abnormal locomotor behavior
    • unable to walk uphill   (MGI Ref ID J:8492)
    • dragging or splaying of the hindlimbs while walking   (MGI Ref ID J:8492)
  • • ataxia
      • age of onset is approximately 5- 11 months of age, progressive with age   (MGI Ref ID J:8492)
  • limb grasping   (MGI Ref ID J:8492)
  • paralysis
    • progress to severe spastic paresis and paralysis by 9 months of age   (MGI Ref ID J:12816)
  • • hindlimb paralysis
      • age of onset is approximately 5- 11 months of age, progressive with age   (MGI Ref ID J:8492)
  • paresis
    • begin to develop paresis by 6 months of age   (MGI Ref ID J:12816)
• nervous system phenotype
• abnormal nervous system morphology
  • contain LFB-positive intracytoplasmic inclusion material in most neurons in virtually all parts of the brain and spinal cord unlike in controls   (MGI Ref ID J:12816)
• • abnormal cranial nerve morphology
    • degeneration of cranial nerves   (MGI Ref ID J:8492)
  • • abnormal hypoglossal nerve morphology
      • degenerating   (MGI Ref ID J:8492)
    • abnormal vagus nerve morphology
      • degenerating dorsal motor vagus   (MGI Ref ID J:8492)
  • abnormal motor neuron morphology
    • inclusion bodies containing ubiquitin were found in spinal neurons of mnd mice, even prior to onset of symptoms   (MGI Ref ID J:8492)
    • motor neurons have eccentric or indiscrete nuclei, disrupted membranes and shape changes   (MGI Ref ID J:8492)
    • exhibit motor neuron disease symptoms at around 6 months of age   (MGI Ref ID J:56219)
  • • motor neuron degeneration
      • degeneration of the upper and lower motor neurons of the spinal cord and cranial nerves and of some areas of the brain   (MGI Ref ID J:8492)
  • abnormal spinal cord ventral horn morphology
    • degeneration of anterior horn cells in the spinal cord   (MGI Ref ID J:8492)
• reproductive system phenotype
• decreased litter size
  • lower number of progeny/litter and lower numbers of total litters   (MGI Ref ID J:8492)
• vision/eye phenotype
• abnormal retinal photoreceptor layer morphology
  • atrophy of the photoreceptor layer in mice older than 3 months and nearly complete loss of this layer by 8 months of age   (MGI Ref ID J:12816)
• blindness
  • begin to become blind by 2 months of age and by 5 months, are completely blind   (MGI Ref ID J:12816)

Cln8^mnd/Cln8^mnd

        involves: AKR/J * B6.KB2

• mortality/aging
• premature death   (MGI Ref ID J:56219)
  • death by 7 months of age, compared to 9-14 months on the inbred B6.KB2 background   (MGI Ref ID J:1224)
• vision/eye phenotype
• abnormal retinal photoreceptor layer morphology   (MGI Ref ID J:19328)
• • abnormal retinal rod cell inner segment morphology
    • rapid thinning of the rod inner segment layer by P25, with a more gradual thinning at later stages   (MGI Ref ID J:19328)
  • abnormal retinal rod cell outer segment morphology
    • rapid thinning of the rod outer segment layer by P25, with a more gradual thinning at later stages   (MGI Ref ID J:19328)
  • short photoreceptor inner segment
    • at very late stages of degeneration, inner segments are shortened and broadened   (MGI Ref ID J:19328)
  • short photoreceptor outer segment
    • progressive shortening of the outer segments while maintaining relatively normal structure   (MGI Ref ID J:19328)
• abnormal retinal pigment epithelium morphology
  • focal thinning of the retinal pigment epithelium occurs at late stages of degeneration   (MGI Ref ID J:19328)
• retinal degeneration
  • detectable at P15 and more pronounced with age   (MGI Ref ID J:19328)
• thin retinal outer nuclear layer
  • at P15, the outer nuclear layer (ONL) contains greater number of pyknotic nuclei and is thinner   (MGI Ref ID J:19328)
  • rapid thinning of the ONL by P25, with a more gradual thinning at later ages   (MGI Ref ID J:19328)
• pigmentation phenotype
• abnormal retinal pigment epithelium morphology
  • focal thinning of the retinal pigment epithelium occurs at late stages of degeneration   (MGI Ref ID J:19328)
• nervous system phenotype
• abnormal nervous system morphology
  • earlier age of onset (4.5-5 months) and increased speed of progression of neurological disease than on the inbred B6.KB2 background   (MGI Ref ID J:1224)
• • abnormal motor neuron morphology
    • motor neuron disease is accelerated with 40% exhibiting symptoms by 4.5 months of age and dying by 6.5-7 months   (MGI Ref ID J:56219)
  • abnormal retinal rod cell inner segment morphology
    • rapid thinning of the rod inner segment layer by P25, with a more gradual thinning at later stages   (MGI Ref ID J:19328)
  • abnormal retinal rod cell outer segment morphology
    • rapid thinning of the rod outer segment layer by P25, with a more gradual thinning at later stages   (MGI Ref ID J:19328)
  • short photoreceptor inner segment
    • at very late stages of degeneration, inner segments are shortened and broadened   (MGI Ref ID J:19328)
  • short photoreceptor outer segment
    • progressive shortening of the outer segments while maintaining relatively normal structure   (MGI Ref ID J:19328)

