# Role of Sphingolipid pathways in the pathobiology of PAH

> **NIH NIH R01** · INDIANA UNIVERSITY INDIANAPOLIS · 2020 · $702,584

## Abstract

ABSTRACT
Pulmonary arterial hypertension (PAH), a rare, debilitating and fatal disease for which there is currently no
available cure. There is compelling evidence that the Sphingosine kinase1/S1P signaling axis is a novel and
therapeutic target for PAH. To facilitate the translation of current in vivo and in vitro observations on the role
of S1P dependent signaling in PAH pathobiology, this proposal will explore the hypothesis that the Sphingosine
kinase1/S1P signaling axis regulates physiologic, cellular and molecular pathways in PAH that result in
pulmonary vascular remodeling. SA1 seeks to define the role of LncRNA Khps1 in miR-1 and SPHK1 expression
and promoting pulmonary vascular remodeling. SA2 seeks to define molecular mechanisms by which LncRNA
Khps1/SPHK1 regulate pulmonary artery smooth muscle cell mitochondrial dynamics. SA3 will investigate the
role of SPHK1 in aberrant chromatin remodeling and gene expression in the development of pulmonary vascular
remodeling.

## Key facts

- **NIH application ID:** 10072350
- **Project number:** 2R01HL127342-05
- **Recipient organization:** INDIANA UNIVERSITY INDIANAPOLIS
- **Principal Investigator:** Roberto F. Machado
- **Activity code:** R01 (R01, R21, SBIR, etc.)
- **Funding institute:** NIH
- **Fiscal year:** 2020
- **Award amount:** $702,584
- **Award type:** 2
- **Project period:** 2016-01-05 → 2024-06-30

## Primary source

NIH RePORTER: https://reporter.nih.gov/project-details/10072350

## Citation

> US National Institutes of Health, RePORTER application 10072350, Role of Sphingolipid pathways in the pathobiology of PAH (2R01HL127342-05). Retrieved via AI Analytics 2026-05-21 from https://api.ai-analytics.org/grant/nih/10072350. Licensed CC0.

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