Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: EC:3.2.1.17 (lysozyme)
 21,489 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Clinical aspects of hereditary amyloidoses are very diverse, and they pose many diagnostic problems to the physician. Biochemical and genetic aspects are also various. Several proteins are implicated in these hereditary diseases: transthyretin, apolipoprotein A1, gelsolin, fibrinogen alpha chain and lysozyme. Studies on structural changes induced by the mutations in these proteins should bring new data relevant to our understanding of the amyloidogenic process. Familial mediterranean fever is a hereditary disease of the inflammatory reaction which is associated with AA amyloidosis.
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PMID:[Hereditary amyloidosis]. 945 3

We aimed to reassess the suitability of immunohistochemical classification of amyloid in surgical pathology. One hundred sixty-nine biopsies from 121 patients diagnosed with amyloid during the period from 1994 to 2004 were included. Amyloid was classified immunohistochemically, using antibodies directed against amyloid P-component, AA amyloid, apolipoprotein AI, fibrinogen, keratoepithelin, lactoferrin, lysozyme, beta2-microglobulin (beta2M), immunoglobulin-derived lambda-light and kappa-light chains, and transthyretin. Amyloid was most commonly present in biopsies from the hepatogastrointestinal tract. The deposits were classified immunohistochemically in 156 (92%) biopsies. In 13 biopsies of 12 patients, amyloid remained unclassified. AL amyloidosis was diagnosed in 76 (45%) biopsies and was further categorized into AL amyloid of kappa-light chain origin [32 (42%) biopsies] or lambda-light chain origin [20 (26%)]. In 24 (32%) biopsies, the amyloid deposits did not show unequivocal staining for lambda-light or kappa-light chain. However, these cases were categorized as "probably AL amyloid, not otherwise specified", because no other antibody showed unequivocal staining of the amyloid deposits. AA amyloidosis was diagnosed in 32, ATTR amyloidosis in 21, and AApoAI amyloidosis in 3 biopsies. Other types of amyloid included AKer and ALac amyloids each in 1, and ALys and ACal amyloids each in 2 biopsies. Abeta2M amyloid was not diagnosed in any case. Immunohistochemical classification of amyloid still poses problems. Although classification of AA, AApoAI, ALys, ALac, and ATTR amyloids is relatively straightforward, classification of AL amyloid and rare hereditary amyloidoses is a serious obstacle and sometimes even impossible when conclusive clinical information or additional protein biochemical or molecular biologic studies are not available.
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PMID:Immunohistochemical classification of amyloid in surgical pathology revisited. 1672 44

We aimed to reassess renal amyloidosis in kidney biopsies with a focus on possibly misclassified or unclassified cases and changes in the prevalence of different amyloid types. Two hundred thirty-three kidney biopsies obtained from 231 patients diagnosed with amyloid during the period from 1990 to 2007 years were included in this retrospective study. Amyloid was identified by Congo red staining and polarization microscopy. Immunohistochemical classification was made with antibodies directed against AA amyloid, apolipoprotein A1, fibrinogen, lysozyme, lambda-light chain, kappa-light chain, beta2-microglobulin, transthyretin, and amyloid P-component. Amyloid was present in each biopsy as vascular, tubulo-interstitial and/or glomerular deposits. Immunoglobulin light chain-derived (AL) amyloidosis was most prevalent and diagnosed in 123 (53.2%) patients. It was categorized into AL amyloid of lambda-light chain (ALlambda) [105 (85.4%) patients] and kappa-light chain origin (ALkappa) [10 (8.1%)]. The amyloid deposits of 8 (6.5%) patients were not clearly distinguishable into ALlambda amyloid or ALkappa amyloid and categorized as AL amyloid, not otherwise specified. Reactive systemic amyloid A (AA) amyloidosis was the second most common type and was found in 93 patients (40.3%). Overall 7 patients were found to suffer from fibrinogen A alpha-chain-[amyloid of fibrinogen (AFib); 4 (1.7%) patients], transthyretin-[amyloid of transthyretin (ATTR); 2 (0.9%)], or apolipoprotein A1-derived (AApoAI) amyloidosis [1 (0.4%)]. In 8 patients (3.4%) the amyloid deposits remained unclassifiable. After additional immunostaining and further clinical information the diagnoses of 12 patients (5.1%) were modified (2 ALlambda amyloid, 4 ALkappa amyloid, 1 amyloid unclassified, 3 mixed-type amyloidosis, AA+ATTR, ALlambda+ATTR, and ALkappa+ATTR, 1 AFib, and 1 AApoAI). Although the histologic and immunohistochemical reevaluation confirmed the classifications in 221 (95.7%) patients. Renal amyloidosis is most commonly of ALlambda-origin, followed by AA amyloidosis. AFib amyloidosis was found to be the most prevalent type of hereditary renal amyloidosis, illustrating the necessity of a thorough classification of the amyloid proteins.
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PMID:Prevalence and origin of amyloid in kidney biopsies. 1956 48

Amyloidosis has traditionally been of a few defined varieties, most commonly including light-chain amyloidosis (AL amyloidosis) and secondary amyloidosis due to chronic inflammation (AA amyloidosis). Apolipoprotein A-I/A-II cystatin C, gelsolin, lysozyme, fibrinogen alpha chain, beta 2 microglobulin, and transthyretin familial amyloidosis represent rarer but reported varieties. Ten years ago, the first reports linked leukocyte chemotactic factor 2 (LECT2) amyloidosis as a pathological agent identified as a novel class of amyloid-generating protein. Epidemiology suggested that this was a new cause of amyloidosis that is especially common in Hispanic patients and somewhat common among patients from the Middle East-North Africa (MENA) region. We report a case of splenic and renal LECT 2 amyloidosis in a 62-year- old Hispanic male with diabetes mellitus. After an unremarkable serological workup, LECT 2 amyloidosis was diagnosed on renal biopsy. The case presentation is reviewed as a typical presentation, and the literature is reviewed regarding this newly reported entity, resulting in infiltrative renal amyloidosis and chronic renal disease.
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PMID:A case of renal and splenic LECT 2 amyloidosis: A recently recognized cause of renal and systemic amyloidosis. 3239 25