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Target intelligence / Profile preview
Nuclear envelope phosphatase 1 regulatory subunit 1 (CNEP1R1)
- Target
- CNEP1R1
- Molecular classification
- Regulatory subunit of a membrane protein phosphatase complex, Transmembrane protein, Phosphatase regulator, Tmemb_18A family member
01
Overview
Nuclear envelope phosphatase 1 regulatory subunit 1 (CNEP1R1) is a transmembrane protein that acts as an activating regulatory subunit for the serine/threonine phosphatase CTDNEP1. Together, CNEP1R1 and CTDNEP1 form a catalytically active complex localized to ER and nuclear envelope membranes, critical for restricting ER expansion and regulating lipid biosynthesis. CNEP1R1 directly binds CTDNEP1, enhancing its phosphatase activity—especially enabling the dephosphorylation and activation of the lipid phosphatases LPIN1 and LPIN2, which convert phosphatidic acid to diacylglycerol and influence cellular lipid composition. The biological role of CNEP1R1 is evolutionarily conserved (e.g., Nem1–Spo7 in yeast) and is essential for proper membrane morphology and function. Dysregulation of this complex has implications for rare hereditary bone diseases and cancer[1][2][3][4][5][6].
- Other names
- NEP1-R1TMEM188C16orf69FLJ38101Transmembrane protein 188NEP1R1TMP125
02
Biological functions
Regulation of ER and nuclear envelope morphologyActivation of serine/threonine phosphatase CTDNEP1Modulation of lipid metabolism via LPIN1 and LPIN2 dephosphorylationPositive regulation of triacylglycerol biosynthesis
03
Disease associations
Endosteal hyperostosis (autosomal dominant)Indirectly associated with cancer via CTDNEP1 mutations, especially medulloblastomaPotential link to defects in lipid metabolism
04
Safety considerations
Loss-of-function or mutational dysfunctions in the complex, particularly CTDNEP1, are linked to cancer (e.g. medulloblastoma) and abnormal lipid storage, implying that therapeutic manipulation may impact ER/nuclear envelope integrity and lipid metabolismOther safety concerns are not established due to the lack of direct clinical targeting
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