Renal Cell Carcinoma and Hepatocellular Carcinoma in a Patient with Wilson's Disease.

No reports of renal cancer in patients with Wilson's disease (WD) exist. We herein report a 37-year-old Japanese man diagnosed with WD who had been treated with d-penicillamine 9 years prior. Hepatocellular carcinoma had been diagnosed at 36 years old and treated with radiofrequency ablation therapy.

Diagnostic copper imaging of Menkes disease by synchrotron radiation-generated X-ray fluorescence analysis.

Copper (Cu) is an indispensable metal for normal development and function of humans, especially in central nervous system (CNS). However, its redox activity requires accurate Cu transport system. ATP7A, a main Cu(2+) transporting-ATPase, is necessary to efflux Cu across the plasma membrane and synthesize cuproenzymes.

Calcium-overloaded sympathetic preganglionic neurons in a case of severe sepsis with anorexia nervosa.

Aim: We aimed to show the status of intracellular elements in sympathetic preganglionic neurons in an autopsy case of a 55-year-old woman with severe sepsis and cardiac dysfunction with anorexia nervosa.

Methods: Our methods include a case report and pathological examinations of autopsied tissues using synchrotron-generated microbeam X-ray fluorescence analysis.

Results: A case report of severe sepsis and myocardial dysfunction.

Transcellular degradation of axonal mitochondria.

It is generally accepted that healthy cells degrade their own mitochondria. Here, we report that retinal ganglion cell axons of WT mice shed mitochondria at the optic nerve head (ONH), and that these mitochondria are internalized and degraded by adjacent astrocytes. EM demonstrates that mitochondria are shed through formation of large protrusions that originate from otherwise healthy axons.

Formal synthesis of (+)-nakadomarin A.

The formal synthesis of (+)-nakadomarin A was completed. The significant points of this synthesis are the highly stereoselective formation of the diazatricyclo[6.4.

The signaling adaptor protein CD3zeta is a negative regulator of dendrite development in young neurons.

A novel idea is emergxsing that a large molecular repertoire is common to the nervous and immune systems, which might reflect the existence of novel neuronal functions for immune molecules in the brain. Here, we show that the transmembrane adaptor signaling protein CD3zeta, first described in the immune system, has a previously uncharacterized role in regulating neuronal development. Biochemical and immunohistochemical analyses of the rat brain and cultured neurons showed that CD3zeta is mainly expressed in neurons.

Contribution of Va24Vb11 natural killer T cells in Wilsonian hepatitis.

Wilson disease (WD) is an autosomal recessive disorder of copper transport, resulting in copper accumulation and toxicity to the liver and brain. There is no evidence that the WD patient's immune system attacks copper accumulated hepatocytes. Here we describe that the frequency and absolute number of Valpha24+Vbeta11+ natural killer T (NKT) cells were significantly increased in 3 cases of WD, whereas those of CD3+CD161+ NKT cells were within the normal range.

Rat T-cell receptor TRAV11 (Valpha14) genes: further evidence of extensive multiplicity with homogeneous CDR1 and diversified CDR2 by genomic contig and cDNA analysis.

The antigen recognition system of NKT cells acts via an invariant T-cell receptor (TR) which recognizes CD1d and is highly conserved in mice, rats and humans. NKT cells expressing an invariant mouse TR composed of TRAV11-TRAJ18 (formerly Valpha14-Jalpha281) are positively selected by CD1d, and recognize an antigen in context with CD1d. Here we show ten distinct TRAV11 genes (previously designated by us as TRAV14) on rat Chromosome 15 (BN/SsNHsd/MCW strain).

The cell surface-expressed HSC70-like molecule preferentially reacts with the rat T-cell receptor Vdelta6 family.

We previously showed that the cell surface-expressed Mr 70,000 heat shock cognate (hsc70, a constitutively expressed member of the hsp70 family) protein-like molecule (#067 molecule) interacts with rat CD3+, CD4-, CD8-, T-cell receptor (TCR)alphabeta-, natural killer recetor-P1- T cells. This 70hsc-like molecule was also suggested to present cellular peptide antigens to these T cells. In the present study, we identified the genetic structure of the TCR by establishing T-cell hybridomas between these T cells and mouse BW5147 cells.

NKT cells in the rat: organ-specific distribution of NK T cells expressing distinct V alpha 14 chains.

Rat invariant TCR alpha-chains and NKT cells were investigated to clarify whether CD1d-mediated recognition by NKT cells is conserved further in evolution. Rats had multiple-copies of TRAV14 genes, which can be categorized into two types according to the diversity accumulated in the CDR2 region. Rats retained invariant TCR alpha forms with the homogeneous junctional region similar to mouse invariant TRAV14-J281.

Genomic organization and chromosome location of the rat CD3zeta locus (Cd3z).

The structure and pattern of expression of the CD3zeta chain have apparently been conserved throughout mammalian evolution. The organization of the rat CD3zeta locus was determined by genomic cloning and nucleotide sequencing. Most of the rat CD3zeta coding region was similar to mouse and human CD3zeta sequences.

A novel monoclonal antibody against rodent common Thy-1.1 epitope.

Here we report a new anti-rat Thy-1 monoclonal antibody (MAb) 12C5 that recognized a novel epitope on a GPI-anchored Thy-1 antigen. In rat thymocytes, MAb 12C5 reacted with 25 kDa Thy-1 antigen purified with MAb OX7. In rats, MAb 12C5 was expressed on 94% of thymocytes, 46% of spleen cells, 31% of mesenteric lymphnode cells, 76% of bone marrow cells, and 24% of splenic T cells.

Deviated overexpression of TCR-beta, TCR-gamma, CD4, and CD8 on thymic lymphomas induced by 1-propyl-1-nitrosourea: destruction of the allelic exclusion of TCR-beta and expression of functional TCR-betagamma heterodimer on a lymphoma, cFTL53.

Thymic lymphomas (FTLs) induced by the chemical carcinogen 1-propyl-1-nitrosourea (PNU) in F344 rats showed deviated overexpression of TCR-beta, TCR-gamma, CD4, and CD8. Even though most FTLs were in the CD4+ CD8+ stage, all FTLs expressed TCR-beta mRNA with TCR-gamma mRNA, but without TCR-alpha mRNA or TCR-delta mRNA. One of the FTLs, cFTL53, expressed two kinds of TCR-beta mRNA and two kinds of TCR-gamma mRNA, but did not express any mRNA of TCR-alpha or TCR-delta.

RT1.P, rat class Ib genes related to mouse TL: evidence that CD1 molecules but not authentic TL antigens are expressed by rat thymus.

CD1 and TL were once thought to be genetic homologues because of their thymus-specific expression. We investigated their equivalents in the rat to clarify whether their structure and pattern of expression are conserved in rodents. Two rat class Ib genes, containing 3' sequences very similar to mouse TL, were identified and designated RT1.