Crescentic nodular glomerulosclerosis secondary to truncated immunoglobulin α heavy chain deposition

Cheng, Ignatius K.P.; Chan, Daniel W.M.; Ng, W K; Chan, Kwok–Wah

doi:10.1016/s0272-6386(96)90315-7

Cited by 31 publications

(22 citation statements)

References 7 publications

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“…1 The results above demonstrated diversity in the molecular weights of the mIg in several cancer cell lines, which suggested the existence of truncated Ig chains. Meanwhile, the production of truncated Ig by B cells has been reported by others [13][14][15][16][17][18] (Figure 5a). These results strongly imply that the protein structure of cancerous Ig differs from that of the normal antibody tetramer.…”

Section: Heterogeneity Of Ig-truncated Ig Is Produced By Cancer Cellssupporting

confidence: 56%

“…1, 12 Interestingly, some researchers found that under certain pathological conditions the B cell itself could produce aberrant or truncated Ig. [13][14][15][16][17][18] For example, in Waldenstrom's macroglobulinemia patients, a 64-kDa truncated m heavy chain fused with the Ig k sequence at its NH 2 terminal was identified by sequence analysis. 13 Additionally, even though the underlying cause is not clear, in one case of heavy-chain deposition disease, a c heavy chain lacking the CH1 region was identified by immunoblot analysis.…”

Section: Introductionmentioning

confidence: 99%

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Heterogeneity of aberrant immunoglobulin expression in cancer cells

Zhi

et al. 2011

Cell Mol Immunol

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Accumulating evidence has shown that immunoglobulin (Ig) is 'unexpectedly' expressed by epithelial cancer cells and that it can promote tumor growth. The main purpose of this study was to explore the components of the cancerous Ig and its possible function. The presence of cancerous Ig in the Golgi apparatus was confirmed by immunofluorescence, indirectly suggesting that the cancerous Ig was processed and packaged in cancer cells. Western blot analysis and ELISA results indicated that cancer cells produced membrane Ig and secreted Ig into the supernatant fraction. The cancerous Ig consists of an a heavy chain and a k light chain. Finally, by analyzing the Ig components pulled down by protein A beads, the cancerous Ig was found to be structurally distinct from normal Ig. The cancerous Ig was truncated or aberrant. Although the underlying mechanism that causes the abnormalities has not been determined, our current discoveries strengthen our previous findings and promise fruitful future explorations.

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Section: Heterogeneity Of Ig-truncated Ig Is Produced By Cancer Cellssupporting

confidence: 56%

Section: Introductionmentioning

confidence: 99%

Heterogeneity of aberrant immunoglobulin expression in cancer cells

Zhi

et al. 2011

Cell Mol Immunol

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“…In the vast majority of reported cases of LHCDD and HCDD, the deposited heavy chain was g. To our knowledge, there are only two previously reported cases of aHCDD (1,21) and two cases of aLHCDD (associated with k in one and with l in one) (8,22). In this study, we report one additional case of aHCDD and two additional cases of aLHCDD.…”

Section: Discussionmentioning

confidence: 55%

Renal Monoclonal Immunoglobulin Deposition Disease

Nasr¹,

Valeri²,

Cornell³

et al. 2012

Clinical Journal of the American Society of Nephrology

246

214

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SummaryBackground and objectives To better define the clinical-pathologic spectrum and prognosis of monoclonal immunoglobulin deposition disease (MIDD), this study reports the largest series.Design, setting, participants, & measurements Characteristics of 64 MIDD patients who were seen at Mayo Clinic are provided.Results Of 64 patients with MIDD, 51 had light chain deposition disease, 7 had heavy chain deposition disease, and 6 had light and heavy chain deposition disease. The mean age at diagnosis was 56 years, and 23 patients (36%) were #50 years of age. Clinical evidence of dysproteinemia was present in 62 patients (97%), including multiple myeloma in 38 (59%). M-spike was detected on serum protein electrophoresis in 47 (73%). Serum free light chain ratio was abnormal in all 51 patients tested. Presentation included renal insufficiency, proteinuria, hematuria, and hypertension. Nodular mesangial sclerosis was seen in 39 patients (61%). During a median of 25 months of follow-up (range, 1-140) in 56 patients, 32 (57%) had stable/improved renal function, 2 (4%) had worsening renal function, and 22 (39%) progressed to ESRD. The mean renal and patient survivals were 64 and 90 months, respectively. The disease recurred in three of four patients who received a kidney transplant.Conclusions Patients with MIDD generally present at a younger age than those with light chain amyloidosis or light chain cast nephropathy. Serum free light chain ratio is abnormal in all MIDD patients, whereas only threequarters have abnormal serum protein electrophoresis. The prognosis for MIDD is improving compared with historical controls, likely reflecting earlier detection and improved therapies.

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“…It also was suggested in a patient with ␣ HCDD (23). A larger deletion that also included the C H 1 domain, the hinge, and the C H 2 domain was found in one case (6).…”

Section: Hcdd: a Disease Featured By Hc Deletionsmentioning

confidence: 96%

Immunoglobulin Light (Heavy)-Chain Deposition Disease

Ronco¹,

Plaisier²,

Mougenot³

et al. 2006

Clinical Journal of the American Society of Nephrology

110

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Light-, light-and heavy-, and heavy-chain deposition diseases belong to a family of diseases that include light-chain (AL)-amyloid, nonamyloid fibrillary and immunotactoid glomerulonephritis, and cryoglobulinemic glomerulonephritis, in which monoclonal Ig or their subunits become deposited in kidney. In clinical and pathologic terms, light-, light-and heavy-, and heavy-chain deposition diseases essentially are similar and are characterized by prominent renal involvement with severe renal failure; extrarenal manifestations; diabetes-like nodular glomerulosclerosis; marked thickening of tubular basement membranes; and monotypic deposits of light chain, mostly , and/or heavy chain that feature a nonorganized granular, electron-dense appearance by electron microscopy. The most common cause is myeloma. Recent progress has been made in the understanding of the molecular pathomechanisms of Ig-chain deposition and extracellular matrix accumulation, which opens up new therapeutic avenues in addition to eradication of the Ig-secreting plasma cell clone. Because these diseases represent a model of glomerular and interstitial fibrosis that is induced by a single molecule species, a better understanding of their pathomechanisms may help to unravel the pathophysiology of kidney fibrosis and renal disease progression.

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Crescentic nodular glomerulosclerosis secondary to truncated immunoglobulin α heavy chain deposition

Cited by 31 publications

References 7 publications

Heterogeneity of aberrant immunoglobulin expression in cancer cells

Heterogeneity of aberrant immunoglobulin expression in cancer cells

Renal Monoclonal Immunoglobulin Deposition Disease

Immunoglobulin Light (Heavy)-Chain Deposition Disease

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