Search results for KDSR

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Species

Types

Compartments

Reaction types

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Protein (1 results from a total of 1)

Identifier: R-HSA-428134
Species: Homo sapiens
Compartment: endoplasmic reticulum membrane
Primary external reference: UniProt: KDSR: Q06136

Reaction (1 results from a total of 1)

Identifier: R-HSA-428123
Species: Homo sapiens
Compartment: cytosol, endoplasmic reticulum membrane
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.
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