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Heparan sulfate storage in the cardiac conduction system triggers atrioventricular block

Rie Kato1, Hiroaki Miyahara2, Tatsuya Kawano2

  • 1Department of Pediatrics and Child Neurology, Oita University School of Medicine, Yufu, Oita 8795593, Japan; Department of Pediatrics and Child Neurology, Beppu Developmental Medical Center for Cerebral Palsy, Mental Retardation and Severely Handicapped Children, Beppu, Oita 8740838, Japan.

Brain & Development
|January 17, 2017
PubMed

Insights

Heparan sulfate (HS) accumulation in the cardiac conduction system (CCS) may cause atrioventricular (AV) block. This study highlights HS

Area of Science:

  • Biochemistry
  • Cardiology
  • Genetics

Background:

  • Heparan sulfate (HS) is a complex polysaccharide with diverse biological roles.
  • Sanfilippo syndrome type A is a lysosomal storage disorder affecting HS metabolism.
  • Cardiac conduction system (CCS) disorders, like atrioventricular (AV) block, can have various underlying causes.
Abstract

Keywords:
Atrioventricular blockCardiac conduction systemHeparan sulfateLong-surviving patientSanfilippo syndrome

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