Reactome | SLC33A1 transports Ac-CoA from cytosol to Golgi lumen

SLC33A1 transports Ac-CoA from cytosol to Golgi lumen

Stable Identifier

R-HSA-727759

Type

Reaction [transition]

Species

Homo sapiens

Compartment

Golgi membrane

ReviewStatus

5/5

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SLC33A1 transports Ac-CoA from cytosol to Golgi lumen

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The human gene SLC33A1 encodes acetyl-CoA transporter AT1 (Kanamori et al. 1997). Acetyl-CoA is transported to the lumen of the Golgi apparatus, where it serves as the substrate of acetyltransferases that O-acetylates sialyl residues of gangliosides and glycoproteins. Defects in SLC33A1 are the cause of spastic paraplegia autosomal dominant type 42 (SPG42) which is a neurodegenerative disorder (Lin et al. 2008).

Literature References

PubMed ID	Title	Journal	Year
9096318	Expression cloning and characterization of a cDNA encoding a novel membrane protein required for the formation of O-acetylated ganglioside: a putative acetyl-CoA transporter Kanamori, A, Nakayama, J, Fukuda, MN, Stallcup, WB, Sasaki, K, Fukuda, M, Hirabayashi, Y	Proc Natl Acad Sci U S A	1997
19061983	A missense mutation in SLC33A1, which encodes the acetyl-CoA transporter, causes autosomal-dominant spastic paraplegia (SPG42) Lin, P, Li, J, Li, J, Liu, Q, Mao, F, Qiu, R, Hu, H, Song, Y, Yang, Y, Gao, G, Yan, C, Yang, W, Shao, C, Gong, Y	Am J Hum Genet	2008