MOUSE:CLN8

Species (Taxon ID)	Mus musculus (Mouse). (10090)
Gene Name(s)	Cln8
Protein Name(s)	Protein CLN8
External Links
UniProt	Q9QUK3
EMBL	AF125308 AF125307 BC021625
CCDS	CCDS40239.1
RefSeq	NP_036130.1 XP_006508869.1 XP_006508870.1
UniGene	Mm.254027
MaxQB	Q9QUK3
PaxDb	Q9QUK3
PRIDE	Q9QUK3
Ensembl	ENSMUST00000027554
GeneID	26889
KEGG	mmu:26889
UCSC	uc009kze.1
CTD	2055
MGI	MGI:1349447
eggNOG	NOG239251
GeneTree	ENSGT00390000001315
HOGENOM	HOG000111792
HOVERGEN	HBG028338
InParanoid	Q9QUK3
KO	K12360
OMA	LTYHMWW
PhylomeDB	Q9QUK3
TreeFam	TF331146
NextBio	304715
PRO	PR:Q9QUK3
Proteomes	UP000000589
Bgee	Q9QUK3
CleanEx	MM_CLN8
ExpressionAtlas	Q9QUK3
Genevestigator	Q9QUK3
GO	GO:0005783 GO:0005789 GO:0005793 GO:0033116 GO:0016021 GO:0005739 GO:0008344 GO:0007628 GO:0001306 GO:0008306 GO:0044265 GO:0044257 GO:0044267 GO:0006672 GO:0008203 GO:0051935 GO:0007040 GO:0007006 GO:0050881 GO:0043066 GO:0051348 GO:0007399 GO:0060052 GO:0050885 GO:0050884 GO:0006644 GO:0045494 GO:0008361 GO:0060041 GO:0035176 GO:0021523 GO:0021522 GO:0007601
InterPro	IPR006634
Pfam	PF03798
SMART	SM00724
PROSITE	PS50922

