LIVIVO - The Search Portal for Life Sciences

zur deutschen Oberfläche wechseln
Advanced search

Search results

Result 1 - 10 of total 842

Search options

  1. Book ; Thesis: Studies of the role of complement factor h in hemolytic uremic syndrome

    Vaziri-Sani, Fariba

    (Doctoral dissertation series ; 2005,37)

    2005  

    Author's details Fariba Vaziri-Sani
    Series title Doctoral dissertation series ; 2005,37
    Collection
    Language English ; Swedish
    Size Getr. Zählung : Ill., graph. Darst.
    Publishing country Sweden
    Document type Book ; Thesis
    Thesis / German Habilitation thesis Lund, Univ., Diss., 2005
    Note Zsfassung in schwed. Sprache
    HBZ-ID HT014719333
    ISBN 91-85481-62-9 ; 978-91-85481-62-0
    Database Catalogue ZB MED Medicine, Health

    More links

    Kategorien

  2. Article ; Online: Recombinant truncated E protein as a new vaccine candidate against nontypeable H. influenzae: Its expression and immunogenic evaluation.

    Behrouzi, Ava / Bouzari, Saeid / Vaziri, Farzam / Fateh, Abolfazl / Afrough, Parviz / Vijeh Motlagh, Atefeh Davoudi / Siadat, Seyed Davar

    Microbial pathogenesis

    2017  Volume 110, Page(s) 431–438

    Abstract: ... encapsulated H. influenzae. This is a small surface lipoprotein, consisting of only 160 amino acids, involved ... in the adhesion of H. influenzae to various types of epithelial cells. A 384-bp-long fragment from NTHi PE was ...

    Abstract Protein E (PE) is a conserved entity observed in both nontypeable Haemophilus influenzae (NTHi) and encapsulated H. influenzae. This is a small surface lipoprotein, consisting of only 160 amino acids, involved in the adhesion of H. influenzae to various types of epithelial cells. A 384-bp-long fragment from NTHi PE was cloned into the prokaryotic expression vector pBAD-gIIIA. The recombinant protein was expressed with arabinose and then purified by affinity purification on an Ni-NTA agarose matrix. BALB/c mice were immunized by subcutaneous injection with purified recombinant truncated PE mixed with an alum adjuvant. Serum antibody response and the functional activity of antibodies were evaluated by enzyme-linked immunosorbent assay (ELISA) and serum bactericidal assay (SBA), respectively. Colony PCR, double digestion, and sequencing were used to verify successful cloning of truncated PE. Sodium dodecyl sulfate-polyacrylamide gel electrophoresis (SDS-PAGE) and western blot analyses indicated the presence of a ∼15-kDa recombinant protein. Serum IgG, IgG1, and IgG2a levels were significantly higher in the group immunized by recombinant truncated PE mixed with an alum adjuvant, compared to the non-vaccinated control group. Development of a strong bactericidal effect against NTHi was observed in the serum samples from immunized animals. Our findings suggest that recombinant truncated PE is a potential vaccine candidate for NTHi.
    Language English
    Publishing date 2017-09
    Publishing country England
    Document type Journal Article
    ZDB-ID 632772-2
    ISSN 1096-1208 ; 0882-4010
    ISSN (online) 1096-1208
    ISSN 0882-4010
    DOI 10.1016/j.micpath.2017.07.025
    Database MEDical Literature Analysis and Retrieval System OnLINE

    More links

    Kategorien

  3. Article ; Online: In silico design, cloning, expression and immunologic evaluation of ED fusion protein of NT H. influenzae.

    Behrouzi, Ava / Bouzari, Saeid / Oloomi, Mana / Fateh, Abolfazl / Vaziri, Farzam / Afrough, Parviz / Amanzadeh, Amir / Siadat, Seyed Davar

    Microbial pathogenesis

    2017  Volume 113, Page(s) 472–479

    Abstract: ... proteins are conserved membrane-specific lipoproteins among encapsulated and non-encapsulated H. influenza ...

