# The Role of RanBP2 in the pathogenesis of acute necrotizing encephalopathy

> **NIH NIH R21** · JOHNS HOPKINS UNIVERSITY · 2021 · $450,313

## Abstract

Project Summary
 Acute necrotizing encephalopathy (ANE) is a devastating neurologic condition associated with
systemic infections, including influenza and SARS-CoV2, the causative agent of Covid-19. During or
just following infection, affected individuals develop lesions in multiple brain areas and rapidly develop
changes in consciousness, seizures, and coma. The disorder preferentially affects children, and
about one third of affected individuals die. Half of all survivors are left with substantial physical and
cognitive disability.
 Little is known about how ANE arises. An increase in inflammatory molecules – termed
cytokines- in the blood and spinal fluid of patients has been noted, leading to the hypothesis that a
“cytokine storm” plays a major role. A genetic mutation has been identified in a protein that places
individuals at risk for ANE, but how that mutation affects brain cells and how it may be related to the
inflammation remains unclear.
 Here, we propose to develop a model of ANE based on human induced pluripotent stem cell
based approaches. The advantages of this model are that we will be able to study human neurons
and supporting cells, as opposed to rodent models that may be less relevant to the disease. We
anticipate that establishment of our model will yield initial insights into how ANE arises and will
eventually lead to the development of preventative or therapeutic measures to combat this
devastating disorder.

## Key facts

- **NIH application ID:** 10190405
- **Project number:** 1R21NS121462-01
- **Recipient organization:** JOHNS HOPKINS UNIVERSITY
- **Principal Investigator:** ARUN VENKATESAN
- **Activity code:** R21 (R01, R21, SBIR, etc.)
- **Funding institute:** NIH
- **Fiscal year:** 2021
- **Award amount:** $450,313
- **Award type:** 1
- **Project period:** 2021-04-01 → 2024-09-30

## Primary source

NIH RePORTER: https://reporter.nih.gov/project-details/10190405

## Citation

> US National Institutes of Health, RePORTER application 10190405, The Role of RanBP2 in the pathogenesis of acute necrotizing encephalopathy (1R21NS121462-01). Retrieved via AI Analytics 2026-05-24 from https://api.ai-analytics.org/grant/nih/10190405. Licensed CC0.

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