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Sc65-Null Mice Provide Evidence for a Novel Endoplasmic Reticulum Complex Regulating Collagen Lysyl Hydroxylation.

PLoS Genet.. 2016; 
HeardMelissa E,BesioRoberta,WeisMaryAnn,RaiJyoti,HudsonDavid M,DimoriMilena,ZimmermanSarah M,KamykowskiJeffrey A,HogueWilliam R,SwainFrances L,BurdineMarie S,MackintoshSamuel G,TackettAlan J,SuvaLarry J,EyreDavid R,Morell
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Catalog Antibody Primary antibodies were Sc65 (cat# 15288-1-AP, ProteinTech), β-actin (cat# A00702, GenScript, Piscataway, NJ, USA)… Get A Quote

摘要

Collagen is a major component of the extracellular matrix and its integrity is essential for connective tissue and organ function. The importance of proteins involved in intracellular collagen post-translational modification, folding and transport was recently highlighted from studies on recessive forms of osteogenesis imperfecta (OI). Here we describe the critical role of SC65 (Synaptonemal Complex 65, P3H4), a leprecan-family member, as part of an endoplasmic reticulum (ER) complex with prolyl 3-hydroxylase 3. This complex affects the activity of lysyl-hydroxylase 1 potentially through interactions with the enzyme and/or cyclophilin B. Loss of Sc65 in the mouse results in instability of this complex�... More

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