Reactome | PXLP-SGPL1 cleaves sphingoid-1-phosphates

PXLP-SGPL1 cleaves sphingoid-1-phosphates

Stable Identifier

R-HSA-428681

Type

Reaction [transition]

Species

Homo sapiens

Compartment

cytosol, endoplasmic reticulum membrane

Synonyms

sphinganine 1-phosphate => phosphoethanolamine + hexadecanal

ReviewStatus

5/5

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PXLP-SGPL1 cleaves sphingoid-1-phosphates

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SGPL1 (sphingosine-1-phosphate lyase 1), associated with the endoplasmic reticulum membrane, catalyzes the cleavage of cytosolic sphinganine (dihydrosphingosine) or sphngosin\e 1-phosphate to form phosphoethanolamine and hexadecanal or hexadecenal, respectively (Van Veldhoven et al. 2000; reviewed by Fyrst and Saba 2008; Saba, 2019). The reaction has no reverse counterpart and, thus, irreversibly removes sphingoids from the lipid pool. SGPL1 deficiency causes a form of nephrotic syndrome, a renal disease clinically characterized by severe proteinuria (NPHS14, MIM:617575; reviewed by Duarte Linhares et al., 2018).

Literature References

PubMed ID	Title	Journal	Year
30090628	Nephrotic syndrome and adrenal insufficiency caused by a variant in SGPL1 Linhares, ND, Araujo, SA, Pena, SDJ, Arantes, RR	Clin Kidney J	2018
11018465	Human sphingosine-1-phosphate lyase: cDNA cloning, functional expression studies and mapping to chromosome 10q22(1) Brys, V, Vermeesch, JR, Van Veldhoven, PP, Mannaerts, GP, Gijsbers, S	Biochim Biophys Acta	2000
18558101	Sphingosine-1-phosphate lyase in development and disease: sphingolipid metabolism takes flight Fyrst, H, Saba, JD	Biochim Biophys Acta	2008
30635364	Fifty years of lyase and a moment of truth: sphingosine phosphate lyase from discovery to disease Saba, JD	J Lipid Res	2019