    Abstract Infections due to nontypeable Haemophilus influenzae (NTHi) are important causes of child mortality throughout the world. Given the lack of effective vaccines for these strains and the spread and prevalence of these infections in the world, it is necessary to design novel vaccine candidates against these strains. D and E proteins are conserved membrane-specific lipoproteins among encapsulated and non-encapsulated H. influenza strains, which, according to the exposure surface and conservation degree between both strains, can be considered as vaccine candidates suitable for studies. This research was conducted to design a recombinant truncated fusion protein ED. Vaccination of BALB/c mice with recombinant truncated fusion protein ED showed high level of protective responses against NTHi. There were also strong responses of IgG and its subclasses (especially IgG1) as well as high titer levels of IL-4. A mixture of responses was observed considering IgG2a and INF-γ antibody titers, but the dominant response was toward Th2. According to the obtained results and the importance of humoral immunity in the immune system and vaccines production, it could be concluded that the produced recombinant construct can be used as a suitable vaccine candidate against NTHi or together with other carrier proteins.
    MeSH term(s) Animals ; Antibodies, Bacterial/blood ; Antibodies, Bacterial/immunology ; Bacterial Outer Membrane Proteins/genetics ; Bacterial Outer Membrane Proteins/immunology ; Bacterial Proteins/genetics ; Bacterial Proteins/immunology ; Carrier Proteins ; Cell Proliferation ; Computer Simulation ; DNA, Bacterial/genetics ; Disease Models, Animal ; Escherichia coli/genetics ; Female ; Gene Expression Regulation, Bacterial ; Genetic Vectors ; Haemophilus Infections/immunology ; Haemophilus Infections/prevention & control ; Haemophilus Vaccines/genetics ; Haemophilus Vaccines/immunology ; Haemophilus influenzae/genetics ; Haemophilus influenzae/immunology ; Immunity, Humoral ; Immunoglobulin G/blood ; Immunoglobulin G/immunology ; Interferon-gamma/metabolism ; Interleukin-4/metabolism ; Lipoproteins ; Macrophages/immunology ; Macrophages/microbiology ; Mice ; Mice, Inbred BALB C ; Recombinant Proteins/genetics ; Recombinant Proteins/immunology ; Spleen/metabolism ; Th2 Cells/immunology ; Vaccination
    Chemical Substances Antibodies, Bacterial ; Bacterial Outer Membrane Proteins ; Bacterial Proteins ; Carrier Proteins ; DNA, Bacterial ; Haemophilus Vaccines ; Immunoglobulin G ; Lipoproteins ; Recombinant Proteins ; Interleukin-4 (207137-56-2) ; Interferon-gamma (82115-62-6)
    Language English
    Publishing date 2017-12
    Publishing country England
    Document type Journal Article
    ZDB-ID 632772-2
    ISSN 1096-1208 ; 0882-4010
    ISSN (online) 1096-1208
    ISSN 0882-4010
    DOI 10.1016/j.micpath.2017.11.007
    Database MEDical Literature Analysis and Retrieval System OnLINE

    More links

    Kategorien

  4. Article ; Online: Relationship between histopathological status of the Helicobacter pylori infected patients and proteases of H. pylori in isolates carrying diverse virulence genotypes.

    Gharibi, Somayyeh / Falsafi, Tahereh / Alebouyeh, Masoud / Farzi, Nastaran / Vaziri, Farzam / Zali, Mohamad Reza

    Microbial pathogenesis

    2017  Volume 110, Page(s) 100–106

    Abstract: ... virulence factors of H. pylori, proteases may also be involved in its pathogenicity. In this study, relationship ... between proteolytic activity of H. pylori strains and histopathological changes of the stomach was ... investigated in the patients infected with strains carrying diverse virulence factors. H. pylori strains were ...

