Wynik wyszukiwania w bazie Polska Bibliografia Lekarska GBL

Zapytanie: NOOR
Liczba odnalezionych rekordów: 4



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Tytuł oryginału: The immunophenotypic and immunogenotypic B-cell differentiation arrest in bone marrow of RAG-deficient SCID patients corresponds to residual recombination activities of mutated RAG proteins.
Autorzy: Noordzij Jeroen G., Bruin-Versteeg Sandra de, Verkaik Nicole S., Vossen Jaak M. J. J., Groot Ronald de, Bernatowska Ewa, Langerak Anton W., Gent Dik C. van, Dongen Jacques J. M. van
Źródło: Blood 2002: 100 (6) s.2145-2152, il., tab., bibliogr. 45 poz.
Sygnatura GBL: 301,770

Hasła klasyfikacyjne GBL:
  • genetyka
  • immunologia

    Typ dokumentu:
  • praca kliniczna
  • praca opublikowana za granicą
  • tytuł obcojęzyczny

    Wskaźnik treści:
  • ludzie

    Streszczenie angielskie: The protein products of the recombination activating genes (RAG1 and RAG2) initiate the formation of immunoglobulin (lg) and T-cell receptors, whichare essential for B- and t-cell development, respectively. Mutations in the RAG genes result in severe combined immunodeficiency disease (SCID), generally characterized by the absence of mature B and T lymphocytes, but presence of natural killer (NK) cells. Biochemically, mutations in the RAG genes result either in nonfunctional proteins or in proteins with partial recombination activity. The mutated RAG genes of 9 patients from 7 families were analyzed for their recombination activity using extrachromosomal recombination substrates, rearrangment of endogenous Ig loci in RAG gene-transfected nonlymphoid cells, or the presence of Ig gene rearrangements in bone marrow (BM). Recombination activity was virtually absent in all 6 patients with mutations in the RAG core domains, but partial activity was present in the other 3 RAG-deficient patients, 2 of them having Omenn syndrome with oligocional T lymphocytes. Using 4-color flow cytometry, we could define the exact stage at which B-cell differentiation was arrested in the BM of 5 RAG-deficient SCID patients. In 4 of 5 patients, the absence of recombination activity was associated with a complete B-cell differentiation arrest at the transition from cytoplasmic (Cy) Igć- pre -B-I cells to Cylgć+ pre -B-II cells. However, the fifth patient showed low frequencies of precursor B cells with Cylgć and surface membrane IgM...

    2/4

    Tytuł oryginału: Advances in immunocytochemistry.
    Autorzy: Noorden Susan Van
    Źródło: Folia Histochem. Cytobiol. 2002: 40 (2) s.121-124, il., bibliogr. 20 poz. - 37 Sympozjum Polskiego Towarzystwa Histochemików i Cytochemików Kraków 02-05.09. 2001
    Sygnatura GBL: 304,846

    Hasła klasyfikacyjne GBL:
  • immunologia

    Typ dokumentu:
  • praca związana ze zjazdem
  • tytuł obcojęzyczny

    Wskaźnik treści:
  • ludzie
  • zwierzęta
  • myszy

    Streszczenie angielskie: Recent advances in immunohisto/cytochemical methods have been directed towards new fluorescent labaels, and to increasing sensitivity and improving methods for multiple immunostaining. The newestfluorescent dyes come in many colours, are much mosre stable than fluorescein isothiocyanate and give brighter fluorescence. The greatest impact on sensitivity comes from heat-induced antigen retreival on paraffin sections. Biotinylasted tyramide can also increase enormously the amount of label on a preparation. The immunogold method with silver enhancement has been improved by nanogold, which is smaller than colloidal gold, attracts more silfer and increases the sensitivity even further. The difficulties of multiple labelling with two or more primary antibodies raised in the same species due to cross-binding between reagents have largely been overcome. There are new methods of blocking spare reactive sites on the first reaction by strong heat, e. g. from microwaving in buffer. Alternatively, one of the antibodis can be directrly biotinylated very easily with a new commercially available kit.

    3/4

    Tytuł oryginału: A paradigm for quantifying ventricular contraction.
    Autorzy: Palladino Joseph L., Noordergraaf Abraham
    Źródło: Cell. Mol. Biol. Lett. 2002: 7 (2) s.331-335, il., tab., bibliogr. 4 poz. - Międzynarodowa konferencja pt. Nauki przyrodnicze Gozd Martuljek 22-26.09. 2001 - Cz. 1 mater. konferencyjnych zamieszczono w nr 1/2002
    Sygnatura GBL: 306,513

    Hasła klasyfikacyjne GBL:
  • kardiologia

    Typ dokumentu:
  • praca związana ze zjazdem
  • praca przeglądowa
  • tytuł obcojęzyczny

    Streszczenie angielskie: The left ventricle may be described as a time, volume and flow dependent pressure generator. First, isovolumic pressure is measured at various and-diastolic volumes. Next, pressure is adjusted to account for small changes accompanying ejection, denoted the ejection effect. The resulting analytical function can describe pressure generation and ventricular outflow of the ventricle under a wide range of contractile and vascular conditions. This paradigm is unique in separating isvolumic from ejecting ventricular properties, as well as ventricular from vascular conditions.

    4/4

    Tytuł oryginału: Superoxide dismutase - applications and relevance to human diseases.
    Autorzy: Noor Rana, Mittal Sonali, Iqbal Jawaid
    Źródło: Med. Sci. Monitor 2002: 8 (9) s.RA210-RA215, bibliogr. 63 poz.
    Sygnatura GBL: 313,278

    Typ dokumentu:
  • tytuł obcojęzyczny

    Streszczenie angielskie: Reactive oxygen species, such as superoxide radicals, are thought to underlie the pathogenesis of various diseases. Almost 3 to 10 p.c. of the oxygen utilized by tissues is converted to its reactive intermediates, which impair the functioning of cells and tissues. Superoxide dismutase (SOD) catalyzes the conversion of single electron reduced species of molecular oxygen to hydrogen peroxide and oxygen. There are several classes of SOD that differ in their metal binding ability, distribution in different cell compartments, and sensitivity to various reagents. Among these, Cu, Zn superoxide dismutase (SOD1) is widely distributed and comprises 90 p.c. of the total SOD. This ubiquitous enzyme, which requires Cu and Zn for its activity, has great physiological significance and therpaeutic potential. The present review describes the role of SODs, especially Cu, Zn SOD, in several diseases, such as familal amyotrophic lateral sclerosis (FAL), Parkinson's disease, Alzheimer's disease, dengue fever, cancer, Down's syndrome, cataract, and several neurological disorders. Mutations in the SOD1 gene cause of familal form of amyotrophic lateral sclerosis. The mechanism by which mutant SOD1 causes the degeneration of motor neurons is not well understood. Transgenic mice expressing multiple copies of FALS-mutant SOD1s develop and ALS-like motor neuron disease. Vacuolar degeneration of mitochondria has been identified as the main pathological feature associated with motor neuron death and paralysis in several lines of FALS-SOD1 mice. Various observations and conclusions linking mutant SOD1 and FALS are discussed in this review in detail.

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