Amyloid-Journal of Protein Folding Disorders最新文献_第5页

Significance of aberrant nerve conduction in hereditary transthyretin amyloidosis. 遗传性甲状腺转蛋白淀粉样变神经传导异常的意义。

IF 5.1 2区医学

Amyloid-Journal of Protein Folding Disorders Pub Date : 2026-06-01 Epub Date: 2026-01-12 DOI: 10.1080/13506129.2026.2613126

Toshihiro Ide, Masaaki Yoshikawa, Kohei Suzuyama, Satoru Yagi, Soma Furukawa, Yuki Fukami, Masahisa Katsuno, Haruki Koike

{"title":"Significance of aberrant nerve conduction in hereditary transthyretin amyloidosis.","authors":"Toshihiro Ide, Masaaki Yoshikawa, Kohei Suzuyama, Satoru Yagi, Soma Furukawa, Yuki Fukami, Masahisa Katsuno, Haruki Koike","doi":"10.1080/13506129.2026.2613126","DOIUrl":"10.1080/13506129.2026.2613126","url":null,"abstract":"Background: In hereditary transthyretin (ATTRv) amyloidosis, axonal neuropathy is the main pathologic basis of polyneuropathy. However, whether nerve conduction slowing resembling chronic inflammatory demyelinating polyneuropathy (CIDP), observed in some patients, reflects primary demyelination or secondary changes related to axonal damage remains unclear.Methods: Electrophysiologic findings were compared between 96 patients with V30M ATTRv amyloidosis from nonendemic areas and 175 patients with nutritional or alcoholic axonal neuropathies. Myelinated fiber density was assessed in sural nerve biopsy specimens from 66 and 133 patients, respectively.Results: Motor nerve conduction velocity (MCV) was reduced and distal motor latency (DML) was prolonged in both groups compared with normal controls, with more marked changes in ATTRv amyloidosis. Compound muscle action potential (CMAP) amplitude and myelinated fiber density were significantly lower in ATTRv amyloidosis than in nutritional/alcoholic neuropathies (p < 0.001). Conduction slowing fulfilling EAN/PNS CIDP criteria was observed only in patients with severe axonal loss. After adjusting for age, disease duration, and CMAP amplitude, only MCV slowing and DML prolongation in median nerve remained more pronounced in ATTRv amyloidosis.Conclusions: CIDP-like conduction slowing in ATTRv amyloidosis largely represents secondary changes caused by axonal degeneration, although additional mechanisms may contribute to slowing in the median nerve.","PeriodicalId":50964,"journal":{"name":"Amyloid-Journal of Protein Folding Disorders","volume":" ","pages":"196-204"},"PeriodicalIF":5.1,"publicationDate":"2026-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145953734","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

The CATCH-ATTR study: CArpal Tunnel ligament amyloid-the Clue for early identification of Hereditary or wildtype systemic ATTR amyloidosis? CATCH-ATTR研究：腕管韧带淀粉样蛋白——早期识别遗传性或野生型系统性ATTR淀粉样变的线索？

IF 5.1 2区医学

Amyloid-Journal of Protein Folding Disorders Pub Date : 2026-06-01 Epub Date: 2026-01-07 DOI: 10.1080/13506129.2025.2611255

Fabian Aus dem Siepen, Frank Staub, Florian Andre, Ute Hegenbart, Stefan O Schönland, Norbert Frey, Christoph Röcken, Arnt V Kristen

