نتایج جستجو برای: metaphyseal chondrodysplasia

تعداد نتایج: 2147  

2015
Trevor L. Cameron Katrina M. Bell Irma L. Gresshoff Lisa Sampurno Lorna Mullan Joerg Ermann Laurie H. Glimcher Raymond P. Boot-Handford John F. Bateman Gregory S. Barsh

Schmid metaphyseal chondrodysplasia (MCDS) involves dwarfism and growth plate cartilage hypertrophic zone expansion resulting from dominant mutations in the hypertrophic zone collagen, Col10a1. Mouse models phenocopying MCDS through the expression of an exogenous misfolding protein in the endoplasmic reticulum (ER) in hypertrophic chondrocytes have demonstrated the central importance of ER stre...

Journal: :Blood 2007
Amy S Rawls Alyssa D Gregory Jill R Woloszynek Fulu Liu Daniel C Link

Shwachman-Diamond syndrome (SDS) is a rare multisystem disorder characterized by exocrine pancreatic insufficiency, multilineage hematopoietic dysfunction, and metaphyseal chondrodysplasia. Bone marrow dysfunction is present in nearly all patients with SDS, with neutropenia being the most common abnormality. The majority of patients with SDS have mutations in the Shwachman Bodian Diamond syndro...

نمودار تعداد نتایج جستجو در هر سال

با کلیک روی نمودار نتایج را به سال انتشار فیلتر کنید