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

Changes in the amyloid editorial board members and in editor positions. 淀粉样蛋白编辑委员会成员和编辑职位的变化。

IF 5.5 2区医学

Amyloid-Journal of Protein Folding Disorders Pub Date : 2024-06-01 Epub Date: 2024-05-23 DOI: 10.1080/13506129.2024.2344167

Stefan Schönland, Per Westermark

引用次数: 0

Circulating transthyretin and retinol binding protein 4 levels among middle-age V122I TTR carriers in the general population. 普通人群中 V122I TTR 中年携带者的循环转甲状腺素和视黄醇结合蛋白 4 水平。

IF 5.5 2区医学

Amyloid-Journal of Protein Folding Disorders Pub Date : 2024-06-01 Epub Date: 2024-03-06 DOI: 10.1080/13506129.2024.2322479

Nicholas S Hendren, James A De Lemos, Jarett D Berry, Julia Kozlitina, Lorena Saelices, Alan X Ji, Zhili Shao, Chia-Feng Liu, Sonia Garg, Maryjane A Farr, Mark H Drazner, W H Wilson Tang, Justin L Grodin

{"title":"Circulating transthyretin and retinol binding protein 4 levels among middle-age V122I TTR carriers in the general population.","authors":"Nicholas S Hendren, James A De Lemos, Jarett D Berry, Julia Kozlitina, Lorena Saelices, Alan X Ji, Zhili Shao, Chia-Feng Liu, Sonia Garg, Maryjane A Farr, Mark H Drazner, W H Wilson Tang, Justin L Grodin","doi":"10.1080/13506129.2024.2322479","DOIUrl":"10.1080/13506129.2024.2322479","url":null,"abstract":"Background: Hereditary transthyretin cardiac amyloidosis (ATTRv-CA) has a long latency phase before clinical onset, creating a need to identify subclinical disease. We hypothesized circulating transthyretin (TTR) and retinol binding protein 4 (RBP4) levels would be associated with TTR carrier status and correlated with possible evidence of subclinical ATTRv-CA.Methods: TTR and RBP4 were measured in blood samples from V122I TTR carriers and age-, sex- and race-matched non-carrier controls (1:2 matching) among Dallas Heart Study participants (phases 1 (DHS-1) and 2 (DHS-2)). Multivariable linear regression models determined factors associated with TTR and RBP4.Results: There were 40 V122I TTR carriers in DHS-1 and 54 V122I TTR carriers in DHS-2. In DHS-1 and DHS-2, TTR was lower in V122I TTR carriers (p < .001 for both), and RBP4 in DHS-2 was lower in V122I TTR carriers than non-carriers (p = .002). Among V122I TTR carriers, TTR was negatively correlated with markers of kidney function, and limb lead voltage (p < .05 for both) and TTR and RBP4 were correlated with atrial volume in DHS-2 (p < .05).Conclusions: V122I TTR carrier status is independently associated with lower TTR and RBP4 in comparison with non-carriers. These findings support the hypothesis that TTR and RBP4 may correlate with evidence of subclinical ATTRv-CA.","PeriodicalId":50964,"journal":{"name":"Amyloid-Journal of Protein Folding Disorders","volume":" ","pages":"124-131"},"PeriodicalIF":5.5,"publicationDate":"2024-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11127723/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140040849","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Longitudinal analysis of serum neurofilament light chain levels as marker for neuronal damage in hereditary transthyretin amyloidosis. 将血清神经丝蛋白轻链水平作为遗传性转甲状腺素淀粉样变性病神经元损伤标志物的纵向分析。

IF 5.5 2区医学

Amyloid-Journal of Protein Folding Disorders Pub Date : 2024-06-01 Epub Date: 2024-03-13 DOI: 10.1080/13506129.2024.2327342

Milou Berends, Anne F Brunger, Johan Bijzet, Bart-Jan Kroesen, Gea Drost, Fiete Lange, Charlotte E Teunissen, Sjors In 't Veld, Alexander Fje Vrancken, Reinold O B Gans, Bouke P C Hazenberg, Paul A van der Zwaag, Hans L A Nienhuis

