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711.
712.
Teodora Oltean Emily Van San Tatyana Divert Tom Vanden Berghe Xavier Saelens Jonathan Maelfait Nozomi Takahashi Peter Vandenabeele 《Cell death & disease》2021,12(5)
RIPK3 was reported to play an important role in the protection against influenza A virus (IAV) in vivo. Here we show that the requirement of RIPK3 for protection against IAV infection in vivo is only apparent within a limited dose range of IAV challenge. We found that this protective outcome is independent from RIPK3 kinase activity and from MLKL. This shows that platform function of RIPK3 rather than its kinase activity is required for protection, suggesting that a RIPK3 function independent of necroptosis is implicated. In line with this finding, we show that FADD-dependent apoptosis has a crucial additional effect in protection against IAV infection. Altogether, we show that RIPK3 contributes to protection against IAV in a narrow challenge dose range by a mechanism that is independent of its kinase activity and its capacity to induce necroptosis.Subject terms: Cell death, Cell death and immune response, Infection 相似文献
713.
Summary A severely retarded child with multiple malformations was found to present a mosaic karyotype 46,XX,-13,+t(13;13)(p11;q11)/46,XX,del (13)(p11), which probably originated as the result of a de novo 13/13 translocation in a parental gamete, followed by postzygotic fission of the translocation chromosomse. 相似文献
714.
Raheem Fazal Steven Boeynaems Ann Swijsen Mathias De Decker Laura Fumagalli Matthieu Moisse Joni Vanneste Wenting Guo Ruben Boon Thomas Vercruysse Kristel Eggermont Bart Swinnen Jimmy Beckers Donya Pakravan Tijs Vandoorne Pieter Vanden Berghe Catherine Verfaillie Ludo Van Den Bosch Philip Van Damme 《The EMBO journal》2021,40(7)
TDP‐43 is the major component of pathological inclusions in most ALS patients and in up to 50% of patients with frontotemporal dementia (FTD). Heterozygous missense mutations in TARDBP, the gene encoding TDP‐43, are one of the common causes of familial ALS. In this study, we investigate TDP‐43 protein behavior in induced pluripotent stem cell (iPSC)‐derived motor neurons from three ALS patients with different TARDBP mutations, three healthy controls and an isogenic control. TARDPB mutations induce several TDP‐43 changes in spinal motor neurons, including cytoplasmic mislocalization and accumulation of insoluble TDP‐43, C‐terminal fragments, and phospho‐TDP‐43. By generating iPSC lines with allele‐specific tagging of TDP‐43, we find that mutant TDP‐43 initiates the observed disease phenotypes and has an altered interactome as indicated by mass spectrometry. Our findings also indicate that TDP‐43 proteinopathy results in a defect in mitochondrial transport. Lastly, we show that pharmacological inhibition of histone deacetylase 6 (HDAC6) restores the observed TDP‐43 pathologies and the axonal mitochondrial motility, suggesting that HDAC6 inhibition may be an interesting therapeutic target for neurodegenerative disorders linked to TDP‐43 pathology. 相似文献
715.
T Vanden Driessche 《Chronobiology international》1984,1(2):113-120
In order to support the hypothesis that circadian rhythms are implicated in cap formation, experiments were undertaken on the possible time-dependency of the effects of (a) a competitive inhibitor of auxins, morphactin and (b) of auxin (IAA). It was found that: (i) the inhibitory effect of morphactin varies dramatically with the time at which the several weeks' treatment was first begun; (ii) the maximum inhibition varies with development and decreases with time; (iii) IAA accelerates cap formation when the algae are submitted to IAA during the exponential growth phase; the effect is time dependent and decreases with time; (iv) IAA first applied on smaller algae has a transient inhibitory effect which is time dependent; (v) anucleate fragments also respond differentially to an IAA treatment begun at several times in the 24-hr cycle, most clearly when newly formed mRNA have been accumulated and (vi) the effect of iAA is not cumulative with that of a LD shift; that of morphactin is not, or only slightly, improved by a LD shift. 相似文献
716.
Mosaic pericentric inversion of chromosome 2 总被引:1,自引:0,他引:1
A pericentric inversion of chromosome number 2 in mosaic with a normal cell line is reported in a 8-year-old boy associated with slight dysmorphic syndrome and moderate mental handicap. 相似文献
717.
Method to identify specific alleles of a Phanerochaete chrysosporium gene encoding lignin peroxidase. 总被引:3,自引:1,他引:2
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A method to identify and differentiate allelic variants of the gene encoding lignin peroxidase isozyme H8 is presented. The strategy involves amplifying a variable region of the gene's carboxy terminus by use of the polymerase chain reaction and then probing with allele-specific oligonucleotides. 相似文献
718.
In this report we describe a female neonate with 12p interstitial deletion (karyotype: 46,XX,del(12)(pter----p13.1::p11.2----cen----qter). In addition to severe psychomotor retardation, facial dysmorphism and Turner like stigmata, she presented marked hypoplasia of the external genitalia and right heart hypoplasia. Study of LDH activity showed a marked decrease of LDHB activity contrasting with an elevated LDHA. 相似文献
719.
720.
Two elderly women had acute myelogenous leukemia with rapidly fatal course. On initial marrow and unsttimulated blood examination, the 5q- chromosome and additional number and structural chromosome analomalies were observed. The specificity of these findings is discussed. 相似文献