Cln8^mnd/Cln8^mnd

        involves: B6.KB2 * C3H/HeJ

• mortality/aging
• premature death   (MGI Ref ID J:56219)
  • death similar to that seen on the inbred B6.KB2 background   (MGI Ref ID J:1224)
• nervous system phenotype
• abnormal nervous system morphology
  • neurological disease progresses in a similar fashion as on the inbred B6.KB2 background   (MGI Ref ID J:1224)
• • abnormal motor neuron morphology
    • exhibit motor neuron disease symptoms at around 6 months of age and do not die prior to 10-12 months of age   (MGI Ref ID J:56219)

Cln8^mnd/Cln8^mnd

        involves: B6.KB2

• nervous system phenotype
• abnormal myelination
  • many profiles of degenerating myelin sheaths and axons are seen in the white matter of the spinal cord   (MGI Ref ID J:47292)
  • lesions in the spinal cord are consistent with Wallerian degeneration   (MGI Ref ID J:47292)
• axon degeneration
  • many profiles of degenerating myelin sheaths and axons are seen in the white matter of the spinal cord   (MGI Ref ID J:47292)
  • lesions in the spinal cord are consistent with Wallerian degeneration   (MGI Ref ID J:47292)
  • however, no necrotic or apoptotic nuclei are detected in the central nervous system   (MGI Ref ID J:47292)
• gliosis   (MGI Ref ID J:47292)
• seizures
  • terminal seizures have been seen   (MGI Ref ID J:47292)
• behavior/neurological phenotype
• seizures
  • terminal seizures have been seen   (MGI Ref ID J:47292)

View Research Applications

Research Applications

This mouse can be used to support research in many areas including:

Cln8^mnd related

Neurobiology Research
Ataxia (Movement) Defects
Behavioral and Learning Defects
Metabolic Defects
Neurodegeneration
Neuromuscular Defects

Sensorineural Research
Retinal Degeneration

Genes & Alleles

Gene & Allele Information provided by MGI

Allele Symbol		Cln8mnd
Allele Name		motor neuron degeneration
Allele Type		Spontaneous
Common Name(s)		Cln8mnd; mnd;
Strain of Origin		B6.KB2-H2
Gene Symbol and Name		Cln8, ceroid-lipofuscinosis, neuronal 8
Chromosome		8
Gene Common Name(s)		C8orf61; EPMR; mnd; motor neuron degeneration;
General Note		It was originally thought that heterozygotes (mnd/+) could be detected at very old ages. However, it has been impossible to repeat these results and, even in 18-month old heterozygotes, no histological pathology is detectable (A. Messer and R. Bronson, personal communication, 1993). Early papers (J:8492, J:1224) state that this allele exhibits phenotypic similarity to amytrophic lateral sclerosis (ALS), however further analysis (J:12816, 56219) revealed that it is a better model for neuronal ceroid lipofuscinoses (Batten's disease) than for ALS.
Molecular Note		A single nucleotide insertion (267-268C, codon 90) predicts a frameshift and a truncated protein. [MGI Ref ID J:57766]

