PXLP-SGPL1 cleaves sphingoid-1-phosphates

Stable Identifier
R-HSA-428681
Type
Reaction [transition]
Species
Homo sapiens
Compartment
Synonyms
sphinganine 1-phosphate => phosphoethanolamine + hexadecanal
ReviewStatus
5/5
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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
Participants
Participates
Catalyst Activity

sphinganine-1-phosphate aldolase activity of PXLP-SGPL1 [endoplasmic reticulum membrane]

Orthologous Events
Cross References
Rhea
Authored
Reviewed
Created
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