The structure of EXTL3 helps to explain the different roles of bi-domain exostosins in heparan sulfate synthesis

LFL Wilson, T Dendooven, SW Hardwick… - Nature …, 2022 - nature.com
Heparan sulfate is a highly modified O-linked glycan that performs diverse physiological
roles in animal tissues. Though quickly modified, it is initially synthesised as a …

Primary and secondary defects of the thymus

SS Dinges, K Amini, LD Notarangelo… - Immunological …, 2024 - Wiley Online Library
The thymus is the primary site of T‐cell development, enabling generation, and selection of
a diverse repertoire of T cells that recognize non‐self, whilst remaining tolerant to self …

EXTL3-Associated Immunoskeletal Dysplasia with Neurodevelopmental Abnormalities: A Lethal Phenotype

E Demir, F Adım, ME Döğen, A Aydoğdu… - Pediatric Allergy …, 2023 - liebertpub.com
Background: Immunoskeletal dysplasia with neurodevelopmental abnormalities (ISDNA)
caused by Exostosin-Like Glycosyltransferase 3 (EXTL3) biallelic mutations is a very rare …

Immune skeletal dysplasia with neurodevelopmental abnormalities caused by a novel variant of EXTL3 gene in a Chinese family

X Tian, X Zhang, Q Zhang, X Chen… - … Genetics & Genomic …, 2024 - Wiley Online Library
Background Immune skeletal dysplasia with neurodevelopmental abnormalities (ISDNA) is
an extremely rare, autosomal recessive genetic disorder characterized by various skeletal …

The structure of EXTL3 helps to explain the different roles of bi-domain exostosins in heparan sulfate synthesis

P Dupree, T Dendooven, S Hardwick, T Tryfona… - 2022 - repository.cam.ac.uk
Heparan sulfate is a highly modified O-linked glycan that performs diverse physiological
roles in animal tissues. Though quickly modified, it is initially synthesised as a …