Treatment-responsive pandysautonomia in an adolescent with ganglionic α3-AChR antibodies.

Treatment-responsive pandysautonomia in an adolescent with ganglionic α3-AChR antibodies.

Autoimmune autonomic ganglionopathy (AAG) is a rare disorder that presents with pandysautonomia typically in middle age and elderly patients. AAG is typically associated with serum autoantibodies that bind to the alpha-3 subunit of the ganglionic acetylcholine receptor (α3-AChR Ab). We report a 13 y...

Podrobná bibliografie
Hlavní autoři:	Dale, R, Lang, B, Brilot, F, Polfrit, Y, Smith, G, Wong, M
Médium:	Journal article
Jazyk:	English
Vydáno:	2012

Podobné jednotky

AChR phosphorylation and indirect inhibition of AChR function in seronegative MG.
Autor: Plested, C, a další
Vydáno: (2002)

Effect of sera from AChR-antibody negative myasthenia gravis patients on AChR and MuSK in cell cultures.
Autor: Farrugia, M, a další
Vydáno: (2007)

Antibodies to AChRs in seronegative myasthenia gravis
Autor: Leite, M, a další
Vydáno: (2006)

Correlation between polymorphisms in the AChR gamma and delta subunit promoters and clinical phenotype in AChR deficiency patients
Autor: Bonifati, D, a další
Vydáno: (2004)

A Stable Cell Line Expressing Clustered AChR: A Novel Cell-Based Assay for Anti-AChR Antibody Detection in Myasthenia Gravis
Autor: Yu Cai, a další
Vydáno: (2021-07-01)

Congenital Myasthenic Syndrome and AChR Mutation
Autor: J Gordon Millichap
Vydáno: (2000-09-01)

Congenital Myasthenic Syndromes: End-Plate AChR Lack
Autor: J Gordon Millichap
Vydáno: (1998-02-01)

A cysteine residue close to the C-terminus of the E subunit is essential for surface acetylcholine receptor (AChR) expression: Implications for AChR deficiency syndrome
Autor: Ealing, J, a další
Vydáno: (2002)

Differential Cytokine Changes in Patients with Myasthenia Gravis with Antibodies against AChR and MuSK.
Autor: Vuslat Yilmaz, a další
Vydáno: (2015-01-01)

alpha-Actinin interacts with rapsyn in agrin-stimulated AChR clustering
Autor: Dobbins G Clement, a další
Vydáno: (2008-12-01)

PKC and PKA regulate AChR dynamics at the neuromuscular junction of living mice.
Autor: Isabel Martinez-Pena y Valenzuela, a další
Vydáno: (2013-01-01)

The Regulation Effect of α7nAChRs and M1AChRs on Inflammation and Immunity in Sepsis
Autor: Song Hu, a další
Vydáno: (2021-01-01)

Characterization of an anti-fetal AChR monoclonal antibody isolated from a myasthenia gravis patient
Autor: Saxena, A, a další
Vydáno: (2017)

Serological diagnosis of MG in Tianjin, China, with non-radioactive assays for AChR and MuSK antibodies
Autor: Yang, L, a další
Vydáno: (2009)

Oral Microbiota Profile in a Group of Anti-AChR Antibody–Positive Myasthenia Gravis Patients
Autor: Chao Huang, a další
Vydáno: (2022-07-01)

Selective silencing of pathogenic a subunit AChR mutant in dominant disorders
Autor: Abdelgany, A, a další
Vydáno: (2004)

Structural abnormalities of the AChR caused by mutations underlying congenital myasthenic syndromes.
Autor: Beeson, D, a další
Vydáno: (2003)

Diverse molecular mechanisms involved in AChR deficiency due to rapsyn mutations
Autor: Cossins, J, a další
Vydáno: (2006)

Congenital myasthenic syndrome patients due to AChR epsilon subunit mutations
Autor: Bonifati, D, a další
Vydáno: (2004)

Diverse molecular mechanisms involved in AChR deficiency due to rapsyn mutations.
Autor: Cossins, J, a další
Vydáno: (2006)