    Abstract Helicobacter pylori is the main cause of several gastroduodenal diseases in Humans. Among various virulence factors of H. pylori, proteases may also be involved in its pathogenicity. In this study, relationship between proteolytic activity of H. pylori strains and histopathological changes of the stomach was investigated in the patients infected with strains carrying diverse virulence factors. H. pylori strains were isolated from the biopsies of 116 patients who referred to hospital for their gastroduodenal disorders, in Tehran, Iran. Biopsies were sent to microbiology and pathology laboratories for further analysis. All the suspected grown colonies were characterized by both biochemical tests and polymerase chain reaction (PCR). Presence of seven protease genes, htrA, clpP, hp0169, hp1012, hp0382, hp1350 and hp1435, and distinct allelic variants of H. pylori virulence factors, cagA, vacA, iceA, babA2 and sabA, were analyzed in each strain. Protease activity of the strains was assessed using spectrophotometric assay. Furthermore, association between diversity in protease genes and virulence genes, protease activity, as well as pathological changes was estimated statistically. Proteases genes, htrA, clpP, hp0169, hp1012, hp0382, hp1350, hp1435, were detected among 100%, 100%, 98%, 98%, 98%, 98%, and 8% of fifty H. pylori strains isolated from the patients, respectively. Status of cagA, vacA s1, vacA s2, vacA m1, vacA m2, iceA1, iceA2, babA2 and sabA genes in isolates were 64%, 68%, 30%, 26%, 74%, 48%, 52%, 100%, and 96%, respectively. Predominant (84%) combined status for protease genes was: htrA/clpP/hp0169/hp1012/hp0382/hP1350/hp1435, while the prevalent combined status (16%) for virulence genes was: cagA+/vacA s1m2/iceA1
    Language English
    Publishing date 2017-09
    Publishing country England
    Document type Journal Article
    ZDB-ID 632772-2
    ISSN 1096-1208 ; 0882-4010
    ISSN (online) 1096-1208
    ISSN 0882-4010
    DOI 10.1016/j.micpath.2017.06.023
    Database MEDical Literature Analysis and Retrieval System OnLINE

    More links

    Kategorien

  5. Article: Recombinant truncated E protein as a new vaccine candidate against nontypeable H. influenzae: Its expression and immunogenic evaluation

    Behrouzi, Ava / Abolfazl Fateh / Atefeh Davoudi Vijeh Motlagh / Farzam Vaziri / Parviz Afrough / Saeid Bouzari / Seyed Davar Siadat

    Microbial pathogenesis. 2017 Sept., v. 110

    2017  

    Abstract: ... encapsulated H. influenzae. This is a small surface lipoprotein, consisting of only 160 amino acids, involved ... in the adhesion of H. influenzae to various types of epithelial cells.A 384-bp-long fragment from NTHi PE was ...

    Abstract Protein E (PE) is a conserved entity observed in both nontypeable Haemophilus influenzae (NTHi) and encapsulated H. influenzae. This is a small surface lipoprotein, consisting of only 160 amino acids, involved in the adhesion of H. influenzae to various types of epithelial cells.A 384-bp-long fragment from NTHi PE was cloned into the prokaryotic expression vector pBAD-gIIIA. The recombinant protein was expressed with arabinose and then purified by affinity purification on an Ni-NTA agarose matrix. BALB/c mice were immunized by subcutaneous injection with purified recombinant truncated PE mixed with an alum adjuvant. Serum antibody response and the functional activity of antibodies were evaluated by enzyme-linked immunosorbent assay (ELISA) and serum bactericidal assay (SBA), respectively.Colony PCR, double digestion, and sequencing were used to verify successful cloning of truncated PE. Sodium dodecyl sulfate-polyacrylamide gel electrophoresis (SDS-PAGE) and western blot analyses indicated the presence of a ∼15-kDa recombinant protein. Serum IgG, IgG1, and IgG2a levels were significantly higher in the group immunized by recombinant truncated PE mixed with an alum adjuvant, compared to the non-vaccinated control group. Development of a strong bactericidal effect against NTHi was observed in the serum samples from immunized animals. Our findings suggest that recombinant truncated PE is a potential vaccine candidate for NTHi.
    Keywords agarose ; alum ; amino acids ; antibacterial properties ; antibodies ; arabinose ; bacterial adhesion ; bacterial proteins ; blood serum ; encapsulation ; enzyme-linked immunosorbent assay ; epithelial cells ; epithelium ; Haemophilus influenzae ; immunoglobulin G ; lipoproteins ; mice ; polyacrylamide gel electrophoresis ; polymerase chain reaction ; recombinant proteins ; subcutaneous injection ; vaccine adjuvants ; vaccines ; Western blotting
    Language English
    Dates of publication 2017-09
    Size p. 431-438.
    Publishing place Elsevier Ltd
    Document type Article
    ZDB-ID 632772-2
    ISSN 1096-1208 ; 0882-4010
    ISSN (online) 1096-1208
    ISSN 0882-4010
    DOI 10.1016/j.micpath.2017.07.025
    Database NAL-Catalogue (AGRICOLA)

