Immunochemical localization of the Batten disease (CLN3) protein in retina

Investigative Ophthalmology and Visual Science

Volumn 38, Issue 11, 1997, Pages 2375-2386

  Katz, Martin L ^a,c   Gao, Chun Lan ^a   Prabhakaram, Malladi ^a   Shibuya, Hisashi ^b   Liu, Po Ching ^b   Johnson, Gary S ^b  

^a University of Missouri   (United States)

^b UNIVERSITY OF MISSOURI   (United States)

^c UNIVERSITY OF MISSOURI   (United States)

Author keywords

Antibody; Ceroid lipofuscinosis; Immunocytochemistry; Mitochondria; Retina

Indexed keywords

COMPLEMENTARY DNA;

ARTICLE; DNA SEQUENCE; IMMUNOHISTOCHEMISTRY; MOUSE; NEURONAL CEROID LIPOFUSCINOSIS; NONHUMAN; NUCLEOTIDE SEQUENCE; PRIORITY JOURNAL; PROTEIN LOCALIZATION; PROTEIN PROCESSING; RETINA DEGENERATION; REVERSE TRANSCRIPTION POLYMERASE CHAIN REACTION;

AMINO ACID SEQUENCE; ANIMALS; DNA PRIMERS; DNA, COMPLEMENTARY; HUMANS; IMMUNOGLOBULIN G; MEMBRANE GLYCOPROTEINS; MICE; MICE, INBRED C57BL; MICROSCOPY, IMMUNOELECTRON; MITOCHONDRIA; MOLECULAR CHAPERONES; MOLECULAR SEQUENCE DATA; NEURONAL CEROID-LIPOFUSCINOSES; PEPTIDE FRAGMENTS; POLYMERASE CHAIN REACTION; PROTEINS; RABBITS; RETINA; SEQUENCE HOMOLOGY, AMINO ACID;

EID: 0030769620     PISSN: 01460404     EISSN: None     Source Type: Journal    
DOI: None     Document Type: Article

References (35)

