Glucose Uptake and Triacylglycerol Synthesis Are Increased in Barth Syndrome Lymphoblasts

Barth syndrome (BTHS) is an X-linked genetic disease resulting in loss of cardiolipin (Ptd 2 Gro). Patients may be predisposed to hypoglycemia and exhibit increases in whole-body glucose disposal rates and a higher fat mass percentage. We examined the reasons for this in BTHS lymphoblasts. BTHS lymp...

Full description

Saved in:
Bibliographic Details
Published in:Lipids 2017-02, Vol.52 (2), p.161-165
Main Authors: Mejia, Edgard M., Zinko, James C., Hauff, Kristin D., Xu, Fred Y., Ravandi, Amir, Hatch, Grant M.
Format: Article
Language:English
Subjects:
AMP-Activated Protein Kinases - metabolism
Barth syndrome
Barth Syndrome - metabolism
Biomedical and Life Sciences
Cardiolipin
Cells, Cultured
Child
Child, Preschool
Glucose
Glucose - metabolism
Glucose transport
Glucose Transporter Type 3 - metabolism
Humans
Life Sciences
Lipidology
Lymphocyte Activation
Lymphocytes - cytology
Lymphocytes - metabolism
Male
Medical Biochemistry
Medicinal Chemistry
Metabolism
Microbial Genetics and Genomics
Neurochemistry
Nutrition
Phosphorylation
Rapid Communication
Triacylglycerol synthesis
Triglycerides - metabolism
Citations: Items that this one cites
Items that cite this one
Online Access:Get full text
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary:Barth syndrome (BTHS) is an X-linked genetic disease resulting in loss of cardiolipin (Ptd 2 Gro). Patients may be predisposed to hypoglycemia and exhibit increases in whole-body glucose disposal rates and a higher fat mass percentage. We examined the reasons for this in BTHS lymphoblasts. BTHS lymphoblasts exhibited a 60% increase ( p  
ISSN:0024-4201
1558-9307
DOI:10.1007/s11745-017-4232-7