# The pathophysiology of vascular remodeling in pulmonary hypertension

> **NIH NIH R01** · BAYLOR COLLEGE OF MEDICINE · 2024 · $764,891

## Abstract

Pulmonary hypertension (PH) represents a grave and relentless medical condition, characterized
clinically by elevated pulmonary arterial pressure and augmented pulmonary vascular resistance
(PVR). This pathophysiological state inevitably progresses to the development of right ventricular
(RV) hypertrophy (RVH), culminating in RV failure, and ultimately, mortality. Moreover, PH could
be a confounding complication of chronic lung pathology particularly secondary to interstitial lung
disease (ILD), including its severe type- Idiopathic pulmonary fibrosis (IPF). The primary driver of
increased PVR in ILD-PH, similarly as in other PH subtypes, is pulmonary vascular remodeling,
a complex process involving various cells in pulmonary arterial wall. However, the progression of
PH from ILD remains largely unknown. Recently, the first inhaled vasoactive drug-treprostinil was
approved for treating ILD-PH. However, concerns about its potential side effects and high costs
highlight the urgent need for innovative strategies to manage ILD-PH. Unfortunately, there is
currently no treatment specifically designed to target vascular remodeling in ILD-PH.
Bone morphogenetic protein (BMP)-binding endothelial regulator (BMPER) is highly expressed in
the lung; however, our understanding about its role in lung diseases is very limited. BMPER is a
secreted regulator that regulates vascular development via fine-tuning the BMP pathway. BMP
plays a pivotal role in the development of pulmonary arterial hypertension. Animal models
targeting BMP pathway components (i.e. BMPR2, Gremlin1) also illuminate their regulatory
functions in other forms of PH, including that associated with ILD. However, the role of BMPER
has not been studied in any forms of PH. In this study, we will define a new and lung-specific
paracrine regulatory mechanism mediated via BMPER for vascular remodeling during the
progression of ILD-PH. First, we will define the transcriptional regulation of BMPER during the
progression of ILD-PH. Next, we will establish mechanisms governing BMPER regulation of
pulmonary vascular remodeling. Last, we will assess the tissue-specific BMPER expression in the
development of vasculopathy in patients with ILD-PH.

## Key facts

- **NIH application ID:** 10980237
- **Project number:** 1R01HL169297-01A1
- **Recipient organization:** BAYLOR COLLEGE OF MEDICINE
- **Principal Investigator:** LAVANNYA M. PANDIT
- **Activity code:** R01 (R01, R21, SBIR, etc.)
- **Funding institute:** NIH
- **Fiscal year:** 2024
- **Award amount:** $764,891
- **Award type:** 1
- **Project period:** 2024-09-06 → 2028-06-30

## Primary source

NIH RePORTER: https://reporter.nih.gov/project-details/10980237

## Citation

> US National Institutes of Health, RePORTER application 10980237, The pathophysiology of vascular remodeling in pulmonary hypertension (1R01HL169297-01A1). Retrieved via AI Analytics 2026-05-24 from https://api.ai-analytics.org/grant/nih/10980237. Licensed CC0.

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