Annotations

Qualifier	GO ID	GO term name	Reference	ECO ID	ECO term name	with/from	Aspect	Extension	Notes	Status
	GO:0051179	localization	PMID:23142642^[1]	ECO:0000314			P		Figure 5 shows the co-localization of CLN8 with the VAPA protein.	complete CACAO 5775
	GO:0051179	localization	PMID:23142642^[1]	ECO:0000314			P		Figure 5 shows the co-localization of CLN8 with the GATE16 protein.	complete CACAO 5776
	GO:0045184	establishment of protein localization	PMID:23142642^[1]	ECO:0000314			P		Figure 6. Co-localization of CLN8 and VAPA in the adult mouse CNS tissues.	complete CACAO 6821
part_of	GO:0005783	endoplasmic reticulum	PMID:19941651^[2]	ECO:0000314	direct assay evidence used in manual assertion		C		Seeded From UniProt	complete
involved_in	GO:0008203	cholesterol metabolic process	GO_REF:0000024	ECO:0000250	sequence similarity evidence used in manual assertion	UniProtKB:Q9UBY8	P		Seeded From UniProt	complete
involved_in	GO:0007399	nervous system development	GO_REF:0000024	ECO:0000250	sequence similarity evidence used in manual assertion	UniProtKB:Q9UBY8	P		Seeded From UniProt	complete
involved_in	GO:0006672	ceramide metabolic process	GO_REF:0000024	ECO:0000250	sequence similarity evidence used in manual assertion	UniProtKB:Q9UBY8	P		Seeded From UniProt	complete
involved_in	GO:0006644	phospholipid metabolic process	GO_REF:0000024	ECO:0000250	sequence similarity evidence used in manual assertion	UniProtKB:Q9UBY8	P		Seeded From UniProt	complete
part_of	GO:0005793	endoplasmic reticulum-Golgi intermediate compartment	GO_REF:0000024	ECO:0000250	sequence similarity evidence used in manual assertion	UniProtKB:Q9UBY8	C		Seeded From UniProt	complete
part_of	GO:0005783	endoplasmic reticulum	GO_REF:0000024	ECO:0000250	sequence similarity evidence used in manual assertion	UniProtKB:Q9UBY8	C		Seeded From UniProt	complete
acts_upstream_of_or_within	GO:0060052	neurofilament cytoskeleton organization	PMID:2061715^[3]	ECO:0000315	mutant phenotype evidence used in manual assertion	MGI:MGI:1856959	P	occurs_in:(EMAPA:17577) occurs_in:(CL:0000100)	Seeded From UniProt	complete
acts_upstream_of_or_within	GO:0060041	retina development in camera-type eye	PMID:21052544^[4]	ECO:0000315	mutant phenotype evidence used in manual assertion		P		Seeded From UniProt	complete
acts_upstream_of_or_within	GO:0060041	retina development in camera-type eye	PMID:15565184^[5]	ECO:0000315	mutant phenotype evidence used in manual assertion	MGI:MGI:1856959	P		Seeded From UniProt	complete
acts_upstream_of_or_within	GO:0051935	glutamate reuptake	PMID:9600674^[6]	ECO:0000315	mutant phenotype evidence used in manual assertion	MGI:MGI:1856959	P	occurs_in:(EMAPA:17577)	Seeded From UniProt	complete
acts_upstream_of_or_within	GO:0051935	glutamate reuptake	PMID:8095977^[7]	ECO:0000315	mutant phenotype evidence used in manual assertion	MGI:MGI:1856959	P	occurs_in:(EMAPA:17577)	Seeded From UniProt	complete
acts_upstream_of_or_within	GO:0050885	neuromuscular process controlling balance	PMID:9918704^[8]	ECO:0000315	mutant phenotype evidence used in manual assertion	MGI:MGI:1856959	P		Seeded From UniProt	complete
acts_upstream_of_or_within	GO:0050884	neuromuscular process controlling posture	PMID:2061715^[3]	ECO:0000315	mutant phenotype evidence used in manual assertion	MGI:MGI:1856959	P		Seeded From UniProt	complete
acts_upstream_of_or_within	GO:0050881	musculoskeletal movement	PMID:2061715^[3]	ECO:0000315	mutant phenotype evidence used in manual assertion	MGI:MGI:1856959	P		Seeded From UniProt	complete
acts_upstream_of_or_within	GO:0045494	photoreceptor cell maintenance	PMID:9151333^[9]	ECO:0000315	mutant phenotype evidence used in manual assertion	MGI:MGI:1856959	P		Seeded From UniProt	complete
acts_upstream_of_or_within	GO:0045494	photoreceptor cell maintenance	PMID:15565184^[5]	ECO:0000315	mutant phenotype evidence used in manual assertion	MGI:MGI:1856959	P		Seeded From UniProt	complete
acts_upstream_of_or_within	GO:0044267	cellular protein metabolic process	PMID:1658691^[10]	ECO:0000315	mutant phenotype evidence used in manual assertion	MGI:MGI:1856959	P	occurs_in:(EMAPA:17577) occurs_in:(CL:0000100)	Seeded From UniProt	complete
acts_upstream_of_or_within	GO:0044265	cellular macromolecule catabolic process	PMID:15647513^[11]	ECO:0000315	mutant phenotype evidence used in manual assertion	MGI:MGI:1856959	P		Seeded From UniProt	complete
