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Sökning: WFRF:(Li L)

  • Resultat 4061-4070 av 5130
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4061.
  • Li, P. L., et al. (författare)
  • Preparation of aligned Ca3Co2O6 nanorods and their steplike magnetization
  • 2007
  • Ingår i: Applied Physics Letters. - : American Institute of Physics (AIP). - 0003-6951 .- 1077-3118. ; 91:4, s. 042505-
  • Tidskriftsartikel (refereegranskat)abstract
    • Well-aligned Ca3Co2O6 nanorods (similar to 300 nm in length and similar to 40 nm in diameter) on Si substrates are prepared by pulsed laser deposition. The steplike magnetization feature as identified in bulk Ca3Co2O6 can be retained in the nanorods in spite of the enhanced distortion of the magnetically ordered spin chains due to the finite size effect. The out-of-plane magnetization value is slightly larger than the in-plane value, which shows the small magnetic anisotropy. An additional distinct transition at 54 K associated with the spin frustration is revealed, different from the bulk samples. (C) 2007 American Institute of Physics.
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4062.
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4063.
  • Li, Ping, et al. (författare)
  • Structures of Atm1 provide insight into [2Fe-2S] cluster export from mitochondria
  • 2022
  • Ingår i: Nature Communications. - : Springer Science and Business Media LLC. - 2041-1723. ; 13:1
  • Tidskriftsartikel (refereegranskat)abstract
    • In eukaryotes, iron-sulfur clusters are essential cofactors for numerous physiological processes, but these clusters are primarily biosynthesized in mitochondria. Previous studies suggest mitochondrial ABCB7-type exporters are involved in maturation of cytosolic iron-sulfur proteins. However, the molecular mechanism for how the ABCB7-type exporters participate in this process remains elusive. Here, we report a series of cryo-electron microscopy structures of a eukaryotic homolog of human ABCB7, CtAtm1, determined at average resolutions ranging from 2.8 to 3.2 Å, complemented by functional characterization and molecular docking in silico. We propose that CtAtm1 accepts delivery from glutathione-complexed iron-sulfur clusters. A partially occluded state links cargo-binding to residues at the mitochondrial matrix interface that line a positively charged cavity, while the binding region becomes internalized and is partially divided in an early occluded state. Collectively, our findings substantially increase the understanding of the transport mechanism of eukaryotic ABCB7-type proteins.
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4064.
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4065.
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4066.
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4067.
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4068.
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4069.
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4070.
  • Li, Q., et al. (författare)
  • Novel AVPR2 mutations and clinical characteristics in 28 Chinese families with congenital nephrogenic diabetes insipidus
  • 2021
  • Ingår i: Journal of Endocrinological Investigation. - : Springer Nature. - 0391-4097 .- 1720-8386. ; 44:12, s. 2777-2783
  • Tidskriftsartikel (refereegranskat)abstract
    • Aims: To investigate genotype and phenotype of congenital nephrogenic diabetes insipidus caused by AVPR2 mutations, which is rare and limitedly studied in Chinese population.Methods: 88 subjects from 28 families with NDI in a department (Beijing, PUMCH) were screened for AVPR2 mutations. Medical records were retrospectively reviewed and characterized. Genotype and phenotype analysis was performed.Results: 23 AVPR2 mutations were identified, including six novel mutations (p.Y117D, p.W208R, p.L313R, p.S127del, p.V162Sfs*30 and p.G251Pfs*96). The onset-age ranged from 1 week to 3 years. Common presentations were polydipsia and polyuria (100%) and intermittent fever (57%). 21% and 14% of patients had short stature and mental impairment. Urine SG and osmolality were decreased, while serum osmolality and sodium were high. Urological ultrasonography results showed hydronephrosis of the kidney (52%), dilation of the ureter (48%), and thickened bladder wall or increased residual urine (32%), led to intermittent urethral catheterization (7%), cystostomy (11%) and binary nephrostomy (4%). Urological defects were developed in older patients. Genotype and phenotype analysis revealed patients with non-missense mutations had higher levels of serum sodium than missense mutations.Conclusion: In the first and largest case series of NDI caused by AVPR2 mutations in Chinese population, we established genetic profile and characterized clinical data, reporting six novel mutations. Further, we found genotype was associated with phenotype. This knowledge broadens genotype and phenotype spectrum of rare congenital NDI caused by AVPR2 mutations, and provides basis for studying molecular biology of AVPR2.
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