PIPages
Labs

Timothy Blackwell

University of Michigan, MI·Medicine & Clinical Research

Pulmonary fibrosis slowly replaces the parts of the lung that exchange gas with scar tissue. It often leads to respiratory failure and death.

Fields

Medicine & Clinical Research

Biology

Genetics

Topics

pulmonary fibrosis

interstitial lung abnormalities

high-resolution CT

Grants
3 known grants, through Jul 2029

What they work on

  1. Detect gene activity biomarkers of pre-clinical disease in asymptomatic relatives of familial pulmonary fibrosis patients, using single-cell RNA sequencing of blood immune cells such as CD14+ monocytes alongside high-resolution CT findings.
  2. Identify cell signatures of early disease in the lungs of at-risk relatives by single-cell profiling of bronchoalveolar lavage cells, which are collected by washing the airways.
  3. Integrate gene activity data with epigenetic age, genetic susceptibility and telomere length to find features of early disease activity that predict progression of pre-clinical pulmonary fibrosis.
  4. Identify factors that drive abnormal transitional epithelial cell states in persistent lung fibrosis, using the repetitive bleomycin mouse model, single-cell RNA sequencing and cell fate mapping.
  5. Determine whether hypoxia-inducible factor (HIF) signaling, especially HIF2, blocks airway progenitors from rebuilding air sacs, using epithelial HIF deletion, a HIF2 inhibitor, and mouse and human alveolar organoids.
  6. Investigate the local niche signals, from neighboring cells, that sustain persistent lung fibrosis after repeated injury in mice.

More about the research

By the time patients have symptoms, much damage is already done. This research has two connected aims. One is to understand the long silent phase before symptoms appear, so that the disease might one day be prevented. The other is to learn why the lung's surface lining fails to repair properly after repeated injury. One line of work studies relatives of patients with familial pulmonary fibrosis (FPF), an inherited form of the disease. The other uses mouse models and lab-grown mini-lungs to test the signaling pathways behind faulty repair.

Why it matters

Pulmonary fibrosis is often fatal, and current treatments only slow the scarring once it has started. Blood and imaging markers that flag at-risk relatives of familial pulmonary fibrosis patients before symptoms appear could allow earlier monitoring and future prevention trials. Showing how HIF2 signaling blocks proper lung repair could point to new drug strategies that improve healing in idiopathic pulmonary fibrosis.

Known grants

From NIH and NSF research grants. Labs often have other funding we can't see.

Project 1: Mechanisms of Early Disease Progression in FPF

NIH · Sep 2024 – Jul 2029

Disease Mechanisms of Early Pulmonary Fibrosis

NIH · Sep 2024 – Jul 2029

Targeting dysfunctional epithelial repair in pulmonary fibrosis

NIH · Sep 2024 – Jun 2028

Closest labs

Beiyun Zhou

University of Southern California, CA·Physiology

Identify how loss of the endoplasmic reticulum chaperone GRP78 disturbs mitochondrial energy production and biosynthesis in alveolar epithelial cells, and how this promotes pulmonary fibrosis, using AT2 cell-specific Grp78 knockout mice.

Yvonne M. W. Janssen-Heininger

University of Vermont, VT·Biochemistry

Determine whether the enzyme GSTP drives glutathionylation of OTUB1 and keeps KRT8-high transitional lung cells from maturing into AT1 cells, using a clinically tested GSTP inhibitor and IPF patient organoids.

Yong Huang

University of Virginia, VA·Medicine & Clinical Research

Test whether reducing lung stretch by paralyzing one side of the diaphragm slows fibrosis progression in the outer and lower lung of mice with bleomycin-induced lung injury.

The school

University of Michigan: 124 labs on PI Pages, $2.1B a year on research, 963 research doctorates a year.

More about University of Michigan

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