Reactome | KDSR reduces 3-ketosphingoid

KDSR reduces 3-ketosphingoid

Stable Identifier

R-HSA-428123

Type

Reaction [transition]

Species

Homo sapiens

Compartment

cytosol, endoplasmic reticulum membrane

Synonyms

3-ketosphingoid + NADPH + H+ => sphingoid + NADP+

ReviewStatus

5/5

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KDSR (3-ketodihydrosphingosine reductase) enzyme associated with the cytosolic face of the endoplasmic reticulum membrane catalyzes the reduction of 3-ketosphingoid by NADPH to form sphingoid (Kihara and Igarashi 2004). Defects in KDSR or missing activity causes Erythrokeratodermia (EKVP4, MIM:617526), associated with sphingolipid dysregulation (Boyden et al., 2017). While there is no data on substrates other than 3-ketosphinganine, it is probable that KDSR has activity toward 3-ketosphingoids with other chain lengths than C16.

Literature References

PubMed ID	Title	Journal	Year
15328338	FVT-1 is a mammalian 3-ketodihydrosphingosine reductase with an active site that faces the cytosolic side of the endoplasmic reticulum membrane Kihara, A, Igarashi, Y	J Biol Chem	2004
28575652	Mutations in KDSR Cause Recessive Progressive Symmetric Erythrokeratoderma Boyden, LM, Vincent, NG, Zhou, J, Hu, R, Craiglow, BG, Bayliss, SJ, Rosman, IS, Lucky, AW, Diaz, LA, Goldsmith, LA, Paller, AS, Lifton, RP, Baserga, SJ, Choate, KA	Am J Hum Genet	2017