Reactome | SLC35A1 exchanges CMP-Neu5Ac for CMP

SLC35A1 exchanges CMP-Neu5Ac for CMP

Stable Identifier

R-HSA-727807

Type

Reaction [transition]

Species

Homo sapiens

Compartment

Golgi membrane, Golgi lumen, cytosol

Synonyms

SLC35A1 mediates the antiport of CMP-sialic acid in exchange for CMP

Locations in the PathwayBrowser

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Metabolism of proteins (Homo sapiens)

- Post-translational protein modification (Homo sapiens)
  - Asparagine N-linked glycosylation (Homo sapiens)
    - Biosynthesis of the N-glycan precursor (dolichol lipid-linked oligosaccharide, LLO) and transfer to a nascent protein (Homo sapiens)
      - Synthesis of substrates in N-glycan biosythesis (Homo sapiens)
        
        Sialic acid metabolism (Homo sapiens)
        
        SLC35A1 exchanges CMP-Neu5Ac for CMP (Homo sapiens)

General

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The human gene SLC35A1 encodes the CMP-sialic acid transporter which mediates the antiport of CMP-sialic acid (CMP-Neu5Ac) into the Golgi lumen in exchange for CMP (Ishida et al. 1996). Defects in SLC35A1 are the cause of congenital disorder of glycosylation type 2F (CDG2F; MIM:603585). CDGs are a family of severe inherited diseases caused by a defect in protein N-glycosylation (Martinez-Duncker et al. 2005).

Literature References

PubMed ID	Title	Journal	Year
9010752	Molecular cloning and characterization of a novel isoform of the human UDP-galactose transporter, and of related complementary DNAs belonging to the nucleotide-sugar transporter gene family Ishida, N, Miura, N, Yoshioka, S, Kawakita, M	J Biochem	1996
15576474	Genetic complementation reveals a novel human congenital disorder of glycosylation of type II, due to inactivation of the Golgi CMP-sialic acid transporter Martinez-Duncker, I, Dupré, T, Piller, V, Piller, F, Candelier, JJ, Trichet, C, Tchernia, G, Oriol, R, Mollicone, R	Blood	2005