    More links

    Kategorien

  6. Article: In silico design, cloning, expression and immunologic evaluation of ED fusion protein of NT H. influenzae

    Behrouzi, Ava / Abolfazl Fateh / Amir Amanzadeh / Farzam Vaziri / Mana Oloomi / Parviz Afrough / Saeid Bouzari / Seyed Davar Siadat

    Microbial pathogenesis. 2017 Dec., v. 113

    2017  

    Abstract: ... proteins are conserved membrane-specific lipoproteins among encapsulated and non-encapsulated H. influenza ...

    Abstract Infections due to nontypeable Haemophilus influenzae (NTHi) are important causes of child mortality throughout the world. Given the lack of effective vaccines for these strains and the spread and prevalence of these infections in the world, it is necessary to design novel vaccine candidates against these strains. D and E proteins are conserved membrane-specific lipoproteins among encapsulated and non-encapsulated H. influenza strains, which, according to the exposure surface and conservation degree between both strains, can be considered as vaccine candidates suitable for studies. This research was conducted to design a recombinant truncated fusion protein ED. Vaccination of BALB/c mice with recombinant truncated fusion protein ED showed high level of protective responses against NTHi. There were also strong responses of IgG and its subclasses (especially IgG1) as well as high titer levels of IL-4. A mixture of responses was observed considering IgG2a and INF-γ antibody titers, but the dominant response was toward Th2. According to the obtained results and the importance of humoral immunity in the immune system and vaccines production, it could be concluded that the produced recombinant construct can be used as a suitable vaccine candidate against NTHi or together with other carrier proteins.
    Keywords antibodies ; children ; encapsulation ; Haemophilus influenzae ; humoral immunity ; immune system ; immunoglobulin G ; influenza ; interferon-gamma ; interleukin-4 ; lipoproteins ; mice ; mortality ; transport proteins ; vaccination ; vaccines
    Language English
    Dates of publication 2017-12
    Size p. 472-479.
    Publishing place Elsevier Ltd
    Document type Article
    ZDB-ID 632772-2
    ISSN 1096-1208 ; 0882-4010
    ISSN (online) 1096-1208
    ISSN 0882-4010
    DOI 10.1016/j.micpath.2017.11.007
    Database NAL-Catalogue (AGRICOLA)

    More links

    Kategorien

  7. Article: Relationship between histopathological status of the Helicobacter pylori infected patients and proteases of H. pylori in isolates carrying diverse virulence genotypes

    Gharibi, Somayyeh / Farzam Vaziri / Masoud Alebouyeh / Mohamad Reza Zali / Nastaran Farzi / Tahereh Falsafi

    Microbial pathogenesis. 2017 Sept., v. 110

    2017  

    Abstract: ... virulence factors of H. pylori, proteases may also be involved in its pathogenicity. In this study, relationship ... between proteolytic activity of H. pylori strains and histopathological changes of the stomach was ... investigated in the patients infected with strains carrying diverse virulence factors. H. pylori strains were ...

