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41.
Fabrizio Grandi Marcia M Colodel Lidianne N Monteiro João Rafael VP Leão Noeme S Rocha 《BMC veterinary research》2010,6(1):45
Backgroud
Extramedullary hematopoiesis (EMH) is defined as the presence of hematopoietic stem cells such as erythroid and myeloid lineage plus megakaryocytes in extramedullary sites like liver, spleen and lymph nodes and is usually associated with either bone marrow or hematological disorders. Mammary EMH is a rare condition either in human and veterinary medicine and can be associated with benign mixed mammary tumors, similarly to that described in this case. 相似文献42.
43.
The F0I protein of apparent Mr 27,000, previously characterized [(1988) Eur. J. Biochem. 173, 1-8] as a genuine component of bovine heart F0, has been sequenced and shown to be identical with the nucleus encoded 24,668 Da protein characterized earlier [(1987) J. Mol. Biol. 197, 89-100]. It is directly shown by proteolytic cleavage and reconstitution experiments that this protein, denoted here as PVP from the single-letter codes of the last three residues of the N-terminus, is involved in proton conduction by F0 and in its sensitivity to oligomycin. 相似文献
44.
Philippe Labrune Anne Myara Michèle Hadchouel Flavio Ronchi Olivier Bernard François Trivin Namita Roy Chowdhury Jayanta Roy Chowdhury Arnold Munnich Michel Odièvre 《Human genetics》1994,94(6):693-697
Crigler-Najjar syndrome type I (CN-I) is an autosomal recessive condition characterized by severe unconjugated hyperbilirubinemia caused by the lack of bilirubin-UDP-glucuronosyltransferase (B-UGT) activity in the liver. Two B-UGTs are coded for by a gene complex (UGT1) that maps to chromosome 2q37 and that also encodes two phenol-UDP-glucuronosyltransferases. Here, we report eleven mutations (including nine novel mutations) of the B-UGT1 gene in a large series of 14 unrelated CN-I children of various geographic origins: France (seven patients: A401P, Q357X, W335X, A368T, 1223insG, A291V, K426E, K437X); Portugal (two patients: G308E); Tunisia (two patients: Q357R); Turkey (one patient: S381R); Italy (two siblings: S381R). Interestingly, 6/14 mutant alleles carried by unrelated probands of French ancestry bore the A401P mutation, indicating a founder effect; this effect is probably also present in Portugal, Turkey, and Tunisia. Since mutations occurred in exons 2-5 shared by all mRNAs species of the gene, a combined deficiency of B-UGT and P-UGT was observed in the liver of five patients in whom these activities were measured. The present study confirms that CN-I is genetically heterogeneous and suggests that different founder effects are involved in Western Europe, the Middle East, and North Africa.These results were presented in part at the American Association for the Study of Liver Diseases, Chicago, November 4–7, 1993 相似文献
45.
Virginia P. Ronchi Ana M. Giudici Julieta R. Mendieta Veronica J. Caballero Andrea N. Chisari Pedro M. Sanllorenti Rubén D. Conde 《Journal of physiology and biochemistry》2010,66(2):93-103
The aim of this work was to evaluate the effects of a diet depleted of amino acids (protein-free diet, or PFD), as well as
the supplementation with methionine (PFD+Met), on the antioxidant status of the female mouse liver. With this purpose, cytosolic
protein spots from two-dimensional non-equilibrium pH gel electrophoresis were identified by several procedures, such as mass
spectrometry, Western blot, gel matching and enzymatic activity. PFD decreased the contents of catalase (CAT), peroxiredoxin
I (Prx-I), and glutathione peroxidase (GPx) by 67%, 37% and 45%, respectively. Gene expression analyses showed that PFD caused
a decrease in CAT (−20%) and GPx (−30%) mRNA levels but did not change that of Prx-I. It was also found that, when compared
to a normal diet, PFD increased the liver contents of both reactive oxygen species (+50%) and oxidized protein (+88%) and
decreased that of glutathione (−45%). Supplementation of PFD with Met prevented these latter effects to varying degrees, whereas
CAT, Prx-I and GPx mRNA levels resulted unmodified. Present results suggest that dietary amino acid deprivation deranges the
liver antioxidant defences, and this can be, in part, overcome by supplementation with Met. 相似文献
46.
