Expanding the clinical spectrum associated with defects in CNTNAP2 and NRXN1

RIGHTS : This article is licensed under the BioMed Central licence at http://www.biomedcentral.com/about/license which is similar to the 'Creative Commons Attribution Licence'. In brief you may : copy, distribute, and display the work; make derivative works; or make commercial use of the work - under the following conditions: the original author must be given credit; for any reuse or distribution, it must be made clear to others what the license terms of this work are.Abstract Background Heterozygous copy-number and missense variants in CNTNAP2 and NRXN1 have repeatedly been associated with a wide spectrum of neuropsychiatric disorders such as developmental language and autism spectrum disorders, epilepsy and schizophrenia. Recently, homozygous or compound heterozygous defects in either gene were reported as causative for severe intellectual disability. Methods 99 patients with severe intellectual disability and resemblance to Pitt-Hopkins syndrome and/or suspected recessive inheritance were screened for mutations in CNTNAP2 and NRXN1. Molecular karyotyping was performed in 45 patients. In 8 further patients with variable intellectual disability and heterozygous deletions in either CNTNAP2 or NRXN1, the remaining allele was sequenced. Results By molecular karyotyping and mutational screening of CNTNAP2 and NRXN1 in a group of severely intellectually disabled patients we identified a heterozygous deletion in NRXN1 in one patient and heterozygous splice-site, frameshift and stop mutations in CNTNAP2 in four patients, respectively. Neither in these patients nor in eight further patients with heterozygous deletions within NRXN1 or CNTNAP2 we could identify a defect on the second allele. One deletion in NRXN1 and one deletion in CNTNAP2 occurred de novo, in another family the deletion was also identified in the mother who had learning difficulties, and in all other tested families one parent was shown to be healthy carrier of the respective deletion or mutation. Conclusions We report on patients with heterozygous defects in CNTNAP2 or NRXN1 associated with severe intellectual disability, which has only been reported for recessive defects before. These results expand the spectrum of phenotypic severity in patients with heterozygous defects in either gene. The large variability between severely affected patients and mildly affected or asymptomatic carrier parents might suggest the presence of a second hit, not necessarily located in the same gene.Peer Reviewe

Biallelic mutations in NBAS cause recurrent acute liver failure with onset in infancy

Acute liver failure (ALF) in infancy and childhood is a life-threatening emergency. Few conditions are known to cause recurrent acute liver failure (RALF), and in about 50% of cases, the underlying molecular cause remains unresolved. Exome sequencing in five unrelated individuals with fever-dependent RALF revealed biallelic mutations in NBAS. Subsequent Sanger sequencing of NBAS in 15 additional unrelated individuals with RALF or ALF identified compound heterozygous mutations in an additional six individuals from five families. Immunoblot analysis of mutant fibroblasts showed reduced protein levels of NBAS and its proposed interaction partner p31, both involved in retrograde transport between endoplasmic reticulum and Golgi. We recommend NBAS analysis in individuals with acute infantile liver failure, especially if triggered by fever

The German National Registry of Primary Immunodeficiencies (2012-2017)

Introduction: The German PID-NET registry was founded in 2009, serving as the first national registry of patients with primary immunodeficiencies (PID) in Germany. It is part of the European Society for Immunodeficiencies (ESID) registry. The primary purpose of the registry is to gather data on the epidemiology, diagnostic delay, diagnosis, and treatment of PIDs. Methods: Clinical and laboratory data was collected from 2,453 patients from 36 German PID centres in an online registry. Data was analysed with the software Stata® and Excel. Results: The minimum prevalence of PID in Germany is 2.72 per 100,000 inhabitants. Among patients aged 1–25, there was a clear predominance of males. The median age of living patients ranged between 7 and 40 years, depending on the respective PID. Predominantly antibody disorders were the most prevalent group with 57% of all 2,453 PID patients (including 728 CVID patients). A gene defect was identified in 36% of patients. Familial cases were observed in 21% of patients. The age of onset for presenting symptoms ranged from birth to late adulthood (range 0–88 years). Presenting symptoms comprised infections (74%) and immune dysregulation (22%). Ninety-three patients were diagnosed without prior clinical symptoms. Regarding the general and clinical diagnostic delay, no PID had undergone a slight decrease within the last decade. However, both, SCID and hyper IgE- syndrome showed a substantial improvement in shortening the time between onset of symptoms and genetic diagnosis. Regarding treatment, 49% of all patients received immunoglobulin G (IgG) substitution (70%—subcutaneous; 29%—intravenous; 1%—unknown). Three-hundred patients underwent at least one hematopoietic stem cell transplantation (HSCT). Five patients had gene therapy. Conclusion: The German PID-NET registry is a precious tool for physicians, researchers, the pharmaceutical industry, politicians, and ultimately the patients, for whom the outcomes will eventually lead to a more timely diagnosis and better treatment

New Cheilostomata (Bryozoa) from NE Atlantic seamounts, islands, and the continental slope: evidence for deep-sea endemism

Ten new species belonging to three new genera (Atlantisina gen. nov., Bathycyclopora gen. nov., Calvetopora gen. nov.) of umbonulomorph bryozoans from northeastern Atlantic seamounts, islands, and the continental slope are introduced. We furthermore erect the new family Atlantisinidae fam. nov. for these genera. Eight new species belong to the new genus Atlantisina: Atlantisina atlantis gen. et sp. nov. (type species), A. acantha gen. et sp. nov., A. gorringensis gen. et sp. nov., A. inarmata gen. et sp. nov., A. lionensis gen. et sp. nov., A. meteor gen. et sp. nov., A. seinensis gen. et sp. nov., and A. tricornis gen. et sp. nov. The genus Bathycyclopora gen. nov. is introduced for ?Phylactella vibraculata Calvet from the Azores, and also includes Bathycyclopora suroiti gen. et sp. nov. The type species of Calvetopora gen. nov. is Lepralia inflata Calvet from the Gulf of Cadiz; this genus also includes Calvetopora otapostasis gen. et sp. nov. and another species left in open nomenclature. Of the 13 species described herein, 11 occur on seamounts and islands, and nine species are endemic to a single seamount, island or station. The present results show that bryozoans provide striking examples of the function of seamounts as areas of endemism, most likely intrinsically linked to the low dispersal abilities of bryozoan larvae.</p

