DOI: 10.1016/s0014-5793(03)00284-9. Membrane topology of CLN3, the protein underlying Batten disease

Membrane topology of CLN3, the protein underlying Batten disease

10.1016/s0014-5793(03)00284-9

Crossref journal-article

Wiley

FEBS Letters (311)

Abstract

Juvenile neuronal ceroid lipofuscinosis, or Batten disease, is an autosomal recessive disorder characterized by progressive loss of motor and cognitive functions, loss of vision, progressively severe seizures, and death. The disease is associated with mutations in the gene CLN3, which encodes a novel 438 amino acid protein, the function of which is currently unknown. Protein secondary structure prediction programs suggest that the CLN3 protein has five to seven membrane‐spanning domains (MSDs). To distinguish among a number of hypothetical models for the membrane topology of CLN3 we used in vitro translation of native, Flag epitope‐labeled and glycosylation site‐mutated CLN3 protein in the presence or absence of canine pancreatic microsomes. These were immunoprecipitated using antibodies specific for Flag or peptide sequences within CLN3 or left untreated. The results indicate that CLN3 contains five MSDs, an extracellular/intraluminal amino‐terminus, and a cytoplasmic carboxy‐terminus.

Bibliography

Mao, Q., Foster, B. J., Xia, H., & Davidson, B. L. (2003). Membrane topology of CLN3, the protein underlying Batten disease. FEBS Letters, 541(1â3), 40â46. Portico.

Authors 4

Qinwen Mao (first)
Brian J Foster (additional)
Haibin Xia (additional)
Beverly L Davidson (additional)

References 22 Referenced 42

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Rumelhart D.E. McClelland J. and P.D.P. research group (1986) Parallel Distributed Processing: Explorations in the Microstructure of Cognition pp. 318–362. MIT Press Cambridge MA. (10.7551/mitpress/5236.001.0001)
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Solovyev V.V. and Salamov A.A. (1994) in: Computer Analysis of Genetic Macromolecules (Kolchanov N.A. and Lim H.A. Eds.) pp. 352–364. World Scientific Singapore.
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Dates

Type	When
Created	22 years, 4 months ago (April 24, 2003, 9:18 p.m.)
Deposited	1 year, 11 months ago (Sept. 17, 2023, 3:40 a.m.)
Indexed	3 days, 23 hours ago (Aug. 23, 2025, 9:39 p.m.)
Issued	22 years, 4 months ago (March 29, 2003)
Published	22 years, 4 months ago (March 29, 2003)
Published Online	22 years, 4 months ago (March 29, 2003)
Published Print	22 years, 4 months ago (April 24, 2003)

Funders 0

None

BibTeX

@article{Mao_2003, title={Membrane topology of CLN3, the protein underlying Batten disease}, volume={541}, ISSN={1873-3468}, url={http://dx.doi.org/10.1016/s0014-5793(03)00284-9}, DOI={10.1016/s0014-5793(03)00284-9}, number={1–3}, journal={FEBS Letters}, publisher={Wiley}, author={Mao, Qinwen and Foster, Brian J and Xia, Haibin and Davidson, Beverly L}, year={2003}, month=mar, pages={40–46} }

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