scispace - formally typeset
S

Stephan Rust

Researcher at Boston Children's Hospital

Publications -  78
Citations -  7297

Stephan Rust is an academic researcher from Boston Children's Hospital. The author has contributed to research in topics: Glycosylation & Congenital disorder of glycosylation. The author has an hindex of 36, co-authored 78 publications receiving 6626 citations. Previous affiliations of Stephan Rust include University of Münster & Leibniz Institute for Neurobiology.

Papers
More filters
Journal ArticleDOI

Tangier disease is caused by mutations in the gene encoding ATP-binding cassette transporter 1

TL;DR: Tangier disease (TD) was first discovered nearly 40 years ago in two siblings living on Tangier Island This autosomal co-dominant condition is characterized in the homozygous state by the absence of HDL-cholesterol (HDL-C) from plasma, hepatosplenomegaly, peripheral neuropathy and frequently premature coronary artery disease (CAD).
Journal ArticleDOI

Human ATP-binding cassette transporter 1 (ABC1): Genomic organization and identification of the genetic defect in the original Tangier disease kindred

TL;DR: The genomic organization of the human ABC1 gene is reported and a frameshift mutation in the ABC2 gene of the index case of Tangier disease is identified, which will be useful in the future characterization of the structure and function of theABC1 gene and the analysis of additional ABC1 mutations in patients withTangier disease.
Journal ArticleDOI

Mutagenically separated PCR (MS-PCR): a highly specific one step procedure for easy mutation detection.

TL;DR: A PCR-based technique, whereby both normal and mutant alleles can be amplified in the same reaction tube, using different length allele-specific primers, which provides a within-assay quality control for the exclusion of false negative results.