8 results for "Brian L. Lin"

Transcriptomic entropy benchmarks stem cell-derived cardiomyocyte maturation against endogenous tissue at single cell level.

Suraj Kannan; Michael Farid; Brian L Lin et al.

PLoS Computational Biology, Vol. 17 No. 9 · 2021 · English · DOAJ Scopus

The immaturity of pluripotent stem cell (PSC)-derived tissues has emerged as a universal problem for their biomedical applications. While efforts have been made to generate adult-like cells from PSCs, direct benchmarking of PSC-derived tissues against in vivo development has not been established…

Also available at: PLOS

Potential limitations of microdystrophin gene therapy for Duchenne muscular dystrophy

Cora C. Hart; Young il Lee; Jun Xie et al.

JCI Insight, Vol. 9 No. 11 · 2024 · English · DOAJ Scopus

Clinical trials delivering high doses of adeno-associated viruses (AAVs) expressing truncated dystrophin molecules (microdystrophins) are underway for Duchenne muscular dystrophy (DMD). We examined the efficiency and efficacy of this strategy with 4 microdystrophin constructs (3 in clinical trials…

Depletion of skeletal muscle satellite cells attenuates pathology in muscular dystrophy

Justin G. Boyer; Jiuzhou Huo; Sarah Han et al.

Nature Communications, Vol. 13 No. 1 · 2022 · English · DOAJ Scopus

Boyer et al. created genetic mouse models of muscular dystrophy in which satellite cells were selectively depleted. The depletion of satellite cells at select times was protective. Myofibers no longer had plasma membrane instability leading to tissue wasting in the muscular dystrophies.

Single cell RNA-seq analysis of the flexor digitorum brevis mouse myofibers

Rohan X. Verma; Suraj Kannan; Brian L. Lin et al.

Skeletal Muscle, Vol. 11 No. 1 · 2021 · English · DOAJ Scopus

Abstract Background Skeletal muscle myofibers can be separated into functionally distinct cell types that differ in gene and protein expression. Current single cell expression data is generally based upon single nucleus RNA, rather than whole myofiber material. We examined if a whole-cell flow…

HIF2A gain-of-function mutation modulates the stiffness of smooth muscle cells and compromises vascular mechanics

Xin Yi Chan; Eugenia Volkova; Joon Eoh et al.

iScience, Vol. 24 No. 4 · 2021 · English · DOAJ Scopus

Summary: Heterozygous gain-of-function (GOF) mutations of hypoxia-inducible factor 2α (HIF2A), a key hypoxia-sensing regulator, are associated with erythrocytosis, thrombosis, and vascular complications that account for morbidity and mortality of patients. We demonstrated that the vascular…

Pharmacological TRPC6 inhibition improves survival and muscle function in mice with Duchenne muscular dystrophy

Brian L. Lin; Joseph Y. Shin; William P.D. Jeffreys et al.

JCI Insight, Vol. 7 No. 19 · 2022 · English · DOAJ Scopus

Gene mutations causing loss of dystrophin result in the severe muscle disease known as Duchenne muscular dystrophy (DMD). Despite efforts at genetic repair, DMD therapy remains largely palliative. Loss of dystrophin destabilizes the sarcolemmal membrane, inducing mechanosensitive cation channels to…

Innate immune activation and mitochondrial ROS induce acute and persistent cardiac conduction system dysfunction after COVID-19

Deepthi Ashok; Ting Liu; Misato Nakanishi-Koakutsu et al.

JCI Insight, Vol. 10 No. 24 · 2025 · English · DOAJ Scopus

Cardiac arrhythmias increase during acute SARS-CoV-2 infection and in long COVID syndrome, by unknown mechanisms. This study explored the acute and long-term effects of COVID-19 on cardiac electrophysiology and the cardiac conduction system (CCS) in a hamster model. Electrocardiograms and…