The spectrum of autoantibodies in IPEX syndrome is broad and includes anti-mitochondrial autoantibodies

IPEX syndrome is a congenital disorder of immune regulation caused by mutations in the FOXP3 gene, which is required for the suppressive function of naturally arising CD4 + CD25 + regulatory T cells. In this case series we evaluated serum samples from 12 patients with IPEX syndrome for the presence of common autoantibodies associated with a broad range of autoimmune disorders. We note that 75% of patients (9/12) had 1 or more autoantibodies, an incidence far above the cumulative rate observed in the general population. The range of autoantibodies differed between patients and there was no predominant autoantibody or pattern of autoantibodies present in this cohort. Surprisingly, one patient had high-titer anti-mitochondrial antibodies (AMA) typically associated with primary biliary cirrhosis (PBC) although the patient had no signs of cholestasis. PBC is a well-characterized autoimmune disease that occurs primarily in women and includes the serological hallmarks of serum AMA and elevated IgM which were both present in this patient. PBC is virtually absent in children with the exception of one reported child with interleukin 2 receptor a (CD25) deficiency which is associated with an IPEX-like regulatory T cell dysfunction. Based on the present data and the available literature we suggest a direct role for CD4 + CD25 + regulatory T cells in restraining B cell autoantibody production and that defects in regulatory T cells may be crucial to the development of PBC. (C) 2010 Elsevier Ltd. All rights reserved.

The region of mouse mammary tumor virus DNA containing the long terminal repeat includes a long coding sequence and signals for hormonally regulated transcription.

Starting from a biologically active recombinant DNA clone of exogenous unintegrated GR mouse mammary tumor virus, we have generated three subclones of PstI fragments of 1.45, 1.1, and 2.0 kb in the plasmid vector PBR322. The nucleotide sequence has been determined for the clone of 1.45 kb which includes almost the complete region of the long terminal repeat (LTR) plus an adjacent stretch of unique sequence DNA. A short region of the 2.0 kb clone, containing the beginning of the LTR, has also been sequenced. Starting with the A of an initiation codon outside the LTR, we detected an open reading frame of 960 nucleotides, potentially coding for a protein of 320 amino acids (36K). Two hundred nucleotides downstream from the termination codon, and approximately 25 nucleotides upstream from the presumptive initiation site of viral RNA synthesis, we found a promoter-like sequence. The sequence AGTAAA was detected approximately 15-20 nucleotides upstream from the 3' end of virion RNA and probably serves as a polyadenylation signal. The 1.45 kb PstI fragment has been transfected into Ltk- cells together with a plasmid containing the thymidine kinase gene of herpes simplex virus. The virus-specific RNA synthesis detected in a Tk+ cell clone was strongly stimulated by the addition of dexamethasone.

Density matrix functional theory that includes pairing correlations

The extension of density functional theory (DFT) to include pairing correlations without formal violation of the particle-number conservation condition is described. This version of the theory can be considered as a foundation of the application of existing DFT plus pairing approaches to atoms, molecules, ultracooled and magnetically trapped atomic Fermi gases, and atomic nuclei where the number of particles is conserved exactly. The connection with Hartree-Fock-Bogoliubov (HFB) theory is discussed, and the method of quasilocal reduction of the nonlocal theory is also described. This quasilocal reduction allows equations of motion to be obtained which are much simpler for numerical solution than the equations corresponding to the nonlocal case. Our theory is applied to the study of some even Sn isotopes, and the results are compared with those obtained in the standard HFB theory and with the experimental ones.

Effect of Waterproofing Admixture Ipanex on Concrete Durability (includes Addendum), MLR-98-2, 1999

The concrete admixture Ipanex (Registered trademark) manufactured by IPA Systems Inc. was submitted to the Iowa Department of Transportation (Iowa DOT) New Products Committee on April 15, 1998. The New Products Committee requested that the Iowa DOT Materials Laboratory evaluate the durability, corrosion inhibiting and concrete permeability reduction effects of this admixture. This report is intended to present the results of testing in Iowa DOT materials laboratories, review a Pennsylvania State University report, as well as review the IPA Systems Inc. marketing literature. The objective is to provide the New Products Committee with a recommendation concerning approval of this product based on the information gathered. The portland cement concrete admixture Ipanex (Registered trademark) did not show any significant benefit in terms of improvement in areas of permeability, chloride resistance and strength in the testing performed at the Iowa DOT. The literature and reports reviewed did not provide enough credible evidence to refute this conclusion. Additionally, the benefits ascribed to this product can be more economically achieved using other currently available products such as slag and silica fume. The recommendation is that this product not be approved for use on State projects in Iowa.

