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1 fibrils in patients overproducing monoclonal immunoglobulin light chains.
2 free kappa (kappa) and free lambda (lambda) immunoglobulin light chains.
3 nd trafficking of other glycoproteins, or of immunoglobulin light chains.
4 n of amyloid fibrils derived from monoclonal immunoglobulin light chains.
5 minating between benign and pathologic human immunoglobulin light chains.
6 idosis is characterized by the misfolding of immunoglobulin light chains, accumulating as amyloid fib
8 outcome were determined for 80 patients with immunoglobulin light chain (AL) amyloidosis treated with
14 of the 2 main types of cardiac amyloidosis, immunoglobulin light chain (AL) and transthyretin (ATTR)
16 and limited therapeutic options characterize immunoglobulin light-chain (AL) amyloidosis with major h
17 e kidney is involved in 70% of patients with immunoglobulin light-chain (AL) amyloidosis, but little
19 uding heart involvement of either monoclonal immunoglobulin light-chain (AL) or transthyretin (ATTR)
32 is study, we address the structural basis of immunoglobulin light chain amyloidosis, which results fr
36 mparisons were made with other patients with immunoglobulin light-chain amyloidosis who did not have
38 al lymphoplasmacytic malignancies, including immunoglobulin light-chain amyloidosis, multiple myeloma
41 transcribed double-stranded (ds) DNA during immunoglobulin light chain and heavy chain class switch
43 is a lethal disease characterized by excess immunoglobulin light chains and light chain fragments co
44 ment of B cells that coexpress two different immunoglobulin light chains and, therefore, two antibody
45 nal B-cell disorders producing amyloidogenic immunoglobulin light chains, and the hereditary and "sen
47 y probabilities of selected positions of the immunoglobulin light chain-binding domain of protein L,
48 l surface and did not efficiently bind kappa immunoglobulin light chains, but did associate with Ig a
50 rimary (light chain-associated) amyloidosis, immunoglobulin light chains deposit as amyloid fibrils i
52 lls expressing CRBN, causing accumulation of immunoglobulin light-chain dimers, significantly increas
54 ta consist of 209 proteins of human antibody immunoglobulin light chains, each represented by aligned
55 BCR knock-in mice lacking self-Thy-1 ligand, immunoglobulin light chain editing occurred, generating
57 L transgene under the control of the 3'kappa immunoglobulin light chain enhancer, which is most activ
58 unction as transcriptional activators of the immunoglobulin light-chain enhancer E lambda 2.4 when co
59 Serum amyloid P component, apolipoprotein E, immunoglobulin light chains, Factor X, and complement pr
60 n amyloidosis is a devastating disease where immunoglobulin light chains form amyloid fibrils, result
62 hological deposition of insoluble fibrils of immunoglobulin light chain fragments in various tissues,
63 hological deposition of insoluble fibrils of immunoglobulin light-chain fragments in various organs a
64 ain gene segments as substrate for secondary immunoglobulin light chain gene rearrangement and is ind
66 for the ability of BCR ligation to stimulate immunoglobulin light chain gene rearrangements in immatu
67 the Fraction C'-D transition is critical for immunoglobulin light chain gene recombination and to pre
68 at is recruited to composite elements within immunoglobulin light-chain gene enhancers through a spec
69 hocyte developmental checkpoints inasmuch as immunoglobulin light-chain gene rearrangement occurred i
74 ing the immunoglobulin heavy chain (Igh) and immunoglobulin light chain (Igk) takes place sequentiall
76 Mass spectrometric analysis identified an immunoglobulin light chain in the band but found no PrP
78 presence of monoclonal free kappa or lambda immunoglobulin light chains in monoclonal gammopathy of
79 at increased monoclonal free kappa or lambda immunoglobulin light chains in smoldering multiple myelo
80 characterized by the deposition of monotypic immunoglobulin light chains in the kidney, resulting in
82 lization and cellular trafficking of urinary immunoglobulin light chains into cardiac fibroblasts.
84 w BiP interacts with a particular substrate, immunoglobulin light chain (lambda LC), during its foldi
89 used by the clonal production of an unstable immunoglobulin light chain (LC), which affects organ fun
90 n both AL and multiple myeloma (MM), soluble immunoglobulin light chains (LC) are produced by clonal
91 crasia characterized by clonal production of immunoglobulin light chains (LC) resulting in the subseq
93 s from overproduction of unstable monoclonal immunoglobulin light chains (LCs) and the deposition of
95 idosis and familial amyloid polyneuropathy), immunoglobulin light chains (light-chain amyloid), beta2
97 e, can also lead to allelic inclusion at the immunoglobulin light chain loci and the development of B
99 center B cells, demonstrating that the kappa immunoglobulin light-chain locus becomes a substrate for
100 ens and cytokines in regulation of the kappa immunoglobulin light-chain locus, we have characterized
101 protein misfolding disease where monoclonal immunoglobulin light chains misfold and deposit as amylo
103 arge membrane pores and high permeability to immunoglobulin light chains) or a conventional high-flux
105 izing and destabilizing mutations is key for immunoglobulin light-chains populating unfolded intermed
106 idosis, amyloid fibril deposits derived from immunoglobulin light chains produced by a clonal plasma
107 in conformation disorder in which monoclonal immunoglobulin light chains produced by clonal plasma ce
108 s element has considerable homology to mouse immunoglobulin light chain promoter sequences to which t
109 ed that the dimer interface of amyloidogenic immunoglobulin light chain protein AL-09 is twisted 90 d
110 ated with the overproduction of a monoclonal immunoglobulin light chain protein by a B-lymphocyte clo
111 egulatory factor 4 (IRF-4) and IRF-8 promote immunoglobulin light-chain rearrangement and transcripti
113 t with aberrant VJ recombination between the immunoglobulin light chain region (Ig kappa) on chromoso
115 t remains tightly bound to newly synthesized immunoglobulin light chains, resulting in retention of l
116 idosis is a protein misfolding disease where immunoglobulin light chains sample partially folded stat
117 ted with amyloid fibril formation, including immunoglobulin light chain, serum amyloid A protein, and
118 631 substitutions present in 90 nurse shark immunoglobulin light chain somatic mutants, 338 constitu
120 e B cells within the HL cell lines expressed immunoglobulin light chain, the memory B-cell antigen CD
122 exchange rates) and the propensity of human immunoglobulin light chains to form amyloid fibrils in v
123 ted an FcgammaRIIB-independent difference in immunoglobulin light-chain usage, consistent with an alt
125 re we identify the monomeric form of the Mcg immunoglobulin light chain variable domain as the quater
127 loid fibrils were investigated for a dimeric immunoglobulin light chain variable domain, employing pr
129 temic extracellular deposition of monoclonal immunoglobulin light chain variable domains in the form
130 This study characterizes the repertoire of immunoglobulin light chain variable genes used by the cl
131 tional dynamics of a pathogenic kappa4 human immunoglobulin light-chain variable domain, SMA, associa
132 model for the assembly of amyloid fibrils of immunoglobulin light-chain variable domains is proposed
133 s the free light-chain assay and the role of immunoglobulin light-chain variable region germline gene
134 The structure of a multisubunit protein (immunoglobulin light chain) was solved in three crystal
135 ubiquitylation sites on the NS-1 nonsecreted immunoglobulin light chain, which is an ERAD substrate.
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