New Cheilostomata (Bryozoa) from NE Atlantic seamounts, islands, and the continental slope: evidence for deep-sea endemism

International audienceTen new species belonging to three new genera (Atlantisina gen. nov., Bathycyclopora gen. nov., Calvetopora gen. nov.) of umbonulomorph bryozoans from northeastern Atlantic seamounts, islands, and the continental slope are introduced. We furthermore erect the new family Atlantisinidae fam. nov. for these genera. Eight new species belong to the new genus Atlantisina: Atlantisina atlantis gen. et sp. nov. (type species), A. acantha gen. et sp. nov., A. gorringensis gen. et sp. nov., A. inarmata gen. et sp. nov., A. lionensis gen. et sp. nov., A. meteor gen. et sp. nov., A. seinensis gen. et sp. nov., and A. tricornis gen. et sp. nov. The genus Bathycyclopora gen. nov. is introduced for ?Phylactella vibraculata Calvet from the Azores, and also includes Bathycyclopora suroiti gen. et sp. nov. The type species of Calvetopora gen. nov. is Lepralia inflata Calvet from the Gulf of Cadiz; this genus also includes Calvetopora otapostasis gen. et sp. nov. and another species left in open nomenclature. Of the 13 species described herein, 11 occur on seamounts and islands, and nine species are endemic to a single seamount, island or station. The present results show that bryozoans provide striking examples of the function of seamounts as areas of endemism, most likely intrinsically linked to the low dispersal abilities of bryozoan larvae

Atlantisina meteor Berning & Harmelin & Bader & Cibio 2017, gen. et sp. nov.

&lt;i&gt;Atlantisina meteor&lt;/i&gt; gen. et sp. nov. &lt;p&gt;urn:lsid:zoobank.org:act: F9928F6E-D74B-47B5-B985-CAC2BB62ED51&lt;/p&gt; &lt;p&gt;Figs 1B, 3 A&ndash;F, Table 3&lt;/p&gt; Diagnosis &lt;p&gt;Frontal shield densely covered by relatively small, irregularly shaped nodules with flattened tips; lateral walls very well developed, septular pores very large, round to elongate transversely oval; distolateral margin of orifice with eight (or rarely nine) slender oral spines, condyles short, blunt and thickened, no suboral mucro. Ectooecium covering more or less the lower half of ooecium; exposed endooecium relatively large and hemispherical, surface topography generally as frontal shield but nodular pattern not as pronounced. Ancestrula presumably with nine spines grouped in five widely spaced proximal ones and four closely spaced distal ones, opesia slightly constricted in distal third, cryptocyst practically absent.&lt;/p&gt; Etymology &lt;p&gt;Named after its type locality, the Great Meteor Bank; used as a noun in apposition.&lt;/p&gt; &lt;p&gt; &lt;b&gt;Holotype&lt;/b&gt;&lt;/p&gt; &lt;p&gt;GREAT METEOR BANK: 2 colonies on limestone, the larger one with eight ovicells is the holotype (OLL 2016/130a), the smaller colony without ovicells is the paratype (OLL 2016/130b), bleached, Stn 20.&lt;/p&gt; &lt;p&gt; &lt;b&gt;Paratypes&lt;/b&gt;&lt;/p&gt; &lt;p&gt; GREAT METEOR BANK: 4 colonies on limestone (2 with ovicells, 1 with ancestrula), unbleached, Stn 20 (MNHN-IB- 2014-50); 2 colonies on limestone (1 with ancestrula, 1 with ovicells), unbleached, Stn 20 (MNHN-IB- 2014-51); 1 colony with ancestrula on limestone, unbleached, Stn 20 (MNHN- IB- 2014-52); 1 colony on &lt;i&gt;Cladocora&lt;/i&gt; &lt;i&gt;debilis&lt;/i&gt; Milne Edwards &amp; Haime, 1849, mounted on stub and sputter-coated, Stn 21 (OLL 2016/131); 1 colony with ancestrula and ovicells on coral base, unbleached, Stn 20 (OLL 2016/132); 2 colonies on limestone, unbleached, Stn 20 (OLL2016/133); 1 colony on bioclast, mounted on stub and sputter-coated, Stn 20 (SMF 40.039); 4 colonies (2 with ancestrula) on limestone, unbleached, Stn 20 (SMF 40.040); 1 colony with ovicells on limestone, bleached, Stn 20 (SMF 40.041).&lt;/p&gt; Other material examined &lt;p&gt; GREAT METEOR BANK: 10 colonies on &lt;i&gt;Anomocora fecunda&lt;/i&gt; (Pourtal&egrave;s, 1871), Stn 19 (unregistered MNHN material); 1 tiny colony on biogenic debris, sputter-coated, Stn 23 (unregistered MNHN material); 1 colony on &lt;i&gt;C. debilis&lt;/i&gt;, mounted on stub and sputter-coated, Stn 21 (OLL 2015/10); 1 colony on &lt;i&gt;C. debilis&lt;/i&gt;, mounted on stub and sputter-coated, Stn 