acts_upstream_of_or_within	GO:0044257	cellular protein catabolic process	PMID:8389815^[12]	ECO:0000315	mutant phenotype evidence used in manual assertion	MGI:MGI:1856959	P	occurs_in:(EMAPA:17577) occurs_in:(CL:0000100)	Seeded From UniProt	complete
acts_upstream_of_or_within	GO:0043066	negative regulation of apoptotic process	PMID:15565184^[5]	ECO:0000315	mutant phenotype evidence used in manual assertion	MGI:MGI:1856959	P	regulates_o_occurs_in:(EMAPA:17168) regulates_o_occurs_in:(CL:0000740)\|regulates_o_occurs_in:(EMAPA:17168) regulates_o_occurs_in:(CL:0000604)	Seeded From UniProt	complete
acts_upstream_of_or_within	GO:0035176	social behavior	PMID:12020865^[13]	ECO:0000315	mutant phenotype evidence used in manual assertion	MGI:MGI:1856959	P		Seeded From UniProt	complete
acts_upstream_of_or_within	GO:0021523	somatic motor neuron differentiation	PMID:21052544^[4]	ECO:0000315	mutant phenotype evidence used in manual assertion		P		Seeded From UniProt	complete
acts_upstream_of_or_within	GO:0021522	spinal cord motor neuron differentiation	PMID:2061715^[3]	ECO:0000315	mutant phenotype evidence used in manual assertion	MGI:MGI:1856959	P	occurs_in:(EMAPA:17577) results_in_acquisition_of_features_of:(CL:0000100)	Seeded From UniProt	complete
acts_upstream_of_or_within	GO:0008361	regulation of cell size	PMID:15565184^[5]	ECO:0000315	mutant phenotype evidence used in manual assertion	MGI:MGI:1856959	P	regulates_o_occurs_in:(EMAPA:17168) regulates_o_occurs_in:(CL:0000740)	Seeded From UniProt	complete
acts_upstream_of_or_within	GO:0008344	adult locomotory behavior	PMID:12020865^[13]	ECO:0000315	mutant phenotype evidence used in manual assertion	MGI:MGI:1856959	P		Seeded From UniProt	complete
acts_upstream_of_or_within	GO:0008306	associative learning	PMID:15885820^[14]	ECO:0000315	mutant phenotype evidence used in manual assertion	MGI:MGI:1856959	P		Seeded From UniProt	complete
acts_upstream_of_or_within	GO:0008203	cholesterol metabolic process	GO_REF:0000096	ECO:0000266	sequence orthology evidence used in manual assertion	UniProtKB:Q9UBY8	P		Seeded From UniProt	complete
acts_upstream_of_or_within	GO:0007628	adult walking behavior	PMID:9918704^[8]	ECO:0000315	mutant phenotype evidence used in manual assertion	MGI:MGI:1856959	P		Seeded From UniProt	complete
acts_upstream_of_or_within	GO:0007628	adult walking behavior	PMID:7683855^[15]	ECO:0000315	mutant phenotype evidence used in manual assertion	MGI:MGI:1856959	P		Seeded From UniProt	complete
acts_upstream_of_or_within	GO:0007628	adult walking behavior	PMID:7668363^[16]	ECO:0000315	mutant phenotype evidence used in manual assertion	MGI:MGI:1856959	P		Seeded From UniProt	complete
acts_upstream_of_or_within	GO:0007628	adult walking behavior	PMID:3783318^[17]	ECO:0000315	mutant phenotype evidence used in manual assertion	MGI:MGI:1856959	P		Seeded From UniProt	complete
acts_upstream_of_or_within	GO:0007628	adult walking behavior	PMID:2061715^[3]	ECO:0000315	mutant phenotype evidence used in manual assertion	MGI:MGI:1856959	P		Seeded From UniProt	complete
acts_upstream_of_or_within	GO:0007628	adult walking behavior	PMID:10215924^[18]	ECO:0000315	mutant phenotype evidence used in manual assertion	MGI:MGI:1856959	P		Seeded From UniProt	complete
acts_upstream_of_or_within	GO:0007601	visual perception	PMID:8282051^[19]	ECO:0000315	mutant phenotype evidence used in manual assertion	MGI:MGI:1856959	P	has_participant:(EMAPA:17168)	Seeded From UniProt	complete
acts_upstream_of_or_within	GO:0007601	visual perception	PMID:8125718^[20]	ECO:0000315	mutant phenotype evidence used in manual assertion	MGI:MGI:1856959	P	has_participant:(EMAPA:17168)	Seeded From UniProt	complete
acts_upstream_of_or_within	GO:0007601	visual perception	PMID:7683855^[15]	ECO:0000315	mutant phenotype evidence used in manual assertion	MGI:MGI:1856959	P		Seeded From UniProt	complete
acts_upstream_of_or_within	GO:0007601	visual perception	PMID:7668363^[16]	ECO:0000315	mutant phenotype evidence used in manual assertion	MGI:MGI:1856959	P	has_participant:(EMAPA:17168)	Seeded From UniProt	complete
acts_upstream_of_or_within	GO:0007399	nervous system development	GO_REF:0000096	ECO:0000266	sequence orthology evidence used in manual assertion	UniProtKB:Q9UBY8	P		Seeded From UniProt	complete