{"title":"Longitudinal analysis of serum neurofilament light chain levels as marker for neuronal damage in hereditary transthyretin amyloidosis.","authors":"Milou Berends, Anne F Brunger, Johan Bijzet, Bart-Jan Kroesen, Gea Drost, Fiete Lange, Charlotte E Teunissen, Sjors In 't Veld, Alexander Fje Vrancken, Reinold O B Gans, Bouke P C Hazenberg, Paul A van der Zwaag, Hans L A Nienhuis","doi":"10.1080/13506129.2024.2327342","DOIUrl":"10.1080/13506129.2024.2327342","url":null,"abstract":"Objective: To evaluate serum neurofilament light chain (sNfL) as biomarker of disease onset, progression and treatment effect in hereditary transthyretin (ATTRv) amyloidosis patients and TTR variant (TTRv) carriers.Methods: sNfL levels were assessed longitudinally in persistently asymptomatic TTRv carriers (N = 12), persistently asymptomatic ATTRv amyloidosis patients (defined as asymptomatic patients but with amyloid detectable in subcutaneous abdominal fat tissue) (N = 8), in TTRv carriers who developed polyneuropathy (N = 7) and in ATTRv amyloidosis patients with polyneuropathy on treatment (TTR-stabiliser (N = 20) or TTR-silencer (N = 18)). Polyneuropathy was confirmed by nerve conduction studies or quantitative sensory testing. sNfL was analysed using a single-molecule array assay.Results: sNfL increased over 2 years in persistently asymptomatic ATTRv amyloidosis patients, but did not change in persistently asymptomatic TTRv carriers. In all TTRv carriers who developed polyneuropathy, sNfL increased from 8.4 to 49.8 pg/mL before the onset of symptoms and before polyneuropathy could be confirmed neurophysiologically. In symptomatic ATTRv amyloidosis patients on a TTR-stabiliser, sNfL remained stable over 2 years. In patients on a TTR-silencer, sNfL decreased after 1 year of treatment.Conclusion: sNfL is a biomarker of early neuronal damage in ATTRv amyloidosis already before the onset of polyneuropathy. Current data support the use of sNfL in screening asymptomatic TTRv carriers and in monitoring of disease progression and treatment effect.","PeriodicalId":50964,"journal":{"name":"Amyloid-Journal of Protein Folding Disorders","volume":" ","pages":"132-141"},"PeriodicalIF":5.5,"publicationDate":"2024-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140112069","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

Mass spectrometry-based proteomic analysis of proteins adsorbed by hexadecyl-immobilized cellulose bead column for the treatment of dialysis-related amyloidosis. 基于质谱的蛋白质组学分析：十六烷基固定化纤维素珠柱吸附的蛋白质用于治疗透析相关淀粉样变性病。

IF 5.5 2区医学

Amyloid-Journal of Protein Folding Disorders Pub Date : 2024-06-01 Epub Date: 2024-02-11 DOI: 10.1080/13506129.2024.2315148

Suguru Yamamoto, Keiko Yamamoto, Yoshitoshi Hirao, Keiichi Yamaguchi, Kichitaro Nakajima, Mami Sato, Miho Kawachi, Mio Domon, Kei Goto, Kentaro Omori, Noriaki Iino, Hisaki Shimada, Ryuzi Aoyagi, Isei Ei, Shin Goto, Yuji Goto, Fumitake Gejyo, Tadashi Yamamoto, Ichiei Narita