    Abstract Helicobacter pylori is the main cause of several gastroduodenal diseases in Humans. Among various virulence factors of H. pylori, proteases may also be involved in its pathogenicity. In this study, relationship between proteolytic activity of H. pylori strains and histopathological changes of the stomach was investigated in the patients infected with strains carrying diverse virulence factors. H. pylori strains were isolated from the biopsies of 116 patients who referred to hospital for their gastroduodenal disorders, in Tehran, Iran. Biopsies were sent to microbiology and pathology laboratories for further analysis. All the suspected grown colonies were characterized by both biochemical tests and polymerase chain reaction (PCR). Presence of seven protease genes, htrA, clpP, hp0169, hp1012, hp0382, hp1350 and hp1435, and distinct allelic variants of H. pylori virulence factors, cagA, vacA, iceA, babA2 and sabA, were analyzed in each strain. Protease activity of the strains was assessed using spectrophotometric assay. Furthermore, association between diversity in protease genes and virulence genes, protease activity, as well as pathological changes was estimated statistically. Proteases genes, htrA, clpP, hp0169, hp1012, hp0382, hp1350, hp1435, were detected among 100%, 100%, 98%, 98%, 98%, 98%, and 8% of fifty H. pylori strains isolated from the patients, respectively. Status of cagA, vacA s1, vacA s2, vacA m1, vacA m2, iceA1, iceA2, babA2 and sabA genes in isolates were 64%, 68%, 30%, 26%, 74%, 48%, 52%, 100%, and 96%, respectively. Predominant (84%) combined status for protease genes was: htrA/clpP/hp0169/hp1012/hp0382/hP1350/hp1435, while the prevalent combined status (16%) for virulence genes was: cagA+/vacA s1m2/iceA1+/sabA+/babA2+. Although most of the strains (91.4%) presented moderate protease activity in vitro, lowest activity was measured in strains isolated from the patients with chronic gastritis (4.25%). Present study provide the new data on diversity of protease genes in H. pylori, as well as the proteolytic activity of these genes in H. pylori strains from the sick patients. Presence of significant association between lower protease activity of the strains and mildness of the pathological changes propose involvement of these proteases in the pathogenesis of H. pylori in vivo.
    Keywords biopsy ; enzyme activity ; gastritis ; genes ; genotype ; Helicobacter pylori ; histopathology ; hospitals ; humans ; microbiology ; pathogenesis ; patients ; polymerase chain reaction ; proteinases ; proteolysis ; stomach ; virulence ; Iran
    Language English
    Dates of publication 2017-09
    Size p. 100-106.
    Publishing place Elsevier Ltd
    Document type Article
    ZDB-ID 632772-2
    ISSN 1096-1208 ; 0882-4010
    ISSN (online) 1096-1208
    ISSN 0882-4010
    DOI 10.1016/j.micpath.2017.06.023
    Database NAL-Catalogue (AGRICOLA)

    More links

    Kategorien

  8. Article: Factor H binds to washed human platelets.

    Vaziri-Sani, F / Hellwage, J / Zipfel, P F / Sjöholm, A G / Iancu, R / Karpman, D

    Journal of thrombosis and haemostasis : JTH

    2003  Volume 3, Issue 1, Page(s) 154–162

    Abstract: Background: Factor H regulates the alternative pathway of complement. The protein has three ... with thrombospondin-1. Factor H mutations at the C-terminus are associated with atypical hemolytic uremic syndrome ... a condition in which platelets are consumed. Objectives The aim of this study was to investigate if factor H ...

