Ccl2/Mcp-1 blockade reduces glomerular and interstitial macrophages but does not ameliorate renal pathology incollagen4A3-deficient mice with autosomal recessive Alport nephropathy

Background: Wolfram syndrome (WFS, OMIM: #222300) is an ultrarare autosomal recessive disorder characterized by diabetes insipidus, diabetes mellitus, optic nerve atrophy and deafness. It has been reported that the average retinal thickness in WFS patients decreases with the progression of the disease. Aim: To investigate retinal thickness and wolframin expression disorders in Wolfram syndrome 1 gene knockout (Wfs1KO) mice compared to their wild-type (WT) littermates. Materials and methods: Both bulbs with optic nerves of three mice Wfs1WT and three Wfs1KO were taken for the histopathological examination. A strain of knockout mice with mutation in exon 8 was used. Results: No expression of wolframin protein in the retina and neurodegeneration of the optic nerve of Wfs1KO mice as compared among Wfs1WT mice was observed. The mean central retinal thickness was thinner and the retinal thickness/longitudinal diameter ratio was significantly lower in hte Wfs1KO as compared to the Wfs1WT mice. In four (67%) eyeballs of Wfs1KO mice, intra-retinal neovessels were observed. Conclusions: Wfs1KO mice retina with mutation in exon 8 present similar clinical features as patients with WFS in the form of reduced retinal thickness and neurodegeneration of the optic nerve. The presence of proliferative retinopathy observed in Wfs1KO mice requires further investigation.

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Interleukin-6 is essential for glomerular immunoglobulin A deposition and the development of renal pathology in Cd37-deficient mice

Kidney International ◽

10.1016/j.kint.2018.01.005 ◽

2018 ◽

Vol 93 (6) ◽

pp. 1356-1366 ◽

Cited By ~ 9

Author(s):

Angelique L.W.M.M. Rops ◽

Erik Jansen ◽

Alie van der Schaaf ◽

Elmar Pieterse ◽

Nils Rother ◽

...

Keyword(s):

Interleukin 6 ◽

Immunoglobulin A ◽

Renal Pathology ◽

Deficient Mice

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Unusual Cause of Childhood Anemia: Imerslund Grasbeck Syndrome

Journal of Laboratory Physicians ◽

10.4103/0974-2727.86845 ◽

2011 ◽

Vol 3 (02) ◽

pp. 113-115 ◽

Cited By ~ 1

Author(s):

Kishan Prasad Hosapatna Laxminarayana ◽

Sunil Kumar Yeshvanth ◽

Jayaprakash K Shetty ◽

Harish S Permi ◽

Chandrika Rao

Keyword(s):

Hemolytic Anemia ◽

Autosomal Recessive ◽

Megaloblastic Anemia ◽

Young Female ◽

Renal Pathology ◽

Complete Evaluation ◽

Childhood Anemia ◽

Chronic Hemolytic Anemia ◽

Asymptomatic Proteinuria ◽

Childhood Disorder

ABSTRACTImerslund Grasbeck syndrome (IGS) is a rare autosomal recessive childhood disorder characterized by selective Vitamin (vit) B 12 malabsorption with asymptomatic proteinuria without any structural renal pathology. The patients stay healthy for decades with life-long parenteral vit B12. We report a case of young female who presented with pancytopenia and proteinuria, evaluated in local hospitals as chronic hemolytic anemia (autoimmune cause), finally diagnosed as IGS on complete evaluation. She was treated with injectable vit B12 (1000 μg cyanocobalalmin) and showed drastic recovery. IGS should be considered in patients with megaloblastic anemia not responding to oral vit B12 and associated proteinuria.

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Exaggerated Renal Pathology of Partial Ablation-Induced Chronic Renal Failure in eNOS Deficient Mice

Biological and Pharmaceutical Bulletin ◽

10.1248/bpb.31.1029 ◽

2008 ◽

Vol 31 (5) ◽

pp. 1029-1031 ◽

Cited By ~ 8

Author(s):

Chika Yamashita ◽

Naoko Tazawa ◽

Mamoru Ohkita ◽

Yasuo Matsumura

Keyword(s):

Renal Failure ◽

Chronic Renal Failure ◽

Renal Pathology ◽

Deficient Mice

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Comparison of localization of metallothionein in tissues of metallothionein-deficient mice

Proceedings, annual meeting, Electron Microscopy Society of America ◽

10.1017/s0424820100141585 ◽

1995 ◽

Vol 53 ◽

pp. 1042-1043

Author(s):

H. Nishimura ◽

R Nishimura ◽

D.L. Adelson ◽

A.E. Michaelska ◽

K.H.A. Choo ◽

...

Keyword(s):

Metal Binding ◽

Immunohistochemical Staining ◽

Squamous Epithelium ◽

Rabbit Antiserum ◽

Slight Modification ◽

Positive Control ◽

Heavy Metal Binding ◽

Critical Organ ◽

Metal Binding Protein ◽

Deficient Mice

Metallothionein (MT), a cysteine-rich heavy metal binding protein, has several isoforms designated from I to IV. Its major isoforms, I and II, can be induced by heavy metals like cadmium (Cd) and, are present in various organs of man and animals. Rodent testes are a critical organ to Cd and it is still a controversial matter whether MT exists in the testis although it is clear that MT is not induced by Cd in this tissue. MT-IV mRNA was found to localize within tongue squamous epithelium. Whether MT-III is present mainly glial cells or neurons has become a debatable topic. In the present study, we have utilized MT-I and II gene targeted mice and compared MT localization in various tissues from both MT-deficient mice and C57Black/6J mice (C57BL) which were used as an MT-positive control. For MT immunostaining, we have used rabbit antiserum against rat MT-I known to cross-react with mammalian MT-I and II and human MT-III. Immunohistochemical staining was conducted by the method described in the previous paper with a slight modification after the tissues were fixed in HistoChoice and embedded in paraffin.

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