{"title":"The CATCH-ATTR study: CArpal Tunnel ligament amyloid-the Clue for early identification of Hereditary or wildtype systemic ATTR amyloidosis?","authors":"Fabian Aus dem Siepen, Frank Staub, Florian Andre, Ute Hegenbart, Stefan O Schönland, Norbert Frey, Christoph Röcken, Arnt V Kristen","doi":"10.1080/13506129.2025.2611255","DOIUrl":"10.1080/13506129.2025.2611255","url":null,"abstract":"Aims: We sought to investigate the prevalence of Transthyretin amyloid (ATTR) deposits in the carpal ligament of patients undergoing surgery for carpal tunnel syndrome (CTS) and the frequency of concomitant ATTR-cardiomyopathy (CM) in a prospective study with long-term follow-up.Methods and results: Our prospective exploratory study enrolled 551 patients treated for CTS. All resection specimens of carpal ligament underwent histopathological evaluation. Patients with confirmed amyloid deposits were referred to cardiac evaluation, including cardiac MRI (CMR) scans, as well as follow-up examinations. Amyloid deposits were found in 52 (9%) specimens (1 light chain amyloid, 51 ATTR). Among patients with positive biopsy ATTR-CM was diagnosed in 4 (8%) patients, 1 patient was diagnosed at initial evaluation, and 3 patients were diagnosed during the follow-up. All patients had ATTRwt at an early stage of disease. Extracellular volume (ECV) measured by CMR was significantly higher in amyloid-positive CTS patients compared to healthy controls (30 ± 2 vs. 24 ± 1%, p < .01).Conclusion: Histopathological evaluation of resected carpal ligament should be considered in patients at least older than 70 years. Even though absolute numbers were low, cardiac screening of patients with evidence of amyloid deposits in in carpal tunnel tissues might facilitate early diagnosis of ATTR-CM in some patients.","PeriodicalId":50964,"journal":{"name":"Amyloid-Journal of Protein Folding Disorders","volume":" ","pages":"180-188"},"PeriodicalIF":5.1,"publicationDate":"2026-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145935997","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Systemic fibrinogen Aα-chain amyloidosis in Manchester Terrier dogs. 曼彻斯特梗犬的系统性纤维蛋白原a α链淀粉样变性。

IF 5.1 2区医学

Amyloid-Journal of Protein Folding Disorders Pub Date : 2026-06-01 Epub Date: 2026-03-04 DOI: 10.1080/13506129.2026.2637775

Natsumi Kobayashi, Keiichi Kuroki, Yoshiyuki Itoh, Tomoaki Murakami

引用次数: 0

Lysozyme encounters transthyretin: rethinking amyloid complexity in ligamentum flavum. 溶菌酶遇到转甲状腺素：重新思考黄韧带淀粉样蛋白的复杂性。

IF 5.1 2区医学

Amyloid-Journal of Protein Folding Disorders Pub Date : 2026-06-01 Epub Date: 2026-03-08 DOI: 10.1080/13506129.2026.2640976

Mitsuharu Ueda

引用次数: 0

Assessing hemorrhagic risk in transthyretin cardiac amyloidosis. The haemorrhagic-embolic conundrum. 评估转甲状腺素型心脏淀粉样变性的出血风险。出血性栓塞难题。

IF 5.1 2区医学

Amyloid-Journal of Protein Folding Disorders Pub Date : 2026-06-01 Epub Date: 2026-06-08 DOI: 10.1080/13506129.2026.2683425

Silvia Vilches

引用次数: 0

Haemorrhagic events in patients with transthyretin cardiac amyloidosis. 转甲状腺素型心脏淀粉样变性患者的出血事件。

IF 5.1 2区医学

Amyloid-Journal of Protein Folding Disorders Pub Date : 2026-06-01 Epub Date: 2026-01-26 DOI: 10.1080/13506129.2025.2612282

Lucía Moreno De Redrojo Cortés, Rocío Eiros Bachiller, Eduardo Villacorta Argüelles, José Cañadas Salazar, Pablo Pérez Sánchez, Luis Miguel Rincón, Mónica García Monsalvo, Armando Oterino Manzanas, Elena Alejo Alonso, Daniel Bracho Bracchitta, María Jesús Andrés Salinas, María de la Soledad García García, Ignacio Cruz González, Pedro Luis Sánchez Fernández, María Gallego Delgado