1. Zeman W. The neuronal ceroid-lipofuscinoses. Prog Neuropathol. 1976;3:203-223.
2. Boustany RN, Alroy J, Kolodny EH. Clinical classification of neuronal ceroid lipofuscinosis subtypes. Am J Med Genetics Suppl. 1988;5(Suppl):47-58.
3. Dyken PR. Reconsideration of the classification of the neuronal ceroid-lipofuscinoses. Am J Med Genetics Suppl. 1988;5(Suppl):69-84.
4. Katz ML, Rodrigues M. Juvenile ceroid-lipofuscinosis: Evidence for methylated lysine in storage body protein. Am J Pathol. 1991;138:323-332.
5. International Batten Disease Consortium. Isolation of a novel gene underlying Batten disease, CLN3. Cell. 1995;82:949-957.
6. Newton CR, Graham A. Introduction to Biotechniques: PCR. Oxford: Bios Scientific Publishers Ltd., 1994.
7. Lee RL, Johnson KR, Lerner TJ. Isolation and chromosomal mapping of a mouse homolog of the Batten disease gene CLN3. Genomics. 1996;35:617-619.
8. Shibuya H, Nonneman DJ, Huang THM, Ganjam VK, Mann FA, Johnson GS. Two polymorphic microsatellites in a coding segment of the canine androgen receptor gene. Anim Genet. 1993;24:345-348.
9. Harlow E, Lane D. Antibodies: A Laboratory Manual. Cold Spring Harbor, NY: Cold Spring Harbor Laboratory; 1988.
10. Posnett DN, Tam JP. Multiple antigenic peptide method for producing antipeptide site-specific antibodies. Meth Enzymol. 1989;178:739-746.
11. Bronson RT, Lake BD, Cook S, Taylor S, Davisson MT. Motor neuron degeneration of mice is a model of neuronal ceroid lipofuscinosis (Batten's disease). Ann Neurol. 1993;33:381-385.
12. Prabhakaram M, Katz ML, Ortwerth BJ. Glycation mediated crosslinking between α-crystallin and MP26 in intact lens membranes. Mech Age Dev. 1996;91:65-78.
13. Newman GR, Jasani B, Williams ED. A simple post-embedding system for the rapid demonstration of tissue antigens under the electron microscope. Histochem J. 1983;15:543-555.
14. Katz ML, Robison WG. Evidence of cell loss from the rat retina during senescence. Exp Eye Res. 1986;42:293-304.
15. Robison WG, Kuwabara T, Zwann J. Eye research. In: Foster HL, Small JP, Fox J, eds. The Mouse in Biomedical Research, vol 4. New York: Academic Press; 1982.
16. Blanks JC, Mullen RJ, LaVail MM. Retinal degeneration in the pcd cerebellar mutant mouse. II. Electron microscopic analysis. J Comp Neurol. 1982;212:231-246.
17. Payne RM, Strauss AW. Expression of the mitochondria! creatine kinase genes. Mol Cell Biochem. 1994; 133:235-243.
18. Kelly DP, Gordon JI, Alpers R, Strauss AW. The tissue-specific expression and developmental regulation of two nuclear genes encoding rat mitochondrial proteins. J Biol Chem. 1989;264:18921-18925.
19. Hall NA, Lake BD, Dewji NN, Patrick AD. Lysosomal storage of subunit c of mitochondrial ATP synthase in Batten's disease (ceroid-lipofuscinosis). Biochem J. 1991;275:269-272.
20. Katz ML, Gao C, Tompkins JA, Chin DT, Bronson RT. Mitochondrial ATP synthase subunit c stored in hereditary ceroid-lipofuscinosis contains trimethyllysine. Biochem J. 1995;310:887-892.
21. Kominami E, Ezaki J, Muno D, Ishidoh K, Ueno T, Wolfe LS. Specific storage of subunit c of mitochondrial ATP synthase in lysosomes of neuronal ceroid lipofuscinosis (Batten's disease). J Biochem. 1992; 111:278-282.
22. Palmer DN, Fearnley IM, Walker JE, et al. Mitochondrial ATP synthase subunit c storage in the ceroid-lipofuscinoses (Batten disease). Am J Med Genet. 1992;42:561-567.
23. Ezaki J, Wolfe LS, Kominami E. Specific delay in degradation of mitochondrial ATP synthase subunit c in late infantile neuronal ceroid lipofuscinosis is derived from cellular proteolytic dysfunction rather than structural alteration of subunit c. J Neurochem. 1996;67:1677-1687.
24. Ezaki J, Wolfe LS, Higuti T, Ishidoh K, Kominami E. Specific delay of degradation of mitochondrial ATP synthase subunit c in late infantile neuronal ceroid lipofuscinosis (Batten disease). J Neurochem. 1995; 64:733-741.
25. Mihara K, Omura T. Protein import into mammalian mitochondria. Meth Enzymol. 1995;260:302-310.
26. Von Heijne G. Mitochondrial targeting sequences may form amphiphilic helices. EMBO J. 1986;5:1335-1342.
27. Watson ME. Compilation of signal sequences. Nucleic Acids Res. 1984;12:5145-5164.
28. Bedwell DM, Strobel SA, Yun K, Jongeward GD, Emr SD. Sequence and structural requirements of a mitochondrial protein import signal denned by saturation cassette mutagenesis. Mol Cell Biol. 1989;9:1014-1025.
29. Lemire BD, Frankhauser C, Baker A, Schatz G. The mitochondrial targeting function of randomly generated peptide sequences correlates with predicted helical amphiphilicity. J Biol Chem. 1989;264:20206-20215.
30. Pfanner N, Hoeben P, Tropschug M, Neupert W. The carboxyl-terminal two-thirds of the ADP/ATP carrier polypeptide contains sufficient information to direct translocation into mitochondria. J Biol Chem. 1987; 262:14851-14854.
31. Applebury ML, Hargrave PA. Molecular biology of the visual pigments. Vis Res. 1986;26:1881-1895.
32. Hargrave PA, McDowell JH. Rhodopsin and phototransduction: A model system for G protein-linked receptors. FASEB J. 1992;6:2323-2331.
33. Mortiz OL, Molday RS. Molecular cloning, membrane topology, and localization of bovine Rom-1 in rod and cone photoreceptor cells. Invest Ophthalmol Vis Sci. 1996;37:352-362.
34. Faust JR, Rodman JS, Daniel PF, Dice JF, Bronson RT. 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;269:10150-10155.
35. Chang B, Bronson RT, Hawes NL, et al. Retinal degeneration in motor neuron degeneration: A mouse model for ceroid-lipofuscinosis. Invest Ophthalmol Vis Sci. 1994;35:1071-1076.