Human MORC3 Protein expressed in HEK-293 Cells - ABIN2726253
Tansley, Betteridge, Shaddick, Gunawardena, Arnold, Wedderburn, McHugh: Calcinosis in juvenile dermatomyositis is influenced by both anti-NXP2 autoantibody status and age at disease onset. in Rheumatology (Oxford, England) 2014
Show all 3 Pubmed References
Mechanism for autoinhibition and activation
our studies provide a molecular framework detailing MORC3 functions and suggest that its modulation may contribute to human disease.
Dermatomyositis patients with anti-NXP-2 antibodies have a distinct and often severe systemic phenotype that includes myalgia, peripheral edema, and significant dysphagia, despite having milder inflammatory skin disease.
MORC3 has antiviral activity during herpes simplex virus 1 and human cytomegalovirus infections.
Case Report: manifestations of interstitial lung disease in adult dermatomyositis patient with anti-NPX2 autoantibodies.
Downregulation of NXP2/MORC3 by use of two independent short hairpin RNAs reduced virus titers in low-multiplicity infections. Analysis of viral RNA in high-multiplicity infections showed a reduction of viral RNA and mRNA after NXP2/MORC3 downregulation.
Case Report: Pemphigus foliaceus associated with anti-NXP2 autoantibody-positive amyopathic dermatomyositis.
These studies demonstrate that anti-NXP-2 and anti-TIF-1gamma antibodies are frequent DM specificities (found in 55% of patients) and are present in most patients with cancer-associated dermatomhyositis.
An anti-MJ antibody that recognizes NXP-2 was found to be a useful biomarker in dermatomyositis-polymyositis patients.
Anti-NXP2 Ab may be associated with adult idiopathic inflammatory myopathies with malignancy.
MORC3 colocalizes with PML by a two-step molecular mechanism.
determination of nuclear matrix-binding, RNA-binding, and coiled-coil domans
When tethered to a promoter by fusion to Gal4, NXP-2 repressed transcription, consistent with a role for NXP-2 in SUMO-mediated repression
MORC3 (microrchidia3)-ATPase activated p53 and induced cellular senescence in normal fibroblasts but not p53-/- fibroblasts
Data show that the MORC3 N-terminal ATPase domain forms a dimer when bound to AMPPNP.
Data indicate that Morc3 mutation leads to altered nuclear localization of the protein, and upregulation of the IFN-beta /STAT1 pathway, which plays a critical role in the maintenance of bone homeostasis. These findings establish Morc3 as a previously unreported regulator of cortical bone homeostasis and haematopoietic stem cells niche, accompanied by altered bone cell differentiation.
This gene encodes a protein that localizes to the nuclear matrix. The protein also has RNA binding activity, and has a predicted coiled-coil domain.
MORC family CW-type zinc finger protein 3
, nuclear matrix protein NXP2
, zinc finger CW-type coiled-coil domain protein 3
, zinc finger, CW type with coiled-coil domain 3
, zinc finger, CW-type with coiled-coil domain 3
, microrchidia 3
, MORC family CW-type zinc finger 3b
, MORC family CW-type zinc finger 3 S homeolog