Genotyping

Genotyping Information

Genotyping Protocols

Cln8^mnd, Standard PCR

Helpful Links

Genotyping resources and troubleshooting

References

References provided by MGI

Additional References

Cln8^mnd related

Bakalian A; Kopmels B; Messer A; Fradelizi D; Delhaye-Bouchaud N; Wollman E; Mariani J. 1992. Peripheral macrophage abnormalities in mutant mice with spinocerebellar degeneration. Res Immunol 143(1):129-39. [PubMed: 1565842]  [MGI Ref ID J:2228]

Battaglioli G; Martin DL; Plummer J; Messer A. 1993. Synaptosomal glutamate uptake declines progressively in the spinal cord of a mutant mouse with motor neuron disease. J Neurochem 60(4):1567-9. [PubMed: 8095977]  [MGI Ref ID J:4191]

Bennett MJ; Boriack RL; Birch DG. 1997. 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 28(1):82-4. [PubMed: 9151333]  [MGI Ref ID J:42641]

Bermingham NA; Martin JE; Fisher EM. 1996. The mouse lysosomal membrane protein 1 gene as a candidate for the motorneuron degeneration (mnd) locus. Genomics 32(2):266-71. [PubMed: 8833154]  [MGI Ref ID J:31840]

Bertamini M; Marzani B; Guarneri R; Guarneri P; Bigini P; Mennini T; Curti D. 2002. Mitochondrial oxidative metabolism in motor neuron degeneration (mnd) mouse central nervous system. Eur J Neurosci 16(12):2291-6. [PubMed: 12492423]  [MGI Ref ID J:108072]

Bihl F; Lariviere L; Qureshi ST; Flaherty L; Malo D. 2001. LPS-hyporesponsiveness of mnd mice is associated with a mutation in Toll-like receptor 4. Genes Immun 2(1):56-9. [PubMed: 11294571]  [MGI Ref ID J:103167]

Bolivar VJ; Scott Ganus J; Messer A. 2002. The development of behavioral abnormalities in the motor neuron degeneration (mnd) mouse. Brain Res 937(1-2):74-82. [PubMed: 12020865]  [MGI Ref ID J:107789]

Boyce S; Webb JK; Carlson E; Rupniak NM; Hill RG; Martin JE. 1999. 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 155(1):49-58. [PubMed: 9918704]  [MGI Ref ID J:52561]

Bronson RT; Donahue LR; Johnson KR; Tanner A; Lane PW; Faust JR. 1998. Neuronal ceroid lipofuscinosis (nclf), a new disorder of the mouse linked to chromosome 9. Am J Med Genet 77(4):289-97. [PubMed: 9600738]  [MGI Ref ID J:47292]

Bronson RT; Lake BD; Cook S; Taylor S; Davisson MT. 1993. Motor neuron degeneration of mice is a model of neuronal ceroid lipofuscinosis (Batten's disease). Ann Neurol 33(4):381-5. [PubMed: 7683855]  [MGI Ref ID J:12816]

Callahan LM; Wylen EL; Messer A; Mazurkiewicz JE. 1991. Neurofilament distribution is altered in the Mnd (motor neuron degeneration) mouse. J Neuropathol Exp Neurol 50(4):491-504. [PubMed: 2061715]  [MGI Ref ID J:121249]

Chang B; Bronson RT; Hawes NL; Roderick TH; Peng C; Hageman GS; Heckenlively JR. 1994. Retinal degeneration in motor neuron degeneration: a mouse model of ceroid lipofuscinosis. Invest Ophthalmol Vis Sci 35(3):1071-6. [PubMed: 8125718]  [MGI Ref ID J:17934]

Chang B; Hawes NL; Hurd RE; Davisson MT; Nusinowitz S; Heckenlively JR. 2002. Retinal degeneration mutants in the mouse. Vision Res 42(4):517-25. [PubMed: 11853768]  [MGI Ref ID J:75095]

