BACKGROUND Intraductal papillary mucinous neoplasm of the pancreas (IPMN) and pancreatic ductal adenocarcinoma (PDAC) often coexist in the same pancreas. Almost all IPMNs involving PDACs concomitant ...with IPMN have been shown to be branch duct type IPMNs (BD-IPMNs), and their histological subtypes are gastric type. Therefore, PDACs concomitant with main duct type IPMNs (MD-IPMNs) are considered to be rare. We herein report a rare case preoperatively diagnosed as being a PDAC concomitant with MD-IPMN on the basis of imaging findings and histological findings of pancreatic specimens endoscopically obtained from 2 lesions. CASE REPORT A 67-year-old man was referred to our hospital due to an enlarged pancreas. Using imaging studies, a solid mass was found in the pancreatic head and intraductal papillary masses in the dilated main pancreatic duct of the body and tail with a fistula in the duodenum. On the basis of histological results using specimens endoscopically obtained from each of the 2 lesions, total pancreatectomy was planned due to suspected PDAC concomitant with an MD-IPMN. Finally, resected specimens were used to confirm the presence of a rare case of PDAC concomitant with MD-IPMN. CONCLUSIONS We encountered a rare case of a PDAC concomitant with an MD-IPMN which could be preoperatively diagnosed by using imaging studies and histological specimens endoscopically obtained. In addition to invasive cancers derived from IPMNs, PDACs concomitant with IPMNs can rarely develop in the pancreas involving MD-IPMNs.
Autosomal recessive hypotrichosis (ARH) is characterized by sparse hair on the scalp without other abnormalities. Three genes, DSG4, LIPH, and LPAR6 (P2RY5), have been reported to underlie ARH. We ...performed a mutation search for the three candidate genes in five independent Japanese ARH families and identified two LIPH mutations: c.736T>A (p.Cys246Ser) in all five families, and c.742C>A (p.His248Asn) in four of the five families. Out of 200 unrelated control alleles, we detected c.736T>A in three alleles and c.742C>A in one allele. Haplotype analysis revealed each of the two mutant alleles is derived from a respective founder. These results suggest the LIPH mutations are prevalent founder mutations for ARH in the Japanese population. LIPH encodes PA-PLA₁α (LIPH), a membrane-associated phosphatidic acid-preferring phospholipase A₁α. Two residues, altered by these mutations, are conserved among PA-PLA₁α of diverse species. Cys²⁴⁶ forms intramolecular disulfide bonds on the lid domain, a crucial structure for substrate recognition, and His²⁴⁸ is one amino acid of the catalytic triad. Both p.Cys246Ser- and p.His248Asn-PA-PLA₁α mutants showed complete abolition of hydrolytic activity and had no P2Y5 activation ability. These results suggest defective activation of P2Y5 due to reduced 2-acyl lysophosphatidic acid production by the mutant PA-PLA₁α is involved in the pathogenesis of ARH. Hum Mutat 31:1-9, 2010.
Escherichia coli
cells that express the full six carotenoid biosynthesis genes (
crtE
,
crtB
,
crtI
,
crtY
,
crtZ
, and
crtX
) of the bacterium
Pantoea ananatis
have been shown to biosynthesize ...zeaxanthin 3,3′-β-
d
-diglucoside. We found that this recombinant
E. coli
also produced a novel carotenoid glycoside that contained a rare carbohydrate moiety, quinovose (chinovose; 6-deoxy-
d
-glucose), which was identified as 3-β-glucosyl-3′-β-quinovosyl zeaxanthin by chromatographic and spectroscopic analyses. The chirality of the aglycone of these zeaxanthin glycosides had been shown to be 3
R
,3′
R
, in which the hydroxyl groups were formed with the CrtZ enzyme. It was here demonstrated that zeaxanthin synthesized from β-carotene with CrtR or CYP175A1, the other hydroxylase with similar catalytic function to CrtZ, possessed the same stereochemistry. It was also suggested that the singlet oxygen-quenching activity of zeaxanthin 3,3′-β-
d
-diglucoside, which has a chemical structure close to the new carotenoid glycoside, was superior to that of zeaxanthin.
Background: Biopsy diagnosis of gastric mucinous carcinoma (GMC) is sometimes difficult. Endoscopic ultrasonography (EUS) can visualize the change in the gastric wall in detail. We reviewed the EUS ...images and histological findings of cases of GMC and evaluated the correlation between them to identify the characteristic findings of GMC on EUS.
Methods: Thirty‐one GMC patients who had undergone both preoperative EUS and surgery were studied. The EUS images of these patients and the histological findings were compared with those for other histological types of gastric cancer (OGC).
