MORC3

Last update 08 May 2025

Basic Info

Synonyms

KIAA0136, MORC family CW-type zinc finger 3, MORC family CW-type zinc finger protein 3

+ [6]

Introduction

Nuclear matrix protein which forms MORC3-NBs (nuclear bodies) via an ATP-dependent mechanism and plays a role in innate immunity by restricting different viruses through modulation of the IFN response (PubMed:27440897, PubMed:34759314). Mechanistically, possesses a primary antiviral function through a MORC3-regulated element that activates IFNB1, and this function is guarded by a secondary IFN-repressing function (PubMed:34759314). Sumoylated MORC3-NBs associates with PML-NBs and recruits TP53 and SP100, thus regulating TP53 activity (PubMed:17332504, PubMed:20501696). Binds RNA in vitro (PubMed:11927593). Histone methylation reader which binds to non-methylated (H3K4me0), monomethylated (H3K4me1), dimethylated (H3K4me2) and trimethylated (H3K4me3) 'Lys-4' on histone H3 (PubMed:26933034). The order of binding preference is H3K4me3 > H3K4me2 > H3K4me1 > H3K4me0 (PubMed:26933034). (Microbial infection) May be required for influenza A transcription during viral infection (PubMed:26202233).

100 Clinical Results associated with MORC3

100 Translational Medicine associated with MORC3

0 Patents (Medical) associated with MORC3

231

Literatures (Medical) associated with MORC3

01 May 2025·Respiratory Medicine and Research

Progressive course of anti-nuclear matrix protein-2 (NXP-2) positive-interstitial lung disease

Article

Author: Synn, Andrew J ; Flashner, Bess M ; Montesi, Sydney B ; Munchel, Julia K ; Gibbons, Fiona K ; Rice, Mary B ; Imai, Ryosuke ; Hariri, Lida P ; Hallowell, Robert W ; Bermea, Rene S ; Shea, Barry S

BACKGROUNDEvaluating the etiology of interstitial lung disease (ILD) commonly involves ordering a myositis panel containing myositis-specific antibodies (MSAs), including anti-NXP-2. However, little is known about the presentation of patients with ILD and anti-NXP-2 positivity. We sought to define the course of anti-NXP-2-positive ILD in order to guide prognosis and potential treatment strategies.METHODSWe performed a retrospective chart review of patients with positive anti-NXP-2 antibodies who presented to two ILD referral centers in Boston, MA between 2012 and 2024. Patients were identified by query of the electronic medical record for patients positive for anti-NXP-2. We included those anti-NXP-2 positive patients with ILD on chest computed tomography (CT). Data regarding clinical presentation and disease course were abstracted from the medical record. For patients following longitudinally in the ILD clinic, we conducted survival analyses for ILD progression (composite of PFT progression, hospitalization, or death) using Kaplan-Meier curves and log-rank tests. Additionally, we used a Cox proportional-hazards model, adjusting for age, gender, forced vital capacity (FVC) at baseline, and immunosuppression to calculate hazard ratios. ILD patients with MSA-positive, NXP-2-negative ILD served as the comparator group.RESULTS31 patients were identified (mean 70 years, SD 9). Three were diagnosed with dermatomyositis (DM) prior to presentation, but the remaining had ILD as the only manifestation of connective tissue disease. Most (97%) patients were symptomatic with dyspnea and/or cough at presentation. Other autoantibody positivity was common; only 42% were positive for anti-NXP-2 alone without positivity for other autoimmune serologies, including MSAs known to be associated with ILD. Clinical follow up data were available for 28 patients for a median follow up period of 24 months (range <1 month-13 years). A majority (61%) were treated with immunosuppression, antifibrotics, or both. Over one third experienced acute exacerbation of ILD or death (N = 11, 35%). Progression-free survival was similar to that of other MSA-positive ILD patients, regardless of whether anti-NXP-2 was positive alone or co-positive for other autoantibodies.CONCLUSIONSWe present the largest single series of anti-NXP-2-positive ILD. Anti-NXP-2-positive ILD can occur in the absence of DM/PM and can manifest as progressive pulmonary disease that is similar to other MSA-positive ILDs.

06 Feb 2025·Cureus

Anti-nuclear Matrix Protein 2 Antibody-Positive Dermatomyositis Relapse With Preceding Panniculitis

Article

Author: Taguchi, Hirokazu ; Shigehara, Yohya ; Ueda, Yoshitaka ; Yokogawa, Naoto

Dermatomyositis (DM) is an idiopathic inflammatory myopathy characterized by cutaneous lesions such as heliotrope rash, Gottron's papules, and Gottron's sign. Panniculitis is rarely reported as a skin manifestation of DM. Herein, we present a case of anti-nuclear matrix protein 2 antibody (NXP-2)-positive DM relapse with panniculitis in the absence of muscle symptoms. A 32-year-old female patient was referred to our department. Nine years ago, she was diagnosed with DM and had received prednisolone (PSL) and tacrolimus therapy. However, she discontinued her visits four years ago. She most recently presented with erythematous lesions on the right thigh and buttocks without any muscle symptoms. Thirteen days later, she experienced upper limb weakness. Laboratory findings at her current presentation demonstrated elevated serum creatine kinase levels and positivity for anti-NXP-2 antibody. A skin biopsy of the erythema on her left thigh demonstrated findings consistent with healed panniculitis. Based on these findings, an anti-NXP-2 antibody-positive DM relapse was diagnosed. Her symptoms resolved with PSL and tacrolimus therapy. It is important to recognize that panniculitis is a significant, cutaneous manifestation of DM and can present as the initial manifestation of a DM relapse even in the absence of muscle symptoms.

16 Jan 2025·Modern Rheumatology Case Reports

A case of vesiculobullous dermatomyositis with anti-NXP-2 antibody without malignancy

Article

Author: Tanaka, Masao ; Murakami, Kosaku ; Ichimura, Yuki ; Nishino, Ichizo ; Onizawa, Hideo ; Shirakashi, Mirei ; Murata, Teruasa ; Kitoh, Akihiko ; Kaku, Yo ; Nakashima, Ran ; Naito, Ryota ; Akizuki, Shuji ; Onishi, Akira ; Okiyama, Naoko ; Yoshifuji, Hajime ; Morinobu, Akio ; Kitagori, Koji ; Inaba, Ryuta ; Hiwa, Ryosuke ; Tsuji, Hideaki

ABSTRACTVesiculobullous dermatomyositis (VD) is a rare manifestation of dermatomyositis (DM) and has been suggested to be associated with malignancy. Although the myositis-specific autoantibodies are associated with distinct clinical presentations of DM, those associated with VD remain unclear. Here, we present the case of a 54-year-old man with VD who tested positive for antinuclear matrix protein 2 (NXP-2) antibodies, one of the DM-specific autoantibodies. Serological and histopathological findings did not support autoimmune blistering disease. Physical and histological findings suggested that the severe oedema in combination with the interface dermatitis of DM contributed to blister formation. Although a systemic examination was performed, no evidence of malignancy was found. Following initiation of immunosuppressive therapy, the patient showed significant improvement in both skin lesions and myositis. This case represents the first report of anti-NXP-2-positive VD without malignancy or autoimmune blistering disease. Subcutaneous oedema, a characteristic feature of anti-NXP-2-positive DM, could be related to the formation of VD.

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MORC3

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