Reactome | CLN3 transports GalCer to plasma membrane

CLN3 transports GalCer to plasma membrane

Stable Identifier

R-HSA-9844955

Type

Reaction [uncertain]

Species

Homo sapiens

Compartment

Golgi membrane, plasma membrane

ReviewStatus

5/5

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CLN3 transports GalCer to plasma membrane

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CLN3 (neuronal ceroid-lipofuscinosis 3) protein (battenin), which has five transmembrane domains, including a GalCer lipid raft-binding domain, is responsible for the transport of GalCer from ER/Golgi to lipid rafts in the membrane. The details of this process need to be clarified (Rusyn et al., 2008; reviewed by Liu & Li, 2013). CLN3 transport of proteins and glycolipids to the plasma membrane and lysosomes is part of various processes. Deficiencies in CLN3 are the cause of neuronal ceroid-lipofuscinosis 3 (CLN3, "Batten's disease," MIM:204200; reviewed by Cotman & Staropoli, 2012; Mirza et al., 2019).

Literature References

PubMed ID	Title	Journal	Year
22545070	The juvenile Batten disease protein, CLN3, and its role in regulating anterograde and retrograde post-Golgi trafficking Cotman, SL, Staropoli, JF	Clin Lipidol	2012
23290777	Ceramide glycosylation catalyzed by glucosylceramide synthase and cancer drug resistance Liu, YY, Hill, RA, Li, YT	Adv. Cancer Res.	2013
31568712	The CLN3 gene and protein: What we know Mirza, M, Vainshtein, A, DiRonza, A, Chandrachud, U, Haslett, LJ, Palmieri, M, Storch, S, Groh, J, Dobzinski, N, Napolitano, G, Schmidtke, C, Kerkovich, DM	Mol Genet Genomic Med	2019
18317235	CLN3p impacts galactosylceramide transport, raft morphology, and lipid content Rusyn, E, Mousallem, T, Persaud-Sawin, DA, Miller, S, Boustany, RM	Pediatr Res	2008