Abstract:
:Abstract Fanconi anemia (FA) is a rare cancer-prone genetic disorder characterized by progressive bone marrow failure, chromosomal instability and redox abnormalities. There is much biochemical and genetic data, which strongly suggest that FA cells experience increased oxidative stress. The present study was designed to elucidate if differences in oxidant state exist between control, idiopathic bone marrow failure (idBMF) and FA cells, and to analyze oxidant state of cells in FA heterozygous carriers as well. The results of the present study confirm an in vivo prooxidant state of FA cells and clearly indicate that FA patients can be distinguished from idBMF patients based on the oxidant state of cells. Female carriers of FA mutation also exhibited hallmarks of an in vivo prooxidant state behaving in a similar manner as FA patients. On the other hand, the oxidant state of cells in FA male carriers and idBMF families failed to show any significant difference vs. controls. We demonstrate that the altered oxidant state influences susceptibility of cells to apoptosis in both FA patients and female carriers. The results highlight the need for further research of the possible role of mitochondrial inheritance in the pathogenesis of FA.
journal_name
Biol Chemjournal_title
Biological chemistryauthors
Petrovic S,Leskovac A,Kotur-Stevuljevic J,Joksic J,Guc-Scekic M,Vujic D,Joksic Gdoi
10.1515/BC.2011.064subject
Has Abstractpub_date
2011-07-01 00:00:00pages
625-32issue
7eissn
1431-6730issn
1437-4315journal_volume
392pub_type
杂志文章abstract::Adenoviral DNA was examined within capsids by dimethyl sulfate footprinting. Protein-DNA interactions were visualized through ligation-mediated PCR (LM-PCR). Signals for protein binding were found adjacent to both inverted terminal repeats (ITR). There were no indications of close protein binding at several other loci...
journal_title:Biological chemistry
pub_type: 杂志文章
doi:10.1515/BC.2001.169
更新日期:2001-09-01 00:00:00
abstract::M.EcoRV is an alpha-adenine DNA methyltransferase. According to structure predictions, the enzyme consists of a catalytic domain, which has a structure similar to all other DNA-methyltransferases, and a smaller DNA-recognition domain. We have investigated this enzyme by random mutagenesis, using error-prone PCR, follo...
journal_title:Biological chemistry
pub_type: 杂志文章
doi:10.1515/bchm.1998.379.4-5.475
更新日期:1998-04-01 00:00:00
abstract::The surface topography of red blood cells (RBCs) was investigated under near-physiological conditions using atomic force microscopy (AFM). An immobilization protocol was established where RBCs are coupled via molecular bonds of the membrane glycoproteins to wheat germ agglutinin (WGA), which is covalently and flexibly...
journal_title:Biological chemistry
pub_type: 杂志文章
doi:10.1515/BC.2004.124
更新日期:2004-10-01 00:00:00
abstract::Abstract Few genes are known to be involved in renal cell carcinoma (RCC) development and progression. The cell-specific transcription factor hepatocyte nuclear factor 4 alpha (HNF4 alpha) is down-regulated in RCC and we have shown that HNF4 alpha inhibits cell proliferation in the embryonic kidney cell line HEK293. T...
journal_title:Biological chemistry
pub_type: 杂志文章
doi:10.1515/BC.2008.011
更新日期:2008-02-01 00:00:00
abstract::Dipeptidyl peptidase-IV has been demonstrated to play a role in cancer biology by many authors. Since then, additional proteins possessing similar enzymatic activity have been described and their role in cancerogenesis has been hypothesized. To assess the complexity of these 'Dipeptidyl peptidase-IV activity and/or st...
journal_title:Biological chemistry
pub_type: 杂志文章
doi:10.1515/BC.2004.067
更新日期:2004-06-01 00:00:00
abstract::The fundamental cellular role and molecular interactions of annexins in vesicle trafficking and membrane remodeling remain to be further clarified in order to better understand and exploit their contributions to health and disease. We focused on distinctive features of atypical annexins from all domains of life using ...
journal_title:Biological chemistry
pub_type: 杂志文章
doi:10.1515/hsz-2016-0273
更新日期:2017-06-27 00:00:00
abstract::An inert polysaccharide scaffold identified as a 5-15% component of prion rods (PrP 27-30) is unambiguously distinguishable from the N-glycosyl groups and the GPI anchor of PrP, and consists predominantly of 1,4-linked glucose with some branching via 1,4,6-linked glucose. We show that this polysaccharide scaffold is a...
journal_title:Biological chemistry
pub_type: 杂志文章
doi:10.1515/BC.2005.131
更新日期:2005-11-01 00:00:00
abstract::Hypoxia-inducible transcription factor (HIF), an αβ dimer, is the key inducer of hypoxia-responsive genes that operate both during normal development and pathological processes in association with decreased oxygen availability. The products of HIF target genes function in, e.g., hematopoiesis, angiogenesis, iron trans...
