Reference values for blood chemistry in the cotton rat (Sigmadon hispidus)

Fifteen blood chemistry values for cotton rat (Sig-modon hispidur) were measured in 17 females ranged from 122.0 and 170.0 g and 11 males ranged from 91.0 to 190.0 g in weight. Significant sex differences were found for creatinine and triclyccridc (P < 0.05). Six items (UA, TB, GPT, CK, HDLCHO, TG) in temales and 4 items (TB, GOT, GPT, CK) in males varied with coefficient of variation being over 40 %. The average values for TP, ALB, UA, TB in the cotton rat tended to be lower than those in the rat. On the contrary, the average values 0f GOT, GPT,  ALP, CK, LDH, GLU tended to be higher than those in the rat

Lack of Galactosylation Enhances the Pathogenic Activity of IgG1 but Not IgG2a Anti-Erythrocyte Autoantibodies

13301甲第4668号博士（医学）金沢大学博士論文要旨Abstract 以下に掲載：The Journal of Immunology 192(2) pp.581-588 2014. The American Association of Immunologists. 共著者：Kiyoaki Ito, Jun-ichi Furukawa, Kazunori Yamada, Ngoc Lan Tran, Yasuro Shinohara, Shozo Izu

The efficacy of radiation monotherapy for Tolosa-Hunt syndrome

金沢大学附属病院神経内

Hints to the diagnosis of uromodulin kidney disease.

Background: Uromodulin kidney disease (UKD) is an inherited kidney disease caused by a uromodulin (UMOD) gene mutation. The UMOD gene encodes the Tamm-Horsfall protein (THP), which is the most abundant protein in healthy human urine. Because of its rarity, the incidence of UKD has not been fully elucidated. The purpose of the present study is to clarify the frequency of UKD among patients who underwent renal biopsy. Methods: Immunostaining for THP was performed for patients <50 years of age with renal insufficiency and hyperuricemia without overt urinalysis abnormality from renal biopsy databases. Serum and urinary THP concentrations were evaluated in available individuals. Results: Fifteen patients were selected for immunostaining from a total of 3787 patients. In three independent patients, abnormal THP accumulation in renal tubular cells was observed. A novel missense A247P UMOD mutation was detected in two of the three patients, including one having a typical family history of familial juvenile hyperuricemic nephropathy. Serum and urinary THP concentrations of all available patients withUMODA247P mutationwere significantly lower than those of controls. Conclusions: In the present study, UKDwas detected in <1 in 1000 subjects who underwent renal biopsies. However, in subjects meeting all of the above criteria, abnormal THP accumulation was detected in 20% (3/15), suggesting that renal biopsy with immunostaining for THP is a good tool for diagnosing UKD. Also, lowserum THP concentration detected in the present subjects might be a good diagnostic marker or important in understanding the pathogenesis of UKD. © The Author 2015.Publisher\u27s version/PDF on institutional repository or centrally organised repositorie

A case report of crystalline light chain inclusion-associated kidney disease affecting podocytes but without Fanconi syndrome

RATIONALE:Crystalline light chain inclusion-associated kidney disease affects mainly tubular epithelial cells and is often clinically manifested as Fanconi syndrome. However, only very few case reports about the crystalline deposits within the podocytes are available, and the nature of the pathogenic monoclonal light chain implicated in these cases is still unknown. We report a case of crystalline inclusion-associated kidney disease manifested as crystalline podocytopathy in which we identified the complete structure of the pathogenic monoclonal light chain as belonging to the germ-line gene of Vκ1-39.PATIENT CONCERNS:We describe a 65-year-old woman with crystalline light chain inclusion-associated kidney disease showing mild proteinuria and renal insufficiency with monoclonal gammopathy of undetermined significance without Fanconi syndrome. She had crystalline inclusions mainly within podocytes, tubular epithelial cells and histiocytes in the kidney. Light microscopy showed vacuolation of podocytes and tubular epithelial cells, while eosin negative pale needle-like crystals were present within these cells. Electron microscopy showed accumulation of club-like crystals with high electron density in podocytes, proximal tubular epithelial cells and interstitial histiocytes. Clonal analysis revealed that a pathogenic monoclonal light chain was derived from germline gene, Vκ1-39.DIAGNOSES:The diagnosis of crystalline light chain inclusion-associated kidney disease was made.INTERVENTIONS AND OUTCOMES:Bortezomib and dexamethasone were started and her renal function improved to eGFR 36 mL/min/1.73 m after 9 courses of therapy.LESSONS:Patients with light chain crystalline podocytopathy may have a similar pathogenic monoclonal light chain derived from the same germline gene, Vκ1-39, to that of patients with light chain proximal tubulopathy

関節リウマチにおける糖鎖制御機構の解明

金沢大学附属病院関節リウマチ患者血中IgG においては、結合しているN結合型糖鎖のガラクトース欠損が知られている。近年TNFα 阻害薬治療により、糖鎖異常が正常パターンに戻ることが報告された。糖鎖は、抗体の受容体であるFcγ 受容体との相互作用や、補体結合作用において重要な働きをしている。我々は，IL-6 阻害薬投与症例およびDMARDs投与症例において，治療前後の血清を用いIgG を精製した。そして，MALDI-TOF MSを用いて血清IgG の糖鎖解析を行った。その結果，治療法に関わらず，治療後にはガラクトースが増加しているプロファイルがみられた。N-glycosylation pattern of IgG in patients with rheumatoid arthritis is reduced galactosylation. This aberrant glycosylation improve after TNF-alfa blockade therapy. Glycosylation of IgG is important in effector function of IgG such as interaction with Fc receptor. We purify serum IgG in patients who recieved IL-6 blockade or DMARDs. And we performed glycan analysis using MALDI-TOF MS. The aberrant glycosylation of IgG improve after each therapy.研究課題/領域番号:15K19572, 研究期間(年度):2015-04-01 – 2019-03-3

ヒトのクリオグロブリン血症性糸球体腎炎モデルマウスの作成

金沢大学附属病院クリオグロブリン(Cryo)血症性糸球体腎炎のモデルマウスを作成することを本研究の目的とし、Cryo血症患者よりCryo活性を持つIgM型モノクローナルRF(Cryo+IgM mRF)産生ハイブリドーマを樹立し、Balb/cマウスに投与した。その結果、尿中アルブミンの増加が認められた。腎組織の検討では、管内増殖およびメサンギウム細胞の増殖、Cryo+IgM mRFの糸球体への沈着を認めた。腎組織の変化は軽度であったが、Cryo血症性腎炎モデルマウス樹立の基礎となるデータが得られた。The purpose of our study is to establish a mouse model of cryoglobulinemic (Cryo) glomerulonephritis (GN). We established the hybridoma cell line producing IgM-kappa cryoglobulin with rheumatoid factor activity (Cryo+IgM RF) from a patient with Cryo GN. We administered Cryo+IgM RF to wild type Balb/c mice. Urinary albumin increased. Pathological analysis of kidney tissue showed mild endocapillary proliferation, mild mesangial cell proliferation, and deposition of Cryo+IgM RF in the glomeruli. These results provide basic data for the establishment of Cryo GN although renal pathological changes are mild.研究課題/領域番号:20790590, 研究期間(年度):2008 – 200