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Intracellular Maturation of Acute Phase Proteins
Published in Andrzej Mackiewicz, Irving Kushner, Heinz Baumann, Acute Phase Proteins, 2020
Erik Fries, E. Mathilda Sjöberg
Bikunin is synthesized as a precursor in which it is linked to a1-microglobulin by two arginine residues.95,96 In plasma, most bikunin occurs as a subunit of the three-chain protein inter-α-inhibitor and of the two-chain protein pre-α-inhibitor.97 In both these proteins, all polypeptides are linked by bikunin’s chondroitin sulfate chain.98,99 To study the late steps in the biosynthesis of bikunin, we did pulse-chase experiments with rat hepatocytes labeled with [35S]sulfate. This labeling revealed a short-lived intermediate of the α1-microglobulin/bikunin precursor with a full-size chondroitin sulfate chain (Figure 3, band c). Chasing for 5 to 10 min showed that this precursor was cleaved to form a 40-kDa band — free bikunin (band c’). Thus, cleavage occurs after sulfation, indicating a spatial separation of the correspoding enzymes. In blood, α1-microglobulin occurs in complex with an unidentified brown-colored ligand.100 This binding capacity of α1-microglobulin could pose a hazard to the cell during intracellular transport and the binding might therefore be blocked in the precursor.
The Non-Hodgkin’s Lymphomas and Plasma Cell Dyscrasias
Published in Harold R. Schumacher, William A. Rock, Sanford A. Stass, Handbook of Hematologic Pathology, 2019
Lynne V. Abruzzo, L. Jeffrey Medeiros
Plasma cell myeloma is a progressive disease with a poor prognosis. The median survival with conventional therapy is approximately 3 years. The length of survival is closely related to the stage of the disease at diagnosis. Patients are monitored with periodic determinations of serum and urine M-protein levels, and serum beta-2 microglobulin levels. Elevated levels of serum beta-2 microglobulin are associated with progressive disease and a worse prognosis. Complete remissions are unusual, and patients die of the disease. Infection is the most common cause of death. Renal failure is a contributing factor in many cases.
Renal Effects
Published in Lars Friberg, Tord Kjellström, Carl-Gustaf Elinder, Gunnar F. Nordberg, Cadmium and Health: A Toxicological and Epidemiological Appraisal, 2019
Human β2-microglobulin has a molecular weight of 11,600.15 The amino acid sequence is identical to a chain in IgG.16 β2-microglobulin has cytophilic properties and is found on the surface of most nonmalignant cells. On the surface of each mammalian lymphocyte there are about 100,000 β2-microglobulin molecules. Malignant human lymphoma cell lines, however, have lower amounts of β2-microglobulin on their surface. β2-microglobulin is identical to the small invariant polypeptide chain of the serologically defined strong histocompatibility antigens, and it appears to be involved in the mechanism of lymphocyte activation and other immunological mechanisms.206 It has been suggested that β2-microglobulin and immunoglobulins evolved from the same ancestral gene, which would explain an evolutionary link between histocompatibility antigens and immunoglobulins. In spite of a wealth of research on this topic in recent years, the function of β2-microglobulin is still not clear. The data point to β2-microglobulin and β2-microglobulin-associated molecules playing a role in immunological cell-cell recognition.16
The Prognostic Impact of Pretreatment Geriatric Nutritional Risk Index in Patients with Diffuse Large B-Cell Lymphoma
Published in Nutrition and Cancer, 2023
Unal Atas, Hasan Sozel, Utku Iltar, Orhan Kemal Yucel, Ozan Salim, Levent Undar
The GNRI calculation has variables such as albumin and weight. Therefore, we attribute the slightly higher GNRI cutoff value in our study compared to other studies to the fact that it was conducted in a different geographical region and in a different ethnic population. When evaluated in terms of features other than age and prognostic risk classifications, in other studies, as in our study, increased LDH levels were reported with a higher rate of B symptoms and the presence of extanodal disease in the group of patients with a low GNRI (20–24). In addition, lymphocyte count, hemoglobin and albumin levels were lower in the low GNRI patient group in the Chinese study (20). In the two Japanese studies, the albumin level was reported to be lower (22, 23), as in our study. In contrast to other studies that showed that the number of patients with advanced stage was higher in the patient group with a low GNRI (20–24), the number of patients with advanced stage in our study was statistically similar in both groups, although the number of patients with a low GNRI was higher in the patient group. Finally, we found that the β2 microglobulin level, which was not evaluated in other studies, was higher in the patient group with a low GNRI.
Dialysis-related amyloidosis associated with a novel β2-microglobulin variant
Published in Amyloid, 2021
Hiroki Mizuno, Junichi Hoshino, Masatomo So, Yuta Kogure, Takeshi Fujii, Yoshifumi Ubara, Kenmei Takaichi, Tetsuko Nakaniwa, Hideaki Tanaka, Genji Kurisu, Fuyuki Kametani, Mayuko Nakagawa, Tsuneaki Yoshinaga, Yoshiki Sekijima, Keiichi Higuchi, Yuji Goto, Masahide Yazaki
A 41-year-old man with a 20-year history of haemodialysis due to IgA vasculitis was referred to our hospital complaining of dysphagia, difficulty in walking, bilateral leg tenderness, and arthralgia for 6 months. His tongue was extremely swollen (Figure 1(A)), resulting in severe dysarthria and dysphagia. Enlarged salivary glands and marked joint swelling with deformity were observed (Figure 1(B)). Peripheral neuropathy including carpal tunnel syndrome were not observed. X-ray analysis and computed tomography revealed swelling with calcification in the elbow, hip joints, and tongue (Figure 1(C–E)). Laboratory examinations revealed highly elevated blood urea nitrogen and serum creatinine levels of 68 mg/dL and 5.7 mg/dL, respectively. The serum level of α1-microglobulin was highly elevated to 130.7 mg/L (normal, 12.5–25.5 mg/L). In contrast, the serum β2m level was mildly raised to 9.4 mg/L(normal, <2 mg/L).
Hemophagocytic lymphohistiocytosis in adults
Published in Baylor University Medical Center Proceedings, 2020
Yadav Pandey, Dinesh Atwal, Manojna Konda, Milan Bimali, Derek Middleton, Naveen Yarlagadda, Belal Firwana, Appalanaidu Sasapu
We also collected data on seven additional variables easily obtained on routine laboratory or physical examination, which have been suggested by experts as useful variables in diagnosing HLH.11,12 Those variables include the presence of hepatitis (defined as liver enzymes 2.5 times the upper limit of normal), hepatomegaly (clinical exam or imaging), elevated lactate dehydrogenase (>200 IU/L), coagulopathy (defined as prothrombin time ≥1.5 times normal, activated partial thromboplastin time ≥1.5 times normal, or D-dimer >10 mcg/mL), monocytosis (absolute monocyte count >1.0 × 109/L), hypoalbuminemia (<3.5 g/dL), and renal failure (≥50% increase in serum creatinine). Some experts also suggest beta-2 microglobulin level as a useful variable for diagnosis of HLH.13 As we had data on only five patients’ beta-2 microglobulin levels, we did not include it in our results. Combining eight HLH-2004 criteria and seven additional variables, we plotted the number of patients meeting each criterion in a graph.