journal_title:Biological chemistry
pub_type: 杂志文章,评审
doi:10.1515/hsz-2012-0328
更新日期:2013-04-01 00:00:00
abstract::Fanconi anemia is a fatal, hereditary chromosome instability syndrome of early childhood with progressive pancytopenia and cancer-proneness. Hypersensitivity to alkylating agents points to DNA repair inefficiency. Excess reactive oxygen intermediates and hypersensitivity to oxygen, all features of Fanconi anemia cells...
journal_title:Biological chemistry
pub_type: 杂志文章
doi:10.1515/BC.2008.151
更新日期:2008-10-01 00:00:00
abstract::Mitochondria contain elaborate machineries for the import of precursor proteins from the cytosol. The translocase of the outer mitochondrial membrane (TOM) performs the initial import of precursor proteins and transfers the precursors to downstream translocases, including the presequence translocase and the carrier tr...
journal_title:Biological chemistry
pub_type: 杂志文章,评审
doi:10.1515/hsz-2016-0145
更新日期:2016-11-01 00:00:00
abstract::Glutaminyl cyclases (QC) catalyze the intramolecular cyclization of N-terminal glutamine residues of peptides and proteins. For a comparison of the substrate specificity of human and papaya QC enzymes, a novel continuous assay was established by adapting an existing discontinuous method. Specificity constants (kcat/Km...
journal_title:Biological chemistry
pub_type: 杂志文章
doi:10.1515/BC.2003.175
更新日期:2003-12-01 00:00:00
abstract::Abstract The R- and K-gingipain proteases of Porphyromonas gingivalis are involved in proteolysis of haemoglobin from which the defensive dimeric haem pigment is formed. Whilst oxyhaemoglobin is refractory towards K-gingipain, methaemoglobin is rapidly degraded. Ligation of methaemoglobin with N3-, which effectively b...
journal_title:Biological chemistry
pub_type: 杂志文章
doi:10.1515/BC.2008.140
更新日期:2008-09-01 00:00:00
abstract::Low NO concentrations synthesized by constitutively expressed NO synthases act on several signaling pathways activating transcription factors (TF), such as NF-kappaB or AP-1, and thereby influence gene expression. In contrast, during inflammatory reactions the inducible NO synthase produces NO for prolonged periods of...
journal_title:Biological chemistry
pub_type: 杂志文章,评审
doi:10.1515/BC.2003.153
更新日期:2003-10-01 00:00:00
abstract::Cytokinins are essential plant hormones that regulate numerous physiological processes. Recently, a protein was identified in mung bean (Vigna radiata) and characterized as a cytokinin-specific binding protein (VrCSBP). Fluorescence correlation spectroscopy was used to investigate the interaction between VrCSBP and it...
journal_title:Biological chemistry
pub_type: 杂志文章
doi:10.1515/BC.2010.005
更新日期:2010-01-01 00:00:00
abstract::Myocardial zinc dyshomeostasis is associated with caspase-3 activation, ErbB2 degradation and apoptosis during hypoxia/reoxygenation. Zinc pyrithione replenishes intracellular zinc, suppresses caspase-3, augments ErbB2 levels and improves cell survival. We hypothesize that zinc is capable of modulating redox and endop...
journal_title:Biological chemistry
pub_type: 杂志文章
doi:10.1515/hsz-2020-0167
更新日期:2020-10-25 00:00:00
abstract::Interleukin-13 (IL-13) is a T-cell-derived pleiotropic cytokine of particular medical importance because of its critical role in the development of allergic asthma. The effects of IL-13 on its target cells are mediated through a dimeric transmembrane receptor (IL-13R), which shares the IL-4Ralpha subunit with the IL-4...
journal_title:Biological chemistry
pub_type: 杂志文章
doi:10.1515/BC.2007.036
更新日期:2007-03-01 00:00:00
abstract::In mammalian cells, glucosylceramide (GlcCer), the simplest glycosphingolipid, is hydrolyzed by the lysosomal enzyme acid beta-glucosidase (GlcCerase). In the human metabolic disorder Gaucher disease, GlcCerase activity is significantly decreased owing to one of approximately 200 mutations in the GlcCerase gene. The m...
journal_title:Biological chemistry
pub_type: 杂志文章,评审
doi:10.1515/BC.2008.163
更新日期:2008-11-01 00:00:00
abstract::A large number of prostate cancer (PCa) patients receive treatment without significant benefits, strengthening the need for accurate prognosis, which can be supported by the study of miRNAs. In silico specificity analysis was performed for the identification of miRNAs able to regulate KLK2 and KLK4 expression. Total R...
journal_title:Biological chemistry
pub_type: 杂志文章
doi:10.1515/hsz-2014-0150
更新日期:2014-09-01 00:00:00
abstract::It is not rare that controversial indications about the presence or the expression level of multidrug-resistant (MDR) proteins come out from different laboratories upon examination of identical tumor specimens. Distinct aspects, including the use of weakly discriminating monoclonal antibodies (MAbs) and/or unsuitable ...
