Cargando...

Impaired Elastogenesis in Hurler Disease : Dermatan Sulfate Accumulation Linked to Deficiency in Elastin-Binding Protein and Elastic Fiber Assembly

Hurler disease resulting from a deficiency in α-l-iduronidase, which causes an accumulation of dermatan sulfate and heparan sulfate glycosaminoglycans, is characterized by connective tissue and skeletal deformations, cardiomyopathy, cardiac valve defects, and progressive coronary artery stenosis. In...

Descrición completa

Gardado en:
Detalles Bibliográficos
Main Authors: Hinek, Aleksander, Wilson, Sarah E.
Formato: Artigo
Idioma:Inglês
Publicado: American Society for Investigative Pathology 2000
Assuntos:
Acceso en liña:https://ncbi.nlm.nih.gov/pmc/articles/PMC1876830/
https://ncbi.nlm.nih.gov/pubmed/10702409
Tags: Engadir etiqueta
Sen Etiquetas, Sexa o primeiro en etiquetar este rexistro!