acts_upstream_of_or_within	GO:0007040	lysosome organization	PMID:8160780^[21]	ECO:0000315	mutant phenotype evidence used in manual assertion	MGI:MGI:1856959	P	occurs_in:(EMAPA:35915) occurs_in:(CL:0000117)\|occurs_in:(EMAPA:32845) occurs_in:(CL:0000117)\|occurs_in:(EMAPA:32767) occurs_in:(CL:0000117)\|occurs_in:(EMAPA:17577) occurs_in:(CL:0000117)\|occurs_in:(EMAPA:17168) occurs_in:(CL:0000117)\|occurs_in:(EMAPA:18234)\|occurs_in:(EMAPA:32688)\|occurs_in:(EMAPA:16846)\|occurs_in:(EMAPA:17373)\|occurs_in:(EMAPA:17503)	Seeded From UniProt	complete
acts_upstream_of_or_within	GO:0007040	lysosome organization	PMID:8144516^[22]	ECO:0000315	mutant phenotype evidence used in manual assertion	MGI:MGI:1856959	P	occurs_in:(EMAPA:16894)\|occurs_in:(EMAPA:17373)\|occurs_in:(EMAPA:16846)\|occurs_in:(EMAPA:16105)\|occurs_in:(EMAPA:17503)	Seeded From UniProt	complete
acts_upstream_of_or_within	GO:0007040	lysosome organization	PMID:7613514^[23]	ECO:0000315	mutant phenotype evidence used in manual assertion	MGI:MGI:1856959	P	occurs_in:(CL:0000057)	Seeded From UniProt	complete
acts_upstream_of_or_within	GO:0007040	lysosome organization	PMID:10087069^[24]	ECO:0000315	mutant phenotype evidence used in manual assertion	MGI:MGI:1856959	P	occurs_in:(EMAPA:32845) occurs_in:(CL:0000099)	Seeded From UniProt	complete
acts_upstream_of_or_within	GO:0007006	mitochondrial membrane organization	PMID:9059519^[25]	ECO:0000315	mutant phenotype evidence used in manual assertion	MGI:MGI:1856959	P	occurs_in:(EMAPA:16846)	Seeded From UniProt	complete
acts_upstream_of_or_within	GO:0006672	ceramide metabolic process	GO_REF:0000096	ECO:0000266	sequence orthology evidence used in manual assertion	UniProtKB:Q9UBY8	P		Seeded From UniProt	complete
acts_upstream_of_or_within	GO:0006644	phospholipid metabolic process	PMID:9245501^[26]	ECO:0000315	mutant phenotype evidence used in manual assertion	MGI:MGI:1856959	P	occurs_in:(EMAPA:16846) occurs_in:(CL:0000182)	Seeded From UniProt	complete
acts_upstream_of_or_within	GO:0006644	phospholipid metabolic process	GO_REF:0000096	ECO:0000266	sequence orthology evidence used in manual assertion	UniProtKB:Q9UBY8	P		Seeded From UniProt	complete
part_of	GO:0005793	endoplasmic reticulum-Golgi intermediate compartment	GO_REF:0000096	ECO:0000266	sequence orthology evidence used in manual assertion	UniProtKB:Q9UBY8	C		Seeded From UniProt	complete
part_of	GO:0005783	endoplasmic reticulum	GO_REF:0000096	ECO:0000266	sequence orthology evidence used in manual assertion	UniProtKB:Q9UBY8	C		Seeded From UniProt	complete
acts_upstream_of_or_within	GO:0001306	age-dependent response to oxidative stress	PMID:15213005^[27]	ECO:0000315	mutant phenotype evidence used in manual assertion	MGI:MGI:1856959	P	has_participant:(EMAPA:17168)	Seeded From UniProt	complete
part_of	GO:0005739	mitochondrion	GO_REF:0000108	ECO:0000364	evidence based on logical inference from manual annotation used in automatic assertion	GO:0007006	C		Seeded From UniProt	complete
part_of	GO:0098793	presynapse	GO_REF:0000108	ECO:0000364	evidence based on logical inference from manual annotation used in automatic assertion	GO:0051935	C		Seeded From UniProt	complete
part_of	GO:0098793	presynapse	GO_REF:0000108	ECO:0000364	evidence based on logical inference from manual annotation used in automatic assertion	GO:0051935	C		Seeded From UniProt	complete
part_of	GO:0016021	integral component of membrane	GO_REF:0000002	ECO:0000256	match to sequence model evidence used in automatic assertion	InterPro:IPR006634	C		Seeded From UniProt	complete
part_of	GO:0016020	membrane	GO_REF:0000037	ECO:0000322	imported manually asserted information used in automatic assertion	UniProtKB-KW:KW-0472	C		Seeded From UniProt	complete
part_of	GO:0005783	endoplasmic reticulum	GO_REF:0000037 GO_REF:0000039	ECO:0000322	imported manually asserted information used in automatic assertion	UniProtKB-KW:KW-0256 UniProtKB-SubCell:SL-0095	C		Seeded From UniProt	complete
part_of	GO:0016021	integral component of membrane	GO_REF:0000037	ECO:0000322	imported manually asserted information used in automatic assertion	UniProtKB-KW:KW-0812	C		Seeded From UniProt	complete
part_of	GO:0005789	endoplasmic reticulum membrane	GO_REF:0000039	ECO:0000322	imported manually asserted information used in automatic assertion	UniProtKB-SubCell:SL-0097	C		Seeded From UniProt	complete
part_of	GO:0033116	endoplasmic reticulum-Golgi intermediate compartment membrane	GO_REF:0000039	ECO:0000322	imported manually asserted information used in automatic assertion	UniProtKB-SubCell:SL-0099	C		Seeded From UniProt	complete
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Notes