    Abstract Background: Factor H regulates the alternative pathway of complement. The protein has three heparin-binding sites, is synthesized primarily in the liver and copurifies from platelets with thrombospondin-1. Factor H mutations at the C-terminus are associated with atypical hemolytic uremic syndrome, a condition in which platelets are consumed. Objectives The aim of this study was to investigate if factor H interacts with platelets.
    Methods: Binding of factor H, recombinant C- or N-terminus constructs and a C-terminus mutant to washed (plasma and complement-free) platelets was analyzed by flow cytometry. Binding of factor H and constructs to thrombospondin-1 was measured by surface plasmon resonance.
    Results: Factor H bound to platelets in a dose-dependent manner. The major binding site was localized to the C-terminus. The interaction was partially blocked by heparin. Inhibition with anti-GPIIb/IIIa, or with fibrinogen, suggested that the platelet GPIIb/IIIa receptor is involved in factor H binding. Factor H binds to thrombospondin-1. Addition of thrombospondin-1 increased factor H binding to platelets. Factor H mutated at the C-terminus also bound to platelets, albeit to a significantly lesser degree.
    Conclusions: This study reports a novel property of factor H, i.e. binding to platelets, either directly via the GPIIb/IIIa receptor or indirectly via thrombospondin-1, in the absence of complement. Binding to platelets was mostly mediated by the C-terminal region of factor H and factor H mutated at the C-terminus exhibited reduced binding.
    MeSH term(s) Binding Sites ; Blood Platelets/cytology ; Blood Platelets/metabolism ; Complement Factor H/chemistry ; Complement Factor H/metabolism ; Complement System Proteins/chemistry ; Dose-Response Relationship, Drug ; Female ; Flow Cytometry ; Hemolytic-Uremic Syndrome/genetics ; Heparin/chemistry ; Humans ; Kinetics ; Liver/metabolism ; Male ; Mutation ; Platelet Glycoprotein GPIIb-IIIa Complex/chemistry ; Protein Binding ; Protein Structure, Tertiary ; Recombinant Proteins/chemistry ; Surface Plasmon Resonance ; Thrombospondin 1/metabolism ; Time Factors
    Chemical Substances CFH protein, human ; Platelet Glycoprotein GPIIb-IIIa Complex ; Recombinant Proteins ; Thrombospondin 1 ; Complement Factor H (80295-65-4) ; Heparin (9005-49-6) ; Complement System Proteins (9007-36-7)
    Language English
    Publishing date 2003-07-14
    Publishing country England
    Document type Journal Article ; Research Support, Non-U.S. Gov't
    ZDB-ID 2112661-6
    ISSN 1538-7836 ; 1538-7933
    ISSN (online) 1538-7836
    ISSN 1538-7933
    DOI 10.1111/j.1538-7836.2004.01010.x
    Database MEDical Literature Analysis and Retrieval System OnLINE

    More links

    Kategorien

  9. Article ; Online: Interval Colorectal Cancer in Inflammatory Bowel Disease: A Review.

    Da Cunha, Teresa / Vaziri, Haleh

    Journal of clinical gastroenterology

    2024  Volume 58, Issue 1, Page(s) 1–11

    Abstract: Inflammatory bowel disease (IBD) increases the risk of dysplasia and colorectal cancer (CRC). Moreover, colitis-associated CRC is responsible for a disproportionate number of CRC-related mortality. For this reason, societies recommend screening and ... ...

    Abstract Inflammatory bowel disease (IBD) increases the risk of dysplasia and colorectal cancer (CRC). Moreover, colitis-associated CRC is responsible for a disproportionate number of CRC-related mortality. For this reason, societies recommend screening and surveillance colonoscopy as the standard of care for patients with ulcerative colitis and Crohn's colitis. Nonetheless, interval cancer defined as CRC detected within the appropriate surveillance interval might still occur despite following guideline recommendations. Even though there is limited data on risk factors associated with interval CRC in IBD, patient and disease-associated factors and technical aspects of the surveillance might play a role. This review aims to provide information on the epidemiology of interval CRC in IBD, the factors that might be associated with its occurrence, and the challenges of CRC screening and dysplasia management in patients with IBD.
    MeSH term(s) Humans ; Inflammatory Bowel Diseases/complications ; Inflammatory Bowel Diseases/epidemiology ; Colitis, Ulcerative/complications ; Colitis, Ulcerative/diagnosis ; Colitis, Ulcerative/epidemiology ; Crohn Disease/complications ; Colorectal Neoplasms/diagnosis ; Colorectal Neoplasms/epidemiology ; Colorectal Neoplasms/etiology ; Colonoscopy ; Risk Factors ; Hyperplasia/complications
    Language English
    Publishing date 2024-01-01
    Publishing country United States
    Document type Review ; Journal Article
    ZDB-ID 448460-5
    ISSN 1539-2031 ; 0192-0790
    ISSN (online) 1539-2031
    ISSN 0192-0790
    DOI 10.1097/MCG.0000000000001901
    Database MEDical Literature Analysis and Retrieval System OnLINE