journal_title:Biological chemistry
pub_type: 杂志文章
doi:10.1515/bchm.1997.378.6.503
更新日期:1997-06-01 00:00:00
abstract::The HIRIP3 protein had been identified from its interaction with the HIRA histone chaperone. Experiments using anti-peptide antisera indicated that this 556-aa protein is nuclear throughout the cell cycle and excluded from condensed chromatin during mitosis. Based on its electrophoretic migration and sensitivity to ph...
journal_title:Biological chemistry
pub_type: 杂志文章
doi:10.1515/BC.2007.045
更新日期:2007-04-01 00:00:00
abstract::Haptoglobin (Hpt) is known to capture circulating free hemoglobin (Hb) and bind apolipoprotein (Apo) A-I or E. Here, we report that Hb can be tightly bound by most of Hpt molecules (TB-Hpt, 80%), whereas loosely bound by a minor part of them (LB-Hpt, 20%). LB-Hpt amount was significantly increased (over 60%) in patien...
journal_title:Biological chemistry
pub_type: 杂志文章
doi:10.1515/BC.2011.033
更新日期:2011-04-01 00:00:00
abstract::The BCL2 family of genes (B-cell CLL/lymphoma 2; Bcl-2) plays a pivotal role in the highly regulated process of apoptosis. We have recently cloned a newly identified member of this family, BCL2L12, which was found to be differentially expressed in many tumors. It is known that topotecan and methotrexate act through in...
journal_title:Biological chemistry
pub_type: 杂志文章
doi:10.1515/BC.2006.203
更新日期:2006-12-01 00:00:00
abstract::A novel antifungal protein, designated chrysancorin, was isolated from seeds of Chrysanthemum coronarium var. spatiosum with a procedure involving ion exchange chromatography on DEAE-cellulose, affinity chromatography on Affi-gel blue resin, ion exchange chromatography on SP-Sepharose and FPLC-gel filtration on Superd...
journal_title:Biological chemistry
pub_type: 杂志文章
doi:10.1515/BC.2001.118
更新日期:2001-06-01 00:00:00
abstract::Many aspects of the RNA maturation leave traces in RNA sequencing data in the form of deviations from the reference genomic DNA. This includes, in particular, genomically non-encoded nucleotides and chemical modifications. The latter leave their signatures in the form of mismatches and conspicuous patterns of sequenci...
journal_title:Biological chemistry
pub_type: 杂志文章
doi:10.1515/BC.2011.043
更新日期:2011-04-01 00:00:00
abstract::The physiological function of the GPI-anchored ectoenzyme aminopeptidase P (APP) is still elusive. Most researchers suppose that this enzyme inactivates biologically active peptides like bradykinin, neuropeptide tyrosine (NPY) and others (Vanhoof et al., 1995). We demonstrate by immunohistology with a specific antibod...
journal_title:Biological chemistry
pub_type: 杂志文章
doi:10.1515/bchm.1998.379.6.705
更新日期:1998-06-01 00:00:00
abstract::The lysosomal cysteine proteinase cathepsin B has been implicated in the progression of various human tumors including ovarian cancer. Included in this study were 63 patients with epithelial ovarian carcinoma. Follow-up information (median follow-up period 7 years) was available for all patients, among whom 42 (66.7%)...
journal_title:Biological chemistry
pub_type: 杂志文章
doi:10.1515/BC.2002.146
更新日期:2002-07-01 00:00:00
abstract::There is increasing evidence that apoptosis and necrosis represent only two of several possible ways for cells to die. These two types of demise can occur simultaneously in tissues or cell cultures exposed to the same stimulus, and often local metabolic conditions and the intensity of the same initial insult decide th...
journal_title:Biological chemistry
pub_type: 杂志文章,评审
doi:10.1515/BC.1999.129
更新日期:1999-09-01 00:00:00
abstract::Bauhinia ungulata factor Xa inhibitor (BuXI) inactivates factor Xa and LOPAP, a prothrombin activator proteinase isolated from the venom of Lonomia obliqua caterpillar bristles. The reactive site of the enzyme-inhibitor interaction was explored to design specific substrates for both enzymes. Methionine is crucial for ...
journal_title:Biological chemistry
pub_type: 杂志文章
doi:10.1515/BC.2003.055
更新日期:2003-03-01 00:00:00
abstract::Nanomolar concentrations of human amylin promote death of RINm5F cells in a time- and concentrationdependent manner. Morphological changes of chromatin integrity suggest that cells are predominantly undergoing apoptosis. Human amylin induces significant activation of caspase-3 and strong and sustained phosphorylation ...
journal_title:Biological chemistry
pub_type: 杂志文章
doi:10.1515/BC.2002.196
更新日期:2002-11-01 00:00:00
abstract::Five moeA mutants were generated by replacing some conserved amino acids of MoeA by site-directed mutagenesis. The mutants were assayed for the ability to restore in vivo nitrate reductase activity of the moeA mutant Escherichia coli JRG97 and in vitro Neurospora crassa nit-1 nitrate reductase activity. The replacemen...
journal_title:Biological chemistry
pub_type: 杂志文章
doi:10.1515/BC.2002.034
更新日期:2002-02-01 00:00:00