Chang B; Hawes NL; Hurd RE; Wang J; Howell D; Davisson MT; Roderick TH; Nusinowitz S; Heckenlively JR. 2005. Mouse models of ocular diseases. Vis Neurosci 22(5):587-93. [PubMed: 16332269]  [MGI Ref ID J:156373]

Cho SK; Gao N; Pearce DA; Lehrman MA; Hofmann SL. 2005. Characterization of lipid-linked oligosaccharide accumulation in mouse models of Batten disease. Glycobiology 15(6):637-48. [PubMed: 15647513]  [MGI Ref ID J:112499]

Cook S; Davisson MT; Bronson R; Messer A. 1993. New mutation - motor neuron degeneration - mnd Mouse Genome 91(2):313.  [MGI Ref ID J:29019]

Cooper JD; Messer A; Feng AK; Chua-Couzens J; Mobley WC. 1999. 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 19(7):2556-67. [PubMed: 10087069]  [MGI Ref ID J:53928]

Faust JR; Rodman JS; Daniel PF; Dice JF; Bronson RT. 1994. Two related proteolipids and dolichol-linked oligosaccharides accumulate in motor neuron degeneration mice (mnd/mnd), a model for neuronal ceroid lipofuscinosis. J Biol Chem 269(13):10150-5. [PubMed: 8144516]  [MGI Ref ID J:17522]

Frey D; Schneider C; Xu L; Borg J; Spooren W; Caroni P. 2000. Early and selective loss of neuromuscular synapse subtypes with low sprouting competence in motoneuron diseases. J Neurosci 20(7):2534-42. [PubMed: 10729333]  [MGI Ref ID J:109472]

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 cord of the development. Neurochem Res 23(4):557-62. [PubMed: 9566592]  [MGI Ref ID J:48902]

Fujita K; Yamauchi M; Matsui T; Titani K; Takahashi H; Kato T; Isomura G; Ando M; Nagata Y. 1998. Increase of glial fibrillary acidic protein fragments in the spinal cord of motor neuron degeneration mutant mouse. Brain Res 785(1):31-40. [PubMed: 9526038]  [MGI Ref ID J:51082]

Galizzi G; Russo D; Deidda I; Cascio C; Passantino R; Guarneri R; Bigini P; Mennini T; Drago G; Guarneri P. 2011. Different early ER-stress responses in the CLN8(mnd) mouse model of neuronal ceroid lipofuscinosis. Neurosci Lett 488(3):258-62. [PubMed: 21094208]  [MGI Ref ID J:168614]

Ghezzi P; Bernardini R; Giuffrida R; Bellomo M; Manzoni C; Comoletti D ; Di Santo E ; Benigni F ; Mennini T. 1998. Tumor necrosis factor is increased in the spinal cord of an animal model of motor neuron degeneration. Eur Cytokine Netw 9(2):139-44. [PubMed: 9681389]  [MGI Ref ID J:48610]

Griffin JL; Muller D; Woograsingh R; Jowatt V; Hindmarsh A; Nicholson JK; Martin JE. 2002. Vitamin E deficiency and metabolic deficits in neuronal ceroid lipofuscinosis described by bioinformatics. Physiol Genomics 11(3):195-203. [PubMed: 12388797]  [MGI Ref ID J:81309]

Guarneri R; Russo D; Cascio C; D'Agostino S; Galizzi G; Bigini P; Mennini T; Guarneri P. 2004. Retinal oxidation, apoptosis and age- and sex-differences in the mnd mutant mouse, a model of neuronal ceroid lipofuscinosis. Brain Res 1014(1-2):209-20. [PubMed: 15213005]  [MGI Ref ID J:90947]

Hawes NL; Smith RS; Chang B; Davisson M; Heckenlively JR; John SW. 1999. Mouse fundus photography and angiography: a catalogue of normal and mutant phenotypes. Mol Vis 5:22. [PubMed: 10493779]  [MGI Ref ID J:59481]

Holmes FE; Haynes LW. 1996. Superactivation of transglutaminase type 2 without change in enzyme level occurs during progressive neurodegeneration in the mnd mouse mutant. Neurosci Lett 213(3):185-8. [PubMed: 8873145]  [MGI Ref ID J:35599]