Impact of Key Nicotinic AChR Subunits on Post-Stroke Pneumococcal Pneumonia
Autor: Sandra Jagdmann, a další
Vydáno: (2020-05-01)

Clinical outcomes in AchR antibody-positive myasthenia gravis: Where does rituximab stand in the current times?
Autor: Megha Chetan Dhamne
Vydáno: (2023-01-01)

AChR deficiency due to epsilon-subunit mutations: two common mutations in the Netherlands.
Autor: Faber, C, a další
Vydáno: (2009)

Preferential expression of AChR epsilon-subunit in thymomas from patients with myasthenia gravis.
Autor: Maclennan, C, a další
Vydáno: (2008)

Nomogram for short-term outcome assessment in AChR subtype generalized myasthenia gravis
Autor: Rui Zhao, a další
Vydáno: (2021-06-01)

Consensus recommendation for the treatment of generalised Myasthenia Gravis with anti-acetylcholine receptor antibodies (gMG AChR+) in Spain
Autor: Elena Cortés-Vicente, a další
Vydáno: (2025-01-01)

Clinical Characteristics and Prognosis of Anti-AChR Positive Myasthenia Gravis Combined With Anti-LRP4 or Anti-Titin Antibody
Autor: Yuping Chen, a další
Vydáno: (2022-05-01)

Severe relapsing autoimmune encephalitis with GABAA receptor, titin and AchR antibodies in a patient with thymoma: a case report
Autor: Melissa Boisclair, a další
Vydáno: (2024-05-01)

Compromised fidelity of B-cell tolerance checkpoints in AChR and MuSK myasthenia gravis
Autor: Lee, J, a další
Vydáno: (2016)

Myasthenia gravis in a woman with congenital AChR deficiency due to epsilon-subunit mutations.
Autor: Croxen, R, a další
Vydáno: (2002)

A mouse model of AChR deficiency syndrome with a phenotype reflecting the human condition.
Autor: Cossins, J, a další
Vydáno: (2004)

The clinical outcome in AChR-positive generalized myasthenia gravis: A retrospective observational study
Autor: Pawan T Ojha, a další
Vydáno: (2023-01-01)

Direct proof of the in vivo pathogenic role of the AChR autoantibodies from myasthenia gravis patients.
Autor: Gregory Kordas, a další
Vydáno: (2014-01-01)

Clinical comparison of anti-MuSK- vs anti-AChR-positive and seronegative myasthenia gravis.
Autor: Deymeer, F, a další
Vydáno: (2007)

Nicotine promotes proliferation of human nasopharyngeal carcinoma cells by regulating α7AChR, ERK, HIF-1α and VEGF/PEDF signaling.
Autor: Dingbo Shi, a další
Vydáno: (2012-01-01)

Discovery of functionally distinct anti-C7 monoclonal antibodies and stratification of anti-nicotinic AChR positive Myasthenia Gravis patients
Autor: Eleonora Lekova, a další
Vydáno: (2022-09-01)

Juvenile Generalized Myasthenia Gravis With AChR and MuSK Antibody Double Positivity: A Case Report With a Review of the Literature
Autor: XiuShan Ge, a další
Vydáno: (2022-05-01)

FoxP3− Tr1 Cell in Generalized Myasthenia Gravis and Its Relationship With the Anti-AChR Antibody and Immunomodulatory Cytokines
Autor: Huanyu Meng, a další
Vydáno: (2021-12-01)

Mutation in the AChR ion channel gate underlies a fast channel congenital myasthenic syndrome.
Autor: Webster, R, a další
Vydáno: (2004)

Performance of Serum m3AchR-Ab for Alpha-fetoprotein-negative Hepatocellular Carcinoma Diagnosis
Autor: Tuoheti YAKUFU·, Kainan ZHANG, Hui ZHAO, Dawuti WUBULITALIFU·, Rui ZHANG, Renyong LIN, Guodong LYU
Vydáno: (2022-06-01)