{"title":"Mass spectrometry-based proteomic analysis of proteins adsorbed by hexadecyl-immobilized cellulose bead column for the treatment of dialysis-related amyloidosis.","authors":"Suguru Yamamoto, Keiko Yamamoto, Yoshitoshi Hirao, Keiichi Yamaguchi, Kichitaro Nakajima, Mami Sato, Miho Kawachi, Mio Domon, Kei Goto, Kentaro Omori, Noriaki Iino, Hisaki Shimada, Ryuzi Aoyagi, Isei Ei, Shin Goto, Yuji Goto, Fumitake Gejyo, Tadashi Yamamoto, Ichiei Narita","doi":"10.1080/13506129.2024.2315148","DOIUrl":"10.1080/13506129.2024.2315148","url":null,"abstract":"Background: Dialysis-related amyloidosis (DRA) is a severe complication in end-stage kidney disease (ESKD) patients undergoing long-term dialysis treatment, characterized by the deposition of β2-microglobulin-related amyloids (Aβ2M amyloid). To inhibit DRA progression, hexadecyl-immobilized cellulose bead (HICB) columns are employed to adsorb circulating β2-microglobulin (β2M). However, it is possible that the HICB also adsorbs other molecules involved in amyloidogenesis.Methods: We enrolled 14 ESKD patients using HICB columns for DRA treatment; proteins were extracted from HICBs following treatment and identified using liquid chromatography-linked mass spectrometry. We measured the removal rate of these proteins and examined the effect of those molecules on Aβ2M amyloid fibril formation in vitro.Results: We identified 200 proteins adsorbed by HICBs. Of these, 21 were also detected in the amyloid deposits in the carpal tunnels of patients with DRA. After passing through the HICB column and hemodialyzer, the serum levels of proteins such as β2M, lysozyme, angiogenin, complement factor D and matrix Gla protein were reduced. These proteins acted in the Aβ2M amyloid fibril formation.Conclusions: HICBs adsorbed diverse proteins in ESKD patients with DRA, including those detected in amyloid lesions. Direct hemoperfusion utilizing HICBs may play a role in acting Aβ2M amyloidogenesis by reducing the amyloid-related proteins.","PeriodicalId":50964,"journal":{"name":"Amyloid-Journal of Protein Folding Disorders","volume":" ","pages":"105-115"},"PeriodicalIF":5.5,"publicationDate":"2024-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139724820","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

Serum neurofilament light chain in hereditary transthyretin amyloidosis: validation in real-life practice. 遗传性转甲状腺素淀粉样变性中的血清神经丝蛋白轻链：在实际生活中的验证。

IF 5.5 2区医学

Amyloid-Journal of Protein Folding Disorders Pub Date : 2024-06-01 Epub Date: 2024-02-13 DOI: 10.1080/13506129.2024.2313218

Antonia S Carroll, Yousuf Razvi, Luke O'Donnell, Elena Veleva, Amanda Heslegrave, Henrik Zetterberg, Steve Vucic, Matthew C Kiernan, Alexander M Rossor, Julian D Gillmore, Mary M Reilly

{"title":"Serum neurofilament light chain in hereditary transthyretin amyloidosis: validation in real-life practice.","authors":"Antonia S Carroll, Yousuf Razvi, Luke O'Donnell, Elena Veleva, Amanda Heslegrave, Henrik Zetterberg, Steve Vucic, Matthew C Kiernan, Alexander M Rossor, Julian D Gillmore, Mary M Reilly","doi":"10.1080/13506129.2024.2313218","DOIUrl":"10.1080/13506129.2024.2313218","url":null,"abstract":"Background: Neurofilament light chain (NfL) has emerged as a sensitive biomarker in hereditary transthyretin amyloid polyneuropathy (ATTRv-PN). We hypothesise that NfL can identify conversion of gene carriers to symptomatic disease, and guide treatment approaches.Methods: Serum NfL concentration was measured longitudinally (2015-2022) in 59 presymptomatic and symptomatic ATTR variant carriers. Correlations between NfL and demographics, biochemistry and staging scores were performed as well as longitudinal changes pre- and post-treatment, and in asymptomatic and symptomatic cohorts. Receiver-operating analyses were performed to determine cut-off values.Results: NfL levels correlated with examination scores (CMTNS, NIS and MRC; all p < .01) and increased with disease severity (PND and FAP; all p < .05). NfL was higher in symptomatic and sensorimotor converters, than asymptomatic or sensory converters irrespective of time (all p < .001). Symptomatic or sensorimotor converters were discriminated from asymptomatic patients by NfL concentrations >64.5 pg/ml (sensitivity= 91.9%, specificity = 88.5%), whereas asymptomatic patients could only be discriminated from sensory or sensorimotor converters or symptomatic individuals by a NfL concentration >88.9 pg/ml (sensitivity = 62.9%, specificity = 96.2%) However, an NfL increment of 17% over 6 months could discriminate asymptomatic from sensory or sensorimotor converters (sensitivity = 88.9%, specificity = 80.0%). NfL reduced with treatment by 36%/year and correlated with TTR suppression (r = 0.64, p = .008).Conclusions: This data validates the use of serum NfL to identify conversion to symptomatic disease in ATTRv-PN. NfL levels can guide assessment of disease progression and response to therapies.","PeriodicalId":50964,"journal":{"name":"Amyloid-Journal of Protein Folding Disorders","volume":" ","pages":"95-104"},"PeriodicalIF":5.5,"publicationDate":"2024-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139724821","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