{"title":"Haemorrhagic events in patients with transthyretin cardiac amyloidosis.","authors":"Lucía Moreno De Redrojo Cortés, Rocío Eiros Bachiller, Eduardo Villacorta Argüelles, José Cañadas Salazar, Pablo Pérez Sánchez, Luis Miguel Rincón, Mónica García Monsalvo, Armando Oterino Manzanas, Elena Alejo Alonso, Daniel Bracho Bracchitta, María Jesús Andrés Salinas, María de la Soledad García García, Ignacio Cruz González, Pedro Luis Sánchez Fernández, María Gallego Delgado","doi":"10.1080/13506129.2025.2612282","DOIUrl":"10.1080/13506129.2025.2612282","url":null,"abstract":"Background: transthyretin cardiac amyloidosis (ATTR-CA) is an infiltrative disease associated with several comorbidities such as Bleeding. The aim of this study was to evaluate the incidence of bleeding in patients with ATTR-CA and the underlying risk factors that promote bleeding.Methods: retrospective observational study of a cohort of patients with ATTR-CA diagnosed from 1st April 2016 to 31st December 2023. Bleeding risk was calculated according to HAS-BLED score and bleeding events at follow-up were analysed.Results: 184 patients were diagnosed with a median age of 84 years [IQR 79-88], 154 (84%) males. 118 patients (64%) were on anticoagulant therapy (AC). 91 patients (50%) had a bleeding event, with a total of 189 events recorded and a median time to bleeding of 23 months [IQR 10-68]. 26 patients (29%) had severe haemorrhages and 3 patients died of haemorrhagic causes. The incidence rate was 50 bleeds per 100 patient-years and 57 per 100 patient-years in AC patients. AC resulted in a 2.5-fold raise in bleeding (95% CI 1.4-4.8; p = .003) and a score ≥2 on HAS-BLED scale in a 5.4-fold increase (95%CI 2.6-11; p < .001).Conclusion: bleeding is a frequent complication in ATTR-CA, with a higher risk in those individuals with a HAS-BLED score ≥2 and/or on AC. These findings could be considered in risk stratification and therapeutic decision-making.","PeriodicalId":50964,"journal":{"name":"Amyloid-Journal of Protein Folding Disorders","volume":" ","pages":"135-145"},"PeriodicalIF":5.1,"publicationDate":"2026-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146054848","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

A clinical and ECG based score to predict incident atrial fibrillation in cardiac amyloidosis: the Amy-Lyon AF score. 预测心脏淀粉样变性患者心房颤动的临床和心电图评分：ami - lyon心房颤动评分。

IF 5.1 2区医学

Amyloid-Journal of Protein Folding Disorders Pub Date : 2026-06-01 Epub Date: 2026-01-18 DOI: 10.1080/13506129.2025.2612567

Tiphaine Bollon, Antoine Jobbe Duval, Simon Leboube, Nathan Mewton, Laurent Sebbag, Kevin Gardey, Eric Bonnefoy-Cudraz, Paul Charles, Mathieu Montoy, Michaël Serraille, Brahim Harbaoui, Pierre Lantelme, Pierre-Yves Courand

{"title":"A clinical and ECG based score to predict incident atrial fibrillation in cardiac amyloidosis: the Amy-Lyon AF score.","authors":"Tiphaine Bollon, Antoine Jobbe Duval, Simon Leboube, Nathan Mewton, Laurent Sebbag, Kevin Gardey, Eric Bonnefoy-Cudraz, Paul Charles, Mathieu Montoy, Michaël Serraille, Brahim Harbaoui, Pierre Lantelme, Pierre-Yves Courand","doi":"10.1080/13506129.2025.2612567","DOIUrl":"10.1080/13506129.2025.2612567","url":null,"abstract":"Background: Atrial fibrillation (AF) is common and poorly tolerated in cardiac amyloidosis (CA) patients. No current tool assesses AF risk in this population.Methods: We enrolled patients with light chain (AL) or transthyretin (ATTR) CA, with no prior history of AF at diagnosis between January 2015 and September 2023 across three university hospitals. Clinical, biochemical and electrocardiographic parameters were retrospectively collected and their predictive value for AF was assessed.Results: A total of 169 patients were included (56.2% wild-type (wt) ATTR, 35.5% AL and 8.3% variant (v) ATTR). Over a median follow-up of 21 months, 55 patients (33%) presented a first episode of AF. Five independent predictors for AF were identified using multivariate logistic regression model: obstructive sleep apnea, hypertension, CA subtype, P wave duration ≥120 ms, 1st degree atrioventricular (AV) block. Based on these variables, the Amy-Lyon AF score (range 0-46) was developed to estimate AF risk (area under the receiver operating characteristic (ROC) curve (AUC) 0.768 [0.685-0.852], p < .001). At two years of follow-up, the incidence of AF reached 82.7% in patients with a score >28. In 80 patients, the AUC of the Amy-Lyon AF score was higher than that of left atrial strain.Conclusions: Five simple and readily available predictors may stratify the risk of AF in CA.","PeriodicalId":50964,"journal":{"name":"Amyloid-Journal of Protein Folding Disorders","volume":" ","pages":"205-215"},"PeriodicalIF":5.1,"publicationDate":"2026-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145994679","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Untwisted amyloid fibrils from a transgenic mouse model of AL amyloidosis. 来自AL淀粉样变性转基因小鼠模型的未扭曲淀粉样原纤维。