The molecular basis of selective permeability of connexins is complex and includes both size and charge

Although gap junction channels are still widely viewed as large, non-specific pores connecting cells, the diversity in the connexin family has led more attention to be focused on their permeability characteristics. We summarize here the current status of these investigations, both published and on-going, that reveal both charge and size selectivity between gap junction channels composed of different connexins. In particular, this review will focus on quantitative approaches that monitor the expression level of the connexins, so that it is clear that differences that are seen can be attributed to channel properties. The degree of selectivity that is observed is modest compared to other channels, but is likely to be significant for biological molecules that are labile within the cell. Of particular relevance to the in vivo function of gap junctions, recent studies are summarized that demonstrate that the connexin phenotype can control the nature of the endogenous traffic between cells, with consequent effects on biological effects of gap junctions such as tumor suppression.

Welland Canal Survey of Lands Nathan Pawling 1826; includes map and text.

Survey map and description of Nathan Pawling's land created by The Welland Canal Company. Included is a written description of the land along with a drawing of the land. Noteable features include; the dock, pier and tow path. The original bed of 12 mile creek is also noted on the map. Surveyor notes are seen in pencil on the map. The map notes the division of land between Pawling and Robert Brown's land. Click on 'detail' to see Map.

Cogswell, Maria, includes: Application for loan on Real Estate, Insurance Policy, Mortgage Loan Envelope

Cogswell, Maria, includes: Application for loan on Real Estate, Feb. 20, 1882; Insurance Policy no. 2199780 from the Royal Insurance Company of Liverpool, March 17, 1887 and Mortgage Loan Envelope for mortgage no. 1535 from March 1, 1882 – March 1, 1887.

Conklin, Pearl J., includes: Application for loan on Real Estate

Conklin, Pearl J., includes: Application for loan on Real Estate, Aug. 11, 1882.

Crew, Isaac M., includes: Application for Loan, Mortgage Loan Envelope, and Abstract of Title

Crew, Isaac M., includes: Application for Loan, March 10, 1885; Mortgage Loan Envelope no. 679 for Jan. 1, 1885 – Jan. 1, 1890 and Abstract of Title, April 13, 1885.

Crick, Henry A., includes Application for Loan, and Mortgage Loan Envelope

Crick, Henry A., includes Application for Loan, Jan. 8, 1885 and Mortgage Loan Envelope no. 637 for Jan. 1, 1885 – Jan. 1, 1890.

Dennis, George W. and Mary E. Dennis, includes: Application for Loan on Real Estate, and Abstract of Title

Dennis, George W. and Mary E. Dennis, includes: Application for Loan on Real Estate, July 10, 1882; and Abstract of Title, Sept. 2, 1882.

Sampson, Albert, includes Application for a Loan on Real Estate, and an envelope with Mr. Sampson’s name crossed out

Sampson, Albert, includes Application for a Loan on Real Estate, April 19, 1882 and an envelope with Mr. Sampson’s name crossed out and postmarked Sept. 1882.

Schmidt, John, includes: Application for Loan, and Mortgage Loan Envelope, and Abstract of Title

Schmidt, John, includes: Application for Loan, Aug. 8, 1884 and Mortgage Loan Envelope no. 302 for July 1, 1884 – July 1, 1889 and Abstract of Title, Sept. 6, 1884.

Voucher #22 to James McCoppen (copy) which includes a letter from James McCoppen regarding a claim for having water backed up into his grain and grass by the embankment of the railroad. The letter is dated June 24, 1857 and the voucher is dated Jul. 1, 1857

Voucher #22 to James McCoppen (copy) which includes a letter from James McCoppen regarding a claim for having water backed up into his grain and grass by the embankment of the railroad. The letter is dated June 24, 1857 and the voucher is dated Jul. 1, 1857.