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  • 1
    Keywords: Konferenzschrift
    Type of Medium: Book
    Pages: S.1 - 135 , Ill., graph.. Darst., Kt
    Series Statement: Fisheries research 84.2007,1
    Language: English
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  • 2
    Publication Date: 2012-10-02
    Description: Proteinuria is considered to play an essential role in the progression of tubulointerstitial damage, which causes end-stage renal disease. Fatty acid-binding albumins are filtered through glomeruli and reabsorbed into proximal tubular epithelial cells (PTECs). However, the role of fatty acid metabolism associated with albuminuria in the development of tubulointerstitial damage remains unclear. Thus, the present study was designed to determine the changes of fatty acid metabolism in the nephrotic kidney. To induce nephrotic syndrome, Sprague-Dawley rats (SDRs) and Nagase analbuminemic rats (NARs) with inherited hypoalbuminemia were treated with a single injection of puromycin aminonucleoside (PAN). In SDRs, PAN treatment induced massive proteinuria and albuminuria and caused tubular damage, apoptosis, and lipid accumulation in PTECs. Among the enzymes of fatty acid metabolism, expressions of medium-chain acyl-CoA dehydrogenase (MCAD) and cytochrome P -450 (CYP)4A significantly decreased in PTECs of PAN-treated SDRs. Expressions of peroxisome proliferator-activated receptor (PPAR)- coactivator (PGC)-1α and estrogen-related receptor (ERR)α also significantly decreased, without changes in the expression of PPAR-α. In NARs, PAN treatment induced proteinuria but not albuminuria and did not cause tubular damage, apoptosis, or lipid accumulation. Expressions of MCAD, PGC-1α, or ERRα did not change in the kidney cortex of PAN-treated NARs, but the expression of CYP4A significantly decreased. These results indicate that massive albuminuria causes tubular damage and lipid accumulation with the reduction of MCAD, CYP4A, PGC-1α, and ERRα in PTECs.
    Print ISSN: 1931-857X
    Electronic ISSN: 1522-1466
    Topics: Medicine
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  • 3
    Publication Date: 2016-03-12
    Description: Uniparental disomy (UPD) is defined as the inheritance of both homologs of a given genomic region from only one parent. The majority of UPD includes an entire chromosome. However, the extent of UPD is sometimes limited to a subchromosomal region (segmental UPD). Mosaic paternal UPD (pUPD) of chromosome 11 is found in approximately 20% of patients with Beckwith–Wiedemann syndrome (BWS) and almost all pUPDs are segmental isodisomic pUPDs resulting from mitotic recombination at an early embryonic stage. A mechanism initiating a DNA double strand break (DSB) within 11p has been predicted to lead to segmental pUPD. However, no consensus motif has yet been found. Here, we analyzed 32 BWS patients with pUPD by SNP array and searched for consensus motifs. We identified four consensus motifs frequently appearing within breakpoint regions of segmental pUPD. These motifs were found in another nine BWS patients with pUPD. In addition, the seven motifs found in meiotic recombination hot spots could not be found within pUPD breakpoint regions. Histone H3 lysine 4 trimethylation, a marker of DSB initiation, could not be found either. These findings suggest that the mechanism(s) of mitotic recombination leading to segmental pUPD are different from that of meiotic recombination. Furthermore, we found seven patients with paternal uniparental diploidy (PUD) mosaicism. Comparison of clinical features between segmental pUPDs and PUDs showed that developmental disability and cardiac abnormalities were additional characteristic features of PUD mosaicism, along with high risk of tumor development. We also found that macroglossia was characteristic of segmental pUPD mosaicism.