Identification of calcitonin receptor-stimulating peptide 1-derived amyloid in a feline C-cell carcinoma. 猫c细胞癌中降钙素受体刺激肽1衍生淀粉样蛋白的鉴定。

IF 5.2 2区医学

Amyloid-Journal of Protein Folding Disorders Pub Date : 2024-06-01 Epub Date: 2023-11-19 DOI: 10.1080/13506129.2023.2282361

Tomoaki Murakami, Natsumi Kobayashi, Susumu Iwaide, Yoshiyuki Itoh, Miki Hisada, Takeshi Izawa, Mitsuru Kuwamura

引用次数: 0

Longitudinal PET/CT imaging with iodine (¹²⁴I) evuzamitide reveals organ response to plasma cell immunotherapy in a patient with AL amyloidosis. 碘(124I) evuzamitide纵向PET/CT成像显示AL淀粉样变性患者对浆细胞免疫治疗的器官反应。

IF 5.2 2区医学

Amyloid-Journal of Protein Folding Disorders Pub Date : 2024-06-01 Epub Date: 2023-11-22 DOI: 10.1080/13506129.2023.2286427

Ronald Lands, Emily B Martin, Dustin Powell, Alan Stuckey, Bryan Whittle, Spencer Guthrie, Renju Raj, Stephen J Kennel, Jonathan S Wall

引用次数: 0

Gait abnormalities in older adults with transthyretin cardiac amyloidosis. 患有转甲状腺素心脏淀粉样变性病的老年人步态异常。

IF 5.5 2区医学

Amyloid-Journal of Protein Folding Disorders Pub Date : 2024-06-01 Epub Date: 2024-03-03 DOI: 10.1080/13506129.2024.2319133

Fitsum E Petros, Alfonsina Mirabal Santos, Adedeji Adeniyi, Sergio Teruya, Jeffeny De Los Santos, Mathew S Maurer, Sunil K Agrawal

{"title":"Gait abnormalities in older adults with transthyretin cardiac amyloidosis.","authors":"Fitsum E Petros, Alfonsina Mirabal Santos, Adedeji Adeniyi, Sergio Teruya, Jeffeny De Los Santos, Mathew S Maurer, Sunil K Agrawal","doi":"10.1080/13506129.2024.2319133","DOIUrl":"10.1080/13506129.2024.2319133","url":null,"abstract":"Background: Transthyretin cardiac amyloidosis (ATTR cardiac amyloidosis) is caused by variant (ATTRv) or wild type (ATTRwt) transthyretin. While gait abnormalities have been studied in younger patients with ATTRv amyloidosis, research on gait in older adults with ATTR cardiac amyloidosis is lacking. Given ATTR cardiac amyloidosis' association with neuropathy and orthopedic manifestations, we explore the gait in this population.Methods: Twenty-eight older male ATTR cardiac amyloidosis patients and 11 healthy older male controls walked overground with and without a dual cognitive task. Gait parameters: stride width, length, velocity and stance time percentage were measured using an instrumented mat. ATTR amyloidosis patients were further categorized based on clinical and functional assessments.Results: We found significant gait differences between ATTR cardiac amyloidosis patients and healthy controls; patients had more variable, slower, narrower and shorter strides, with their feet spending more time in contact with the ground as opposed to in swing. However, the observed gait differences did not correlate with clinical and functional measures of ATTR cardiac amyloidosis severity.Conclusions: Our results suggest that gait analysis could be a complementary tool for characterizing ATTR cardiac amyloidosis patients and may inform clinical care as it relates to falls, management of anticoagulation, and functional independence.","PeriodicalId":50964,"journal":{"name":"Amyloid-Journal of Protein Folding Disorders","volume":" ","pages":"116-123"},"PeriodicalIF":5.5,"publicationDate":"2024-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11116048/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140023213","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Sequence diversity of kappa light chains from patients with AL amyloidosis and multiple myeloma. AL 淀粉样变性和多发性骨髓瘤患者卡帕轻链的序列多样性。