IF 5.1 2区医学

Amyloid-Journal of Protein Folding Disorders Pub Date : 2026-06-01 Epub Date: 2025-12-21 DOI: 10.1080/13506129.2025.2605332

Luca Broggini, Diane Marie Valérie Bonnet, Karolina W Swiderska, Sebastien Bender, Gilles R Codo, Alessio Lampis, Gemma Martinez-Rivas, Pauline Duchatelet, Aurore Danigo, Antonio Chaves-Sanjuan, Christophe Sirac, Stefano Ricagno

引用次数: 0

Clarifying the immunoglobulin light chain variable gene usage in Chinese patients with renal AL amyloidosis. 澄清中国肾AL淀粉样变患者免疫球蛋白轻链可变基因的使用。

IF 5.1 2区医学

Amyloid-Journal of Protein Folding Disorders Pub Date : 2026-06-01 Epub Date: 2026-01-27 DOI: 10.1080/13506129.2026.2619419

Shuang Wang, Dan-Yang Li, Dan Liu, Xu Zhang, Yue-Yue Zhu, Hui Wang, Xiao-Juan Yu, Fu-de Zhou, Gang Liu, Li Yang, Ming-Hui Zhao, Su-Xia Wang

{"title":"Clarifying the immunoglobulin light chain variable gene usage in Chinese patients with renal AL amyloidosis.","authors":"Shuang Wang, Dan-Yang Li, Dan Liu, Xu Zhang, Yue-Yue Zhu, Hui Wang, Xiao-Juan Yu, Fu-de Zhou, Gang Liu, Li Yang, Ming-Hui Zhao, Su-Xia Wang","doi":"10.1080/13506129.2026.2619419","DOIUrl":"10.1080/13506129.2026.2619419","url":null,"abstract":"Background: The association between immunoglobulin light chain variable (IGVL) gene usage and clinicopathological features in renal AL amyloidosis requires further research. This study analyzed IGVL genes and their associations with clinicopathological characteristics, organ involvement and survival outcomes in a Chinese cohort.Methods: We retrospectively enrolled 273 renal AL amyloidosis patients. Amyloid deposits were microdissected and subjected to mass spectrometry (MS)-based proteomics to identify IGVL genes. Clinicopathological features, organ involvement and survival data were systematically analyzed.Results: IGVL genes were successfully identified in 250 patients (92%). IGLV6-57 (32%) was predominant, IGKV1 family (12%) was the most prevalent in AL-κ cases. IGLV6-57 was associated with a higher rate of full nephrotic syndrome, while was linked to milder vascular amyloid deposition and total amyloid deposition. IGKV1 was associated with heavier amyloid burden, severe interstitial inflammation and a higher rate of hepatic involvement, IGLV6-57 was correlated with reduced hepatic involvement. IGLV1-51 potentially predicted rapid renal progression and dialysis risk.Conclusion: The IGVL gene usage is associated with distinct clinicopathological features in renal AL amyloidosis, IGLV6-57 is linked to a higher frequency of full nephrotic syndrome, IGKV1 is associated with severe kidney structural damage and hepatic tropism, and IGLV1-51 potentially predicts poor renal survival.","PeriodicalId":50964,"journal":{"name":"Amyloid-Journal of Protein Folding Disorders","volume":" ","pages":"168-179"},"PeriodicalIF":5.1,"publicationDate":"2026-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146054779","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

In memoriam: Shukuro Araki, MD, PhD (1927-2025) pioneer of Japanese neurology and a global leader in familial amyloid polyneuropathy. 纪念：荒木修郎，医学博士，博士（1927-2025）日本神经病学的先驱，家族性淀粉样蛋白多发性神经病的全球领导者。

IF 5.1 2区医学

Amyloid-Journal of Protein Folding Disorders Pub Date : 2026-06-01 Epub Date: 2026-02-16 DOI: 10.1080/13506129.2026.2622971

Mitsuharu Ueda, Yohei Misumi, Yukio Ando

引用次数: 0