    Print ISSN: 0964-6906
    Electronic ISSN: 1460-2083
    Topics: Biology , Medicine
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  • 4
    ISSN: 1440-1797
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Type of Medium: Electronic Resource
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  • 5
    Electronic Resource
    Electronic Resource
    Melbourne, Australia : Blackwell Science Pty
    Nephrology 7 (2002), S. 0 
    ISSN: 1440-1797
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Type of Medium: Electronic Resource
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  • 6
    ISSN: 1440-1797
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Application criteria of steroid therapy for the patients of IgA nephropathy (IgAN) have not yet been established. The purpose of the present study was to establish retrospectively the clinical and pathological criteria for the steroid therapy by using a histological scoring on 104 adult patients of IgAN. Steroid therapy was designated as an administration of prednisolone in the amount of more than 30 mg per day in the period of more than 4 weeks within 1 year of kidney biopsy.We developed our own scoring system for the following main glomerular and tubulointerstitial changes as shown in 〈link href="#t1"〉Table 1. The histological scoring was expressed by evaluating semiquantitatively the extent of glomerular and tubulointerstitial lesions in terms of activity index (AI) and chronicity index (CI). Activity index is the sum of graded score according to the extent of glomeruli with mesangial hypercellularity, intracapillary macrophagic infiltration and cellular crescent as well as to the extent of interstitial inflammation and tubulitis. Chronicity index is the sum of graded score according to the extent of glomeruli with global sclerosis, increase of extracellular matrices or periglomerular fibrosis, and tuft adhesion or fibrous (or fibrocellular) crescent as well as to the extent of interstitial fibrosis (〈link href="#t1"〉Table 1).〈tabular xml:id="t1"〉1〈title type="main"〉 Histological scoring 〈table frame="topbot"〉〈tgroup cols="5" align="left"〉〈colspec colnum="1" colname="col1" align="left"/〉〈colspec colnum="2" colname="col2" align="center"/〉〈colspec colnum="3" colname="col3" align="center"/〉〈colspec colnum="4" colname="col4" align="center"/〉〈colspec colnum="5" colname="col5" align="center"/〉〈thead valign="bottom"〉〈row rowsep="1"〉Score0123〈tbody valign="top"〉〈entry namest="col1" nameend="col5" align="left"〉AI: Activity Index〈entry namest="col1" nameend="col5" align="left"〉G: glomerularm: mesangial hypercellularity−〈 40%〈 80%≥ 80%i: intracapillary macrophage infiltration−+++e: cellular crescent0〈 30%≥ 30%〈entry namest="col1" nameend="col5" align="left"〉I: tubulointerstitiali: interstitial inflammation−+++t: tubulitis−+++〈entry namest="col1" nameend="col5" align="left"〉CI: Chronicity Index〈entry namest="col1" nameend="col5" align="left"〉G: glomerulars: global sclerosis〈 10%〈 30%〈 50%≥ 50%i: increase of extracellular matrix〈 10%〈 30%〈 50%≥ 50%e: fibrous (or fibrocellular) crescent, adhesion〈 10%〈 30%〈 50%≥ 50% I: tubulointerstitial
 interstitial fibrosis〈 10%〈 30%〈 50%≥ 50%〈note xml:id="t1_note5" numbered="no"〉AI: AGm + AGi + AGe*2 + AIi + Ait, CI: CGS + CGi + CGe + CIFor the applicability of steroid therapy, three groups were categorized by evaluating the statistical significance for the correlation of AI, CI and daily amount of urine protein to the outcome of the patients as follows (〈link href="#t2"〉Table 2). In group A (inappropriate indication of steroid therapy) which showed CI ≥ 5 alone, 10 out of 11 cases revealed decline of renal function (Cr ≥ 1.2 mg/dL and Ccr 〈 80 mL/min) within 2.2–19.3 years (mean 9.0 ± 6.4 years) without respect to steroid therapy. In group B (unnecessary indication of steroid therapy) which showed CI 〈 5, AI 〈 5, and UP 〈 1 g/day, 58 out of 60 cases showed normal renal function (Cr 〈 1.2 mg/dL and Ccr ≥ 80 mL/min) within 4.2–21.6 years (mean 10.1 ± 4.7 years). In group C (necessary indication of steroid therapy) which showed