Li J; Nixon R; Messer A; Berman S; Bursztajn S. 1998. Altered gene expression for calpain/calpastatin system in motor neuron degeneration (Mnd) mutant mouse brain and spinal cord. Brain Res Mol Brain Res 53(1-2):174-86. [PubMed: 9473662]  [MGI Ref ID J:46702]

Mazurkiewicz JE. 1991. 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 128(2):182-6. [PubMed: 1658691]  [MGI Ref ID J:624]

Mazurkiewicz JE; Callahan LM; Swash M; Martin JE; Messer A. 1993. Cytoplasmic inclusions in spinal neurons of the motor neuron degeneration (Mnd) mouse. I. Light microscopic analysis. J Neurol Sci 116(1):59-66. [PubMed: 8389815]  [MGI Ref ID J:4750]

Mennini T; Bastone A; Crespi D; Comoletti D; Manzoni C. 1998. Spinal cord GLT-1 glutamate transporter and blood glutamic acid alterations in motor neuron degeneration (Mnd) mice. J Neurol Sci 157(1):31-6. [PubMed: 9600674]  [MGI Ref ID J:47141]

Mennini T; Bigini P; Cagnotto A; Carvelli L; Di Nunno P; Fumagalli E; Tortarolo M; Buurman WA; Ghezzi P; Bendotti C. 2004. Glial activation and TNFR-I upregulation precedes motor dysfunction in the spinal cord of mnd mice. Cytokine 25(3):127-35. [PubMed: 14698139]  [MGI Ref ID J:101859]

Mennini T; Bigini P; Ravizza T; Vezzani A; Calvaresi N; Tortarolo M; Bendotti C. 2002. Expression of glutamate receptor subtypes in the spinal cord of control and mnd mice, a model of motor neuron disorder. J Neurosci Res 70(4):553-60. [PubMed: 12404509]  [MGI Ref ID J:105145]

Mennini T; Cagnotto A; Carvelli L; Comoletti D; Manzoni C; Muzio V ; Rizzi M ; Vezzani A. 1999. Biochemical and pharmacological evidence of a functional role of AMPA receptors in motor neuron dysfunction in mnd mice. Eur J Neurosci 11(5):1705-10. [PubMed: 10215924]  [MGI Ref ID J:55254]

Messer A; Flaherty L. 1986. Autosomal dominance in a late-onset motor neuron disease in the mouse. J Neurogenet 3(6):345-55. [PubMed: 3783318]  [MGI Ref ID J:8492]

Messer A; Manley K; Plummer JA. 1999. An early-onset congenic strain of the motor neuron degeneration (mnd) mouse. Mol Genet Metab 66(4):393-7. [PubMed: 10191135]  [MGI Ref ID J:56219]

Messer A; Plummer J. 1993. Accumulating autofluorescent material as a marker for early changes in the spinal cord of the Mnd mouse. Neuromuscul Disord 3(2):129-34. [PubMed: 8358238]  [MGI Ref ID J:16252]

Messer A; Plummer J; MacMillen MC; Frankel WN. 1995. Genetics of primary and timing effects in the mnd mouse. Am J Med Genet 57(2):361-4. [PubMed: 7668363]  [MGI Ref ID J:25748]

Messer A; Plummer J; Maskin P; Coffin JM; Frankel WN. 1992. Mapping of the motor neuron degeneration (Mnd) gene, a mouse model of amyotrophic lateral sclerosis (ALS). Genomics 13(3):797-802. [PubMed: 1639406]  [MGI Ref ID J:1224]

Messer A; Plummer J; Wong V; Lavail MM. 1993. Retinal degeneration in motor neuron degeneration (mnd) mutant mice [letter] Exp Eye Res 57(5):637-41. [PubMed: 8282051]  [MGI Ref ID J:19328]

Oh C; Murray B; Bhattacharya N; Holland D; Tatton WG. 1994. (-)-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 38(1):64-74. [PubMed: 8057392]  [MGI Ref ID J:18052]

Pardo CA; Rabin BA; Palmer DN; Price DL. 1994. Accumulation of the adenosine triphosphate synthase subunit C in the mnd mutant mouse. A model for neuronal ceroid lipofuscinosis. Am J Pathol 144(4):829-35. [PubMed: 8160780]  [MGI Ref ID J:17600]