References

See Help:References for how to manage references in GONUTS.

↑ ^1.0 ^1.1 ^1.2 Passantino, R et al. (2013) Identifying protein partners of CLN8, an ER-resident protein involved in neuronal ceroid lipofuscinosis. Biochim. Biophys. Acta 1833 529-40 PubMed GONUTS page
↑ Lyly, A et al. (2009) Novel interactions of CLN5 support molecular networking between Neuronal Ceroid Lipofuscinosis proteins. BMC Cell Biol. 10 83 PubMed GONUTS page
↑ ^3.0 ^3.1 ^3.2 ^3.3 ^3.4 Callahan, LM et al. (1991) Neurofilament distribution is altered in the Mnd (motor neuron degeneration) mouse. J. Neuropathol. Exp. Neurol. 50 491-504 PubMed GONUTS page
↑ ^4.0 ^4.1 Won, J et al. (2011) Mouse model resources for vision research. J Ophthalmol 2011 391384 PubMed GONUTS page
↑ ^5.0 ^5.1 ^5.2 ^5.3 Seigel, GM et al. (2005) Progression of early postnatal retinal pathology in a mouse model of neuronal ceroid lipofuscinosis. Eye (Lond) 19 1306-12 PubMed GONUTS page
↑ Mennini, T et al. (1998) Spinal cord GLT-1 glutamate transporter and blood glutamic acid alterations in motor neuron degeneration (Mnd) mice. J. Neurol. Sci. 157 31-6 PubMed GONUTS page
↑ Battaglioli, G et al. (1993) Synaptosomal glutamate uptake declines progressively in the spinal cord of a mutant mouse with motor neuron disease. J. Neurochem. 60 1567-9 PubMed GONUTS page
↑ ^8.0 ^8.1 Boyce, S et al. (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 49-58 PubMed GONUTS page
↑ Bennett, MJ et al. (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 82-4 PubMed GONUTS page
↑ 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. 128 182-6 PubMed GONUTS page
↑ Cho, SK et al. (2005) Characterization of lipid-linked oligosaccharide accumulation in mouse models of Batten disease. Glycobiology 15 637-48 PubMed GONUTS page
↑ Mazurkiewicz, JE et al. (1993) Cytoplasmic inclusions in spinal neurons of the motor neuron degeneration (Mnd) mouse. I. Light microscopic analysis. J. Neurol. Sci. 116 59-66 PubMed GONUTS page
↑ ^13.0 ^13.1 Bolivar, VJ et al. (2002) The development of behavioral abnormalities in the motor neuron degeneration (mnd) mouse. Brain Res. 937 74-82 PubMed GONUTS page
↑ Wendt, KD et al. (2005) Behavioral assessment in mouse models of neuronal ceroid lipofuscinosis using a light-cued T-maze. Behav. Brain Res. 161 175-82 PubMed GONUTS page
↑ ^15.0 ^15.1 Bronson, RT et al. (1993) Motor neuron degeneration of mice is a model of neuronal ceroid lipofuscinosis (Batten's disease). Ann. Neurol. 33 381-5 PubMed GONUTS page
↑ ^16.0 ^16.1 Messer, A et al. (1995) Genetics of primary and timing effects in the mnd mouse. Am. J. Med. Genet. 57 361-4 PubMed GONUTS page
↑ Messer, A & Flaherty, L (1986) Autosomal dominance in a late-onset motor neuron disease in the mouse. J. Neurogenet. 3 345-55 PubMed GONUTS page
↑ Mennini, T et al. (1999) Biochemical and pharmacological evidence of a functional role of AMPA receptors in motor neuron dysfunction in mnd mice. Eur. J. Neurosci. 11 1705-10 PubMed GONUTS page
↑ Messer, A et al. (1993) Retinal degeneration in motor neuron degeneration (mnd) mutant mice. Exp. Eye Res. 57 637-41 PubMed GONUTS page
↑ Chang, B et al. (1994) Retinal degeneration in motor neuron degeneration: a mouse model of ceroid lipofuscinosis. Invest. Ophthalmol. Vis. Sci. 35 1071-6 PubMed GONUTS page
↑ Pardo, CA et al. (1994) Accumulation of the adenosine triphosphate synthase subunit C in the mnd mutant mouse. A model for neuronal ceroid lipofuscinosis. Am. J. Pathol. 144 829-35 PubMed GONUTS page
↑ Faust, JR et al. (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 10150-5 PubMed GONUTS page
↑ Tanner, A & Dice, JF (1995) Batten disease fibroblasts in culture accumulate mitochondrial ATP synthase subunit 9. Cell Biol. Int. 19 71-5 PubMed GONUTS page
↑ Cooper, JD et al. (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 2556-67 PubMed GONUTS page
↑ Vance, JE et al. (1997) Abnormalities in mitochondria-associated membranes and phospholipid biosynthetic enzymes in the mnd/mnd mouse model of neuronal ceroid lipofuscinosis. Biochim. Biophys. Acta 1344 286-99 PubMed GONUTS page
↑ Porter, JC et al. (1997) The motor neuron degeneration (mnd) gene acts intrinsically in motor neurons and peripheral fibroblasts. Mol. Cell. Neurosci. 9 185-93 PubMed GONUTS page
↑ Guarneri, R et al. (2004) Retinal oxidation, apoptosis and age- and sex-differences in the mnd mutant mouse, a model of neuronal ceroid lipofuscinosis. Brain Res. 1014 209-20 PubMed GONUTS page