F Guerrieri F Zanotti G Capozza G Colaianni S Ronchi S Papa 《Biochimica et biophysica acta》1991,1059(3):348-354
Proteolytic digestion of F1-depleted submitochondrial particles (USMP), reconstitution with isolated subunits and titration with inhibitors show that the nuclear-encoded PVP protein, previously identified as an intrinsic component of bovine heart F0 (F01) (Zanotti, F. et al. (1988) FEBS Lett. 237, 9-14), is critically involved in maintaining the proper H+ translocating configuration of this sector and its correct binding to the F1 catalytic moiety. Trypsin digestion of USMP, under conditions leading to cleavage of the carboxyl region of the PVP protein and partial inhibition of transmembrane H+ translocation, results in general loss of sensitivity of this process to F0 inhibitors. This is restored by addition of the isolated PVP protein. Trypsin digestion of USMP causes also loss of oligomycin sensitivity of the catalytic activity of membrane reconstituted soluble F1, which can be restored by the combined addition of PVP and OSCP, or PVP and F6. Amino acid sequence analysis shows that, in USMP, modification by [14C] N,N'-dicyclohexylcarbodiimide of subunit c of F0 induces the formation of a dimer of this protein, which retains the 14C-labelled group. Chemical modification of cysteine-64 of subunit c results in inhibition of H+ conduction by F0. The results indicate that proton conduction in mitochondrial F0 depends on interaction of subunit c with the PVP protein. 相似文献
47.
48.
Eleonora Mauri Robertino Dilena Antonio Boccazzi Dario Ronchi Daniela Piga Fabio Triulzi Delia Gagliardi Roberta Brusa Irene Faravelli Nereo Bresolin Francesca Magri Stefania Corti Giacomo P. Comi 《BMC neurology》2018,18(1):220
Background
Leber’s hereditary optic neuropathy (LHON) is a mitochondrial disease characterized by visual loss consequent to optic nerve atrophy. In some cases, LHON is associated with heterogeneous neurological extraocular manifestations and is referred to as “Leber plus disease”; rarely it is associated with a multiple sclerosis (MS)-like syndrome known as Harding disease, but no pediatric extraocular acute spinal onset is reported.Case presentation
We describe the case of a 5-year-old girl carrying the G3460A mtDNA mutation who was referred to clinical examination for bilateral upper and lower limb weakness with no sign of optic neuropathy. Spinal cord MRI showed hyperintense signal alterations in T2-weighted and restricted diffusion in DWI sequences in the anterior portion of the cervical and dorsal spinal cord resembling a spinal cord vascular injury. No association between this mutation and pediatric spinal cord lesions has previously been reported. Alternative diagnostic hypotheses, including infective, ischemic and inflammatory disorders, were not substantiated by clinical and instrumental investigations.Conclusions
Our case reports a novel pediatric clinical manifestation associated with the m.3460G?>?A mtDNA mutation, broadening the clinical spectrum of this disease. Early identification of new cases and monitoring of carriers beginning in childhood is important to prevent neurological deterioration and preserve long-term function.49.
50.
Properties of D-amino-acid oxidase from Rhodotorula gracilis 总被引:2,自引:0,他引:2
M Pilone Simonetta L Pollegioni P Casalin B Curti S Ronchi 《European journal of biochemistry》1989,180(1):199-204
The flavoprotein D-amino-acid oxidase was purified to homogeneity from the yeast Rhodotorula gracilis by a highly reproducible procedure. The amino acid composition of the protein was determined; the protein monomer had a molecular mass of 39 kDa and contained one molecule of FAD. The ratio between A274/A455 was about 8.2. D-Amino-acid oxidase from yeast showed typical flavin spectral perturbations on binding of the competitive inhibitor benzoate and was reduced by D-alanine under anaerobiosis. The enzyme reacted readily with sulfite to form a covalent reversible adduct and stabilized the red anionic form of the flavin semiquinone on photoreduction in the presence of 5-deazariboflavin; the 3,4-dihydro-FAD form was not detectable after reduction with sodium borohydride. Thus D-amino-acid oxidase from yeast exhibited most of the general properties of the dehydrogenase/oxidase class of flavoproteins; at the same time, the enzyme showed some peculiar features with respect to the same protein from pig kidney. 相似文献