PPARα regulates ER–lipid droplet protein Calsyntenin-3β to promote ketogenesis in hepatocytes

L Lauren F. Uchiyama (Department of Pathology and Laboratory Medicine, David Geffen School of Medicine, University of California) A Alexander Nguyen (Department of Medicine, Division of Digestive Diseases, David Geffen School of Medicine, University of California) K Kevin Qian (Department of Chemistry) L Liujuan Cui (Department of Pathology and Laboratory Medicine, David Geffen School of Medicine, University of California) K Khoi T. Pham (Department of Pathology and Laboratory Medicine, David Geffen School of Medicine, University of California) X Xu Xiao (Department of Pathology and Laboratory Medicine, David Geffen School of Medicine, University of California) Y Yajing Gao (Department of Pathology and Laboratory Medicine, David Geffen School of Medicine, University of California) Y Yuta Shimanaka (Department of Pathology and Laboratory Medicine, David Geffen School of Medicine, University of California) M Marcus J. Tol (Department of Pathology and Laboratory Medicine, David Geffen School of Medicine, University of California) L Laurent Vergnes (Department of Human Genetics, University of California) K Karen Reue (Department of Human Genetics, University of California) P Peter Tontonoz (Department of Pathology and Laboratory Medicine, David Geffen School of Medicine, University of California)

Abstract

Ketogenesis requires fatty acid flux from intracellular (lipid droplets) and extrahepatic (adipose tissue) lipid stores to hepatocyte mitochondria. However, whether interorganelle contact sites regulate this process is unknown. Recent studies have revealed a role for Calsyntenin-3β (CLSTN3β), an endoplasmic reticulum–lipid droplet contact site protein, in the control of lipid utilization in adipose tissue. Here, we show that Clstn3b expression is induced in the liver by the nuclear receptor PPARα in settings of high lipid utilization, including fasting and ketogenic diet feeding. Hepatocyte-specific loss of CLSTN3β in mice impairs ketogenesis independent of changes in PPARα activation. Conversely, hepatic overexpression of CLSTN3β promotes ketogenesis in mice. Mechanistically, CLSTN3β affects LD–mitochondria crosstalk, as evidenced by changes in fatty acid oxidation, lipid-dependent mitochondrial respiration, and the mitochondrial integrated stress response. These findings define a function for CLSTN3β-dependent membrane contacts in hepatic lipid utilization and ketogenesis.

Article Details

Volume / Issue Vol. 122, Issue 17
Published April 29, 2025
ISSN 0027-8424
Publisher National Academy of Sciences

Authors (12)

L

Lauren F. Uchiyama

Department of Pathology and Laboratory Medicine, David Geffen School of Medicine, University of California

A

Alexander Nguyen

Department of Medicine, Division of Digestive Diseases, David Geffen School of Medicine, University of California

K

Kevin Qian

Department of Chemistry

L

Liujuan Cui

Department of Pathology and Laboratory Medicine, David Geffen School of Medicine, University of California

K

Khoi T. Pham

Department of Pathology and Laboratory Medicine, David Geffen School of Medicine, University of California

X

Xu Xiao

Department of Pathology and Laboratory Medicine, David Geffen School of Medicine, University of California

Y

Yajing Gao

Department of Pathology and Laboratory Medicine, David Geffen School of Medicine, University of California

Y

Yuta Shimanaka

Department of Pathology and Laboratory Medicine, David Geffen School of Medicine, University of California

M

Marcus J. Tol

Department of Pathology and Laboratory Medicine, David Geffen School of Medicine, University of California

L

Laurent Vergnes

Department of Human Genetics, University of California

K

Karen Reue

Department of Human Genetics, University of California

P

Peter Tontonoz

Department of Pathology and Laboratory Medicine, David Geffen School of Medicine, University of California