Results: The tumor echo showed high echoic in GMC cases more frequently than that in OGC. The frequency of visualization of the layer structure above the tumor was higher compared with OGC. Histology verified the presence of fine septa composed of thin fibrotic tissue and floating cancer cells in the mucus lake. The mucus lake was often located in the submucosa and the tumor was covered with cancerous mucosa of other histological types with preserved lamina muscularis mucosae.
Conclusions: The EUS findings of GMC are characteristic and reflect its histological changes well, and would complement the preoperative diagnosis of this tumor.
Endosonography-guided biliary drainage (ESBD) is a new option that allows establishment of biliary drainage. Due to the diameter of the working channel of an echoendoscope, it is necessary to replace ...a small caliber stent with a larger one to lessen the risk of stent occlusion. However, insertion of a guidewire into the bile duct via the hole of the sinus tract following direct removal of a previously placed stent is not always possible, resulting in guidewire passage outside the fistula and bile leakage. Cannulation of the previously deployed stent, guidewire insertion into the bile duct via the cannula and the stent, and removal of the stent with the snare over the guidewire leaving the guidewire in place (the snare-over-the-wire technique SOW) for stent exchange following ESBD was attempted. Four patients who required stent exchange following ESBD were included in the present study to evaluate the feasibility and usefulness of SOW. SOW was successful in all the cases. A new stent was also successfully deployed over the guidewire in all the cases. No complications were encountered. The snare-over-the-wire technique is feasible and useful in stent exchange following ESBD for the reduction of the risk of guidewire migration.
Pedunculated polypoid cancer of the gallbladder ordinarily shows cancer spread within the polyp consisting mainly of adenoma. We experienced a case of pedunculated mucosal cancer associated with ...flat‐type cancer of the gallbladder without an adenomatous component, the details of which are reported herein. The lesion was first detected by transabdominal ultrasonography (US) as a polypoid lesion of the gallbladder, 5 mm in size. Distinct growth of the lesion was revealed at follow‐up US after a year. Endoscopic ultrasonography showed not only a pedunculated polypoid lesion, 9 mm in size, with a solid internal echo pattern and a nodular surface, but also slight thickening of the surrounding gallbladder wall. Cholecystectomy was performed with a preoperative diagnosis of early gallbladder cancer, and a pedunculated polypoid lesion, 8 × 8 × 3 mm in size with a thin stalk and a nodular contour, surrounded by a widely spreading flat lesion with a coarse‐granular surface, was confirmed. Microscopically, the stalk was 700 µm in size, and both the pedunculated polypoid lesion and flat lesion consisted of well‐differentiated tubular adenocarcinoma limited to the mucosa, without an adenomatous component.
This review summarizes technical development of the functional manipulation of specific neural circuits through genetic techniques in Drosophila. Long after pioneers' efforts for the genetic ...dissection of behavior using this organism as a model, analyses with acute activation of specific neural circuits have finally become feasible using transgenic Drosophila that expresses light-, heat-, or cold-activatable cation channels by xxx/upstream activation sequence (Gal4/UAS)-based induction system. This methodology opened a new avenue to dissect functions of neural circuits to make dreams of the pioneers into reality.
Although the gustatory system provides animals with sensory cues important for food choice and other critical behaviors, little is known about neural circuitry immediately following gustatory sensory ...neurons (GSNs). Here, we identify and characterize a bilateral pair of gustatory second-order neurons (G2Ns) in Drosophila. Previous studies identified GSNs that relay taste information to distinct subregions of the primary gustatory center (PGC) in the gnathal ganglia (GNG). To identify candidate G2Ns, we screened ∼5,000 GAL4 driver strains for lines that label neural fibers innervating the PGC. We then combined GRASP (GFP reconstitution across synaptic partners) with presynaptic labeling to visualize potential synaptic contacts between the dendrites of the candidate G2Ns and the axonal terminals of Gr5a-expressing GSNs, which are known to respond to sucrose. Results of the GRASP analysis, followed by a single-cell analysis by FLP-out recombination, revealed a pair of neurons that contact Gr5a axon terminals in both brain hemispheres and send axonal arborizations to a distinct region outside the PGC but within the GNG. To characterize the input and output branches, respectively, we expressed fluorescence-tagged acetylcholine receptor subunit (Dα7) and active-zone marker (Brp) in the G2Ns. We found that G2N input sites overlaid GRASP-labeled synaptic contacts to Gr5a neurons, while presynaptic sites were broadly distributed throughout the neurons' arborizations. GRASP analysis and further tests with the Syb-GRASP method suggested that the identified G2Ns receive synaptic inputs from Gr5a-expressing GSNs, but not Gr66a-expressing GSNs, which respond to caffeine. The identified G2Ns relay information from Gr5a-expressing GSNs to distinct regions in the GNG, and are distinct from other, recently identified gustatory projection neurons, which relay information about sugars to a brain region called the antennal mechanosensory and motor center (AMMC). Our findings suggest unexpected complexity for taste information processing in the first relay of the gustatory system.
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