IF 5.5 2区医学

Amyloid-Journal of Protein Folding Disorders Pub Date : 2024-06-01 Epub Date: 2024-01-11 DOI: 10.1080/13506129.2023.2295221

Sarah Schreiner, Natalie Berghaus, Alexandra M Poos, Marc S Raab, Britta Besemer, Roland Fenk, Hartmut Goldschmidt, Elias K Mai, Carsten Müller-Tidow, Niels Weinhold, Ute Hegenbart, Stefanie Huhn, Stefan O Schönland

{"title":"Sequence diversity of kappa light chains from patients with AL amyloidosis and multiple myeloma.","authors":"Sarah Schreiner, Natalie Berghaus, Alexandra M Poos, Marc S Raab, Britta Besemer, Roland Fenk, Hartmut Goldschmidt, Elias K Mai, Carsten Müller-Tidow, Niels Weinhold, Ute Hegenbart, Stefanie Huhn, Stefan O Schönland","doi":"10.1080/13506129.2023.2295221","DOIUrl":"10.1080/13506129.2023.2295221","url":null,"abstract":"Background: AL amyloidosis (AL) results from the misfolding of immunoglobulin light chains (IG LCs). Aim of this study was to comprehensively analyse kappa LC sequences from AL patients in comparison with multiple myeloma (MM).Objective: We analysed IGKV/IGKJ usage and associated organ tropism and IGKV1/D-33 in terms of mutational analysis and theoretical biochemical properties.Material and methods: cDNA and bulk RNA sequencing of the LCs of AL and MM patients.Results: We studied 41 AL and 83 MM patients showing that IGKV1 was most expressed among kappa AL and MM, with higher frequency in AL (80% vs. 53%, p = .002). IGKV3 was underrepresented in AL (10% vs. 30%, p = .014). IGKJ2 was more commonly used in AL than in MM (39% vs. 29%). Patients with IGKV1/D-33 were associated with heart involvement (75%, p = .024). IGKV1/D-33-segments of AL had a higher mutation count (AL = 12.0 vs. MM = 10.0). FR3 and CDR3 were most frequently mutated in both, with a median mutation count in FR3 being the highest (AL = 4.0; MM = 3.5) and one mutation hotspot (FR3 (83I)) for IGKV1/D-33/IGKJ2 was associated with cardiac involvement.Conclusion: This study confirmed that germline usage has an influence on AL amyloidosis risk and organ involvement.","PeriodicalId":50964,"journal":{"name":"Amyloid-Journal of Protein Folding Disorders","volume":" ","pages":"86-94"},"PeriodicalIF":5.5,"publicationDate":"2024-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139418530","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

Symptomatic SARS-CoV2 infection associated with high mortality in AA amyloidosis. 无症状 SARS-CoV2 感染与 AA 淀粉样变性的高死亡率有关。

IF 5.2 2区医学

Amyloid-Journal of Protein Folding Disorders Pub Date : 2024-06-01 Epub Date: 2023-12-21 DOI: 10.1080/13506129.2023.2294434

Rim Bourguiba, Alexandre Terré, Lea Savey, Eric Oziol, Thomas Hanslik, Jean-Emmanuel Kahn, Raphael Borie, Alexandre Cez, David Buob, Gilles Grateau, Jean-Jacques Boffa, Sophie Georgin-Lavialle

引用次数: 0