CI 〈 5 and AI ≥ 5 or UP ≥ 1 g/day, patients with steroid therapy revealed significantly higher incidence of outcome with normal renal function (12 out of 13 patients, final evaluation of renal function in 6.8 ± 2.3 years after renal biopsy) than that of the patient without steroid therapy (seven out of 20 cases, evaluation of renal function in 9.2 ± 4.0 years after renal biopsy) (P 〈 0.01) (〈link href="#t3"〉Table 3). In the 13 patients with steroid therapy in group C (steroid pulse in four patients, prednisolone 40 mg/day internally in three patients, predonisolone 30 mg/day internally in six patients) showed a significant decrease of proteinuria and remained until final evaluation time (〈link href="#t4"〉Table 4).〈tabular xml:id="t2"〉2〈title type="main"〉 Application criteria of steroid therapy 〈mediaResource alt="image" href="urn:x-wiley:13205358:NEP15:NEP_15_t2"/〉〈tabular xml:id="t3"〉3〈title type="main"〉 Comparison of steroid (−) with steroid (+) in the group of ‘necessary’ 〈table frame="topbot"〉〈tgroup cols="4" align="left"〉〈colspec colnum="1" colname="col1" align="left"/〉〈colspec colnum="2" colname="col2" align="center"/〉〈colspec colnum="3" colname="col3" align="center"/〉〈colspec colnum="4" colname="col4" align="center"/〉〈thead valign="bottom"〉〈row rowsep="1"〉steroid therapy+− P 〈row rowsep="1"〉no.1320〈tbody valign="top"〉〈entry namest="col1" nameend="col4" align="left"〉At Renal Biopsyage (years)〈entry align="char" char="[plusmn]"〉31.5 ± 12.936.3 ± 13.0nsUP (g/day)〈entry align="char" char="[plusmn]"〉2.3 ± 1.91.3 ± 0.5〈 0.05Cr (mg/dL)〈entry align="char" char="[plusmn]"〉0.8 ± 0.30.9 ± 0.1nsCCr (mL/min)〈entry align="char" char="[plusmn]"〉111 ± 4392 ± 34nsHypertension (n)19ns+ ACEI (n)410nsFollow-up years from
 renal biopsy〈entry align="char" char="[plusmn]"〉6.8 ± 2.39.2 ± 4.0ns〈entry namest="col1" nameend="col4" align="left"〉End of follow-upCr (mg/dL)〈entry align="char" char="[plusmn]"〉0.8 ± 0.35.5 ± 6.6〈 0.01 Normal renal function
 (n)127 Renal insufficiency (n)16〈 0.01 Dialysis (n)07〈tabular xml:id="t4"〉4〈title type="main"〉 Change of urine protein in the 13 cases with steroid treatment in the group of 'necessary' 〈mediaResource alt="image" href="urn:x-wiley:13205358:NEP15:NEP_15_t4"/〉From the results above, our evaluation system using histological scoring together with grading proteinuria was proven to be useful in estimating the applicability of steroid therapy for adult IgAN patients.
    Type of Medium: Electronic Resource
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  • 7
    Electronic Resource
    Electronic Resource
    Oxford, UK : Blackwell Publishing Ltd
    Nephrology 7 (2002), S. 0 
    ISSN: 1440-1797
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Type of Medium: Electronic Resource
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  • 8
    Electronic Resource
    Electronic Resource
    Amsterdam : Elsevier
    BBA Section Nucleic Acids And Protein Synthesis 655 (1981), S. 189-194 
    ISSN: 0005-2787
    Keywords: (E. coli) ; DNA polymerase ; Nucleoid protein
    Source: Elsevier Journal Backfiles on ScienceDirect 1907 - 2002
    Topics: Biology
    Type of Medium: Electronic Resource
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  • 9
    Electronic Resource
    Electronic Resource
    Amsterdam : Elsevier
    FEBS Letters 292 (1991), S. 128-132 
    ISSN: 0014-5793
    Keywords: Aldolase A ; CAT assay ; Gene expression ; Promoter ; Rat
    Source: Elsevier Journal Backfiles on ScienceDirect 1907 - 2002
    Topics: Biology , Chemistry and Pharmacology , Physics
    Type of Medium: Electronic Resource
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  • 10
    Electronic Resource
    Electronic Resource
    Amsterdam : Elsevier
    FEBS Letters 292 (1991), S. 128-132 
    ISSN: 0014-5793
    Keywords: Aldolase A ; CAT assay ; Gene expression ; Promoter ; Rat
    Source: Elsevier Journal Backfiles on ScienceDirect 1907 - 2002
    Topics: Biology , Chemistry and Pharmacology , Physics
    Type of Medium: Electronic Resource
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