[PMID:23142642-1] 1.0 ^1.1 ^1.2 Passantino, R et al. (2013) Identifying protein partners of CLN8, an ER-resident protein involved in neuronal ceroid lipofuscinosis. Biochim. Biophys. Acta 1833 529-40 PubMed GONUTS page

[PMID:19941651-2] Lyly, A et al. (2009) Novel interactions of CLN5 support molecular networking between Neuronal Ceroid Lipofuscinosis proteins. BMC Cell Biol. 10 83 PubMed GONUTS page

[PMID:2061715-3] 3.0 ^3.1 ^3.2 ^3.3 ^3.4 Callahan, LM et al. (1991) Neurofilament distribution is altered in the Mnd (motor neuron degeneration) mouse. J. Neuropathol. Exp. Neurol. 50 491-504 PubMed GONUTS page

[PMID:21052544-4] 4.0 ^4.1 Won, J et al. (2011) Mouse model resources for vision research. J Ophthalmol 2011 391384 PubMed GONUTS page

[PMID:15565184-5] 5.0 ^5.1 ^5.2 ^5.3 Seigel, GM et al. (2005) Progression of early postnatal retinal pathology in a mouse model of neuronal ceroid lipofuscinosis. Eye (Lond) 19 1306-12 PubMed GONUTS page

[PMID:9600674-6] Mennini, T et al. (1998) Spinal cord GLT-1 glutamate transporter and blood glutamic acid alterations in motor neuron degeneration (Mnd) mice. J. Neurol. Sci. 157 31-6 PubMed GONUTS page

[PMID:8095977-7] Battaglioli, G et al. (1993) Synaptosomal glutamate uptake declines progressively in the spinal cord of a mutant mouse with motor neuron disease. J. Neurochem. 60 1567-9 PubMed GONUTS page

