Reaction: SMPD4 hydrolyzes sphingomyelin (ER membrane)
- in pathway: Glycosphingolipid catabolism
ER membrane-bound sphingomyelin phosphodiesterase 4 (SMPD4) hydrolyses sphingomyelin to ceramide (Krut et al. 2006). Loss-of-function mutations in the SMPD4 gene cause neurodevelopmental disorder with microcephaly, arthrogryposis, and structural brain anomalies (NEDMABA) (Magini et al., 2019).
Reaction - small molecule participants:
H+ [cytosol]
CERA [endoplasmic reticulum membrane]
ChoP [endoplasmic reticulum lumen]
H2O [endoplasmic reticulum lumen]
SPHM [endoplasmic reticulum membrane]
Reactome.org reaction link: R-HSA-1606288
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Reaction input - small molecules:
water
sphingomyelin d18:1
Reaction output - small molecules:
hydron
N-acylsphingoid
phosphocholine
Reactome.org link: R-HSA-1606288