    More links

    Kategorien

  10. Article: Phenotypic expression of factor H mutations in patients with atypical hemolytic uremic syndrome.

    Vaziri-Sani, F / Holmberg, L / Sjöholm, A G / Kristoffersson, A-C / Manea, M / Frémeaux-Bacchi, V / Fehrman-Ekholm, I / Raafat, R / Karpman, D

    Kidney international

    2006  Volume 69, Issue 6, Page(s) 981–988

    Abstract: We investigated the phenotypic expression of factor H mutations in two patients with atypical ... hemolytic uremic syndrome (HUS). Factor H in serum was assayed by rocket immunoelectrophoresis, immunoblotting, and double ... 10 of factor H was identified in an adult man who first developed ...

    Abstract We investigated the phenotypic expression of factor H mutations in two patients with atypical hemolytic uremic syndrome (HUS). Factor H in serum was assayed by rocket immunoelectrophoresis, immunoblotting, and double immunodiffusion and in tissue by immunohistochemistry. Functional activity was analyzed by hemolysis of sheep erythrocytes and binding to endothelial cells. A homozygous mutation in complement control protein (CCP) domain 10 of factor H was identified in an adult man who first developed membranoproliferative glomerulonephritis and later HUS. C3 levels were very low. The patient had undetectable factor H levels in serum and a weak factor H 150 kDa band. Double immunodiffusion showed partial antigenic identity with factor H in normal serum owing to the presence of factor H-like protein 1. Strong specific labeling for factor H was detected in glomerular endothelium, mesangium and in glomerular and tubular epithelium as well as in bone marrow cells. A heterozygous mutation in CCP 20 of factor H was found in a girl with HUS. C3 levels were moderately decreased at onset. Factor H levels were normal and a normal 150 kDa band was present. Double immunodiffusion showed antigenic identity with normal factor H. Factor H labeling was minimal in the renal cortex. Factor H dysfunction was demonstrated by increased sheep erythrocyte hemolysis and decreased binding to endothelial cells. In summary, two different factor H mutations associated with HUS were examined: in one, factor H accumulated in cells, and in the other, membrane binding was reduced.
    MeSH term(s) Animals ; Bone Marrow Cells/chemistry ; Child ; Complement C3/analysis ; Complement Factor H/analysis ; Complement Factor H/genetics ; Complement Factor H/physiology ; Endothelium/chemistry ; Endothelium/pathology ; Endothelium/physiopathology ; Erythrocytes/pathology ; Female ; Flow Cytometry ; Gene Expression ; Glomerulonephritis, Membranoproliferative/complications ; Glomerulonephritis, Membranoproliferative/genetics ; Hemolysis/genetics ; Hemolysis/physiology ; Hemolytic-Uremic Syndrome/blood ; Hemolytic-Uremic Syndrome/etiology ; Hemolytic-Uremic Syndrome/genetics ; Humans ; Immunodiffusion ; Immunoelectrophoresis ; Immunohistochemistry ; Kidney Cortex/chemistry ; Male ; Mesangial Cells/chemistry ; Middle Aged ; Mutation ; Phenotype ; Protein Binding/genetics ; Protein Binding/physiology ; Protein Structure, Tertiary/genetics ; Sheep
    Chemical Substances Complement C3 ; Complement Factor H (80295-65-4)
    Language English
    Publishing date 2006-03
    Publishing country United States
    Document type Case Reports ; Journal Article ; Research Support, Non-U.S. Gov't
    ZDB-ID 120573-0
    ISSN 1523-1755 ; 0085-2538
    ISSN (online) 1523-1755
    ISSN 0085-2538
    DOI 10.1038/sj.ki.5000155
    Database MEDical Literature Analysis and Retrieval System OnLINE

    More links

    Kategorien

To top