[PMID:9918704-8] 8.0 ^8.1 Boyce, S et al. (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 49-58 PubMed GONUTS page

[PMID:9151333-9] Bennett, MJ et al. (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 82-4 PubMed GONUTS page

[PMID:1658691-10] 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. 128 182-6 PubMed GONUTS page

[PMID:15647513-11] Cho, SK et al. (2005) Characterization of lipid-linked oligosaccharide accumulation in mouse models of Batten disease. Glycobiology 15 637-48 PubMed GONUTS page

[PMID:8389815-12] Mazurkiewicz, JE et al. (1993) Cytoplasmic inclusions in spinal neurons of the motor neuron degeneration (Mnd) mouse. I. Light microscopic analysis. J. Neurol. Sci. 116 59-66 PubMed GONUTS page

[PMID:12020865-13] 13.0 ^13.1 Bolivar, VJ et al. (2002) The development of behavioral abnormalities in the motor neuron degeneration (mnd) mouse. Brain Res. 937 74-82 PubMed GONUTS page

[PMID:15885820-14] Wendt, KD et al. (2005) Behavioral assessment in mouse models of neuronal ceroid lipofuscinosis using a light-cued T-maze. Behav. Brain Res. 161 175-82 PubMed GONUTS page

[PMID:7683855-15] 15.0 ^15.1 Bronson, RT et al. (1993) Motor neuron degeneration of mice is a model of neuronal ceroid lipofuscinosis (Batten's disease). Ann. Neurol. 33 381-5 PubMed GONUTS page

[PMID:7668363-16] 16.0 ^16.1 Messer, A et al. (1995) Genetics of primary and timing effects in the mnd mouse. Am. J. Med. Genet. 57 361-4 PubMed GONUTS page

[PMID:3783318-17] Messer, A & Flaherty, L (1986) Autosomal dominance in a late-onset motor neuron disease in the mouse. J. Neurogenet. 3 345-55 PubMed GONUTS page

[PMID:10215924-18] Mennini, T et al. (1999) Biochemical and pharmacological evidence of a functional role of AMPA receptors in motor neuron dysfunction in mnd mice. Eur. J. Neurosci. 11 1705-10 PubMed GONUTS page

[PMID:8282051-19] Messer, A et al. (1993) Retinal degeneration in motor neuron degeneration (mnd) mutant mice. Exp. Eye Res. 57 637-41 PubMed GONUTS page

[PMID:8125718-20] Chang, B et al. (1994) Retinal degeneration in motor neuron degeneration: a mouse model of ceroid lipofuscinosis. Invest. Ophthalmol. Vis. Sci. 35 1071-6 PubMed GONUTS page

[PMID:8160780-21] Pardo, CA et al. (1994) Accumulation of the adenosine triphosphate synthase subunit C in the mnd mutant mouse. A model for neuronal ceroid lipofuscinosis. Am. J. Pathol. 144 829-35 PubMed GONUTS page

[PMID:8144516-22] Faust, JR et al. (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 10150-5 PubMed GONUTS page

[PMID:7613514-23] Tanner, A & Dice, JF (1995) Batten disease fibroblasts in culture accumulate mitochondrial ATP synthase subunit 9. Cell Biol. Int. 19 71-5 PubMed GONUTS page

[PMID:10087069-24] Cooper, JD et al. (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 2556-67 PubMed GONUTS page

[PMID:9059519-25] Vance, JE et al. (1997) Abnormalities in mitochondria-associated membranes and phospholipid biosynthetic enzymes in the mnd/mnd mouse model of neuronal ceroid lipofuscinosis. Biochim. Biophys. Acta 1344 286-99 PubMed GONUTS page

[PMID:9245501-26] Porter, JC et al. (1997) The motor neuron degeneration (mnd) gene acts intrinsically in motor neurons and peripheral fibroblasts. Mol. Cell. Neurosci. 9 185-93 PubMed GONUTS page

[PMID:15213005-27] Guarneri, R et al. (2004) Retinal oxidation, apoptosis and age- and sex-differences in the mnd mutant mouse, a model of neuronal ceroid lipofuscinosis. Brain Res. 1014 209-20 PubMed GONUTS page

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MOUSE:CLN8

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