Elevated C1s/C1-INH in serum and plasma of myasthenia gravis patients

被引:0
|
作者
Huang, Yu-Fang [1 ]
Briggs, Caitlin M. [2 ]
Gokhale, Sankalp [2 ]
Punga, Anna Rostedt [1 ]
机构
[1] Uppsala Univ, Dept Med Sci, Clin Neurophysiol, Uppsala, Sweden
[2] Dianthus Therapeut, New York, NY USA
关键词
Myasthenia gravis; Complement system; C1s; Biomarker; MOTOR END-PLATE; COMPLEMENT; COMPLEXES; LOCALIZATION; ACTIVATION; C1; C3;
D O I
10.1016/j.jneuroim.2024.578447
中图分类号
R392 [医学免疫学]; Q939.91 [免疫学];
学科分类号
100102 ;
摘要
Myasthenia Gravis (MG) is an autoimmune neuromuscular disorder where acetylcholine receptor (AChR) antibodies induce membrane attack complex formation at the muscle membrane. The C1-inhibitor (C1-INH) regulates the classical pathway and is a promising marker in other autoimmune disorders. Treatment options for AChR antibody MG include complement inhibitors; nevertheless, the early pathway activation in MG remains unclear. Serum and plasma C1s-C1-INH levels were higher in MG patients than in matched healthy controls, supporting early classical pathway activation in most MG patients. These findings allow prospective validation studies of activated C1s as a putative treatment target and potential accompanying biomarker in MG.
引用
收藏
页数:5
相关论文
共 50 条
  • [21] C1-INH REPLACEMENT THERAPY IN THE COMMON FORM OF HEREDITARY ANGIOEDEMA - FURTHER EVIDENCE OF A FUNCTIONALLY CRITICAL-LEVEL OF C1-INH CONCENTRATION
    SPATH, PJ
    WUTHRICH, B
    JOURNAL OF INVESTIGATIVE DERMATOLOGY, 1986, 87 (01) : 168 - 168
  • [22] Plasma-derived C1-INH for managing hereditary angioedema in pediatric patients: A systematic review
    Craig, Timothy J.
    Schneider, Lynda C.
    MacGinnitie, Andrew J.
    PEDIATRIC ALLERGY AND IMMUNOLOGY, 2015, 26 (06) : 537 - 544
  • [23] Role of vascular permeability factors in patients with normal C1-INH angioedema
    Ferrara, A. L.
    Suffritti, C.
    Bova, M.
    Petraroli, A.
    Firinu, D.
    Modestino, L.
    Scala, S.
    Del Giacco, S.
    Zanichelli, A.
    Loffredo, S.
    Cicardi, M.
    ALLERGY, 2019, 74 : 215 - 215
  • [24] Acquired angioedema due to C1-INH deficiency: a report of 24 patients
    Baeza, M. L.
    Caballero, T.
    Cabanas, R.
    Castro, M.
    Escudero, E.
    Gonzalez-Quevedo, T.
    Guilarte, M.
    Lleonart, R.
    Marcos, C.
    Nunez, R.
    Prior, N.
    Rivero, D.
    Saenz, de San Pedro B.
    Sala-Cunill, A.
    Varela, S.
    Prieto, A.
    ALLERGY, 2016, 71 : 85 - 85
  • [25] Glucocorticoid polymorphism in patients with hereditary angioedema due to C1-INH deficiency
    Zotter, Z.
    Nagy, Z.
    Csuka, D.
    Patocs, A.
    Farkas, H.
    ALLERGY, 2014, 69 : 484 - 484
  • [26] Adverse effects of danazol prophylaxis in female patients with hereditary angioedema due to C1-INH deficiency (HAE-C1-INH)
    Zotter, Z.
    Czaller, I
    Csuka, D.
    Szabo, E.
    Kohalmi, K., V
    Varga, L.
    Farkas, H.
    ALLERGY, 2013, 68 : 430 - 430
  • [27] The crux of C1-INH testing in everyday lab work
    Schoffl, Clemens
    Haas, Andrea
    Herrmann, Markus
    Aberer, Werner
    JOURNAL OF IMMUNOLOGICAL METHODS, 2021, 497
  • [28] Hereditary angioedema due to C1-INH: Case report
    Loloci, Qirko E.
    Gurakuqi, A.
    Pupo, L.
    Skenderi, A.
    Asllani, J.
    ALLERGY, 2019, 74 : 754 - 754
  • [29] High prevalence of epilepsy in HAE with normal C1-INH
    Kuwahara, Saki
    Fukunaga, Atsushi
    Ohata, Marie
    Nakatani, Shoko
    Yokoyama, Daisuke
    Nakauchi, Emi
    Yoshioka, Ai
    Kinoshita, Masako
    Nishigori, Chikako
    ALLERGOLOGY INTERNATIONAL, 2020, 69 (04) : 630 - 632
  • [30] C1-INH replacement: Twice a week may not be enough
    Sargur, Ravishankar
    Lunn, Rod
    Wild, Graeme
    Ashworth, Fran
    Egner, William
    CLINICAL AND EXPERIMENTAL ALLERGY, 2007, 37 (12): : 1874 - 1874