Plummer J; Peterson A; Messer A. 1995. Accelerated and widespread neuronal loss occurs in motor neuron degeneration (mnd) mice expressing a neurofilament-disrupting transgene. Mol Cell Neurosci 6(6):532-43. [PubMed: 8742270]  [MGI Ref ID J:31812]

Porter JC; Messer A; Peterson A. 1997. The motor neuron degeneration (mnd) gene acts intrinsically in motor neurons and peripheral fibroblasts. Mol Cell Neurosci 9(3):185-93. [PubMed: 9245501]  [MGI Ref ID J:42419]

Ranta S; Zhang Y; Ross B; Lonka L; Takkunen E; Messer A; Sharp J; Wheeler R; Kusumi K; Mole S; Liu W; Soares MB; Bonaldo MF; Hirvasniemi A; de la Chapelle A; Gilliam TC; Lehesjoki AE. 1999. The neuronal ceroid lipofuscinoses in human EPMR and mnd mutant mice are associated with mutations in CLN8. Nat Genet 23(2):233-6. [PubMed: 10508524]  [MGI Ref ID J:57766]

Seigel GM; Wagner J; Wronska A; Campbell L; Ju W; Zhong N. 2005. Progression of early postnatal retinal pathology in a mouse model of neuronal ceroid lipofuscinosis. Eye 19(12):1306-12. [PubMed: 15565184]  [MGI Ref ID J:116862]

Vance JE; Stone SJ; Faust JR. 1997. Abnormalities in mitochondria-associated membranes and phospholipid biosynthetic enzymes in the mnd/mnd mouse model of neuronal ceroid lipofuscinosis. Biochim Biophys Acta 1344(3):286-99. [PubMed: 9059519]  [MGI Ref ID J:39062]

Wendt KD; Lei B; Schachtman TR; Tullis GE; Ibe ME; Katz ML. 2005. Behavioral assessment in mouse models of neuronal ceroid lipofuscinosis using a light-cued T-maze. Behav Brain Res 161(2):175-82. [PubMed: 15885820]  [MGI Ref ID J:98844]

Won J; Shi LY; Hicks W; Wang J; Hurd R; Naggert JK; Chang B; Nishina PM. 2011. Mouse model resources for vision research. J Ophthalmol 2011:391384. [PubMed: 21052544]  [MGI Ref ID J:166679]

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In case of dissatisfaction for a valid reason and claimed in writing by a purchaser within ninety (90) days of receipt of mice, products or services, JACKSON will, at its option, provide credit or replacement for the mice or product received or the services provided.

No Liability

In no event shall JACKSON, its trustees, directors, officers, employees, and affiliates be liable for any causes of action or damages, including any direct, indirect, special, or consequential damages, arising out of the provision of MICE, PRODUCTS or services, including economic damage or injury to property and lost profits, and including any damage arising from acts or negligence on the part of JACKSON, its agents or employees. Unless prohibited by law, in purchasing or receiving MICE, PRODUCTS or services from JACKSON, purchaser or recipient, or any party claiming by or through them, expressly releases and discharges JACKSON from all such causes of action or damages, and further agrees to defend and indemnify JACKSON from any costs or damages arising out of any third party claims.

MICE and PRODUCTS are to be used in a safe manner and in accordance with all applicable governmental rules and regulations.

The foregoing represents the General Terms and Conditions applicable to JACKSON’s MICE, PRODUCTS or services. In addition, special terms and conditions of sale of certain MICE, PRODUCTS or services may be set forth separately in JACKSON web pages, catalogs, price lists, contracts, and/or other documents, and these special terms and conditions shall also govern the sale of these MICE, PRODUCTS and services by JACKSON, and by its licensees and distributors.

Acceptance of delivery of MICE, PRODUCTS or services shall be deemed agreement to these terms and conditions. No purchase order or other document transmitted by purchaser or recipient that may modify the terms and conditions hereof, shall be in any way binding on JACKSON, and instead the terms and conditions set forth herein, including any special terms and conditions set forth separately, shall govern the sale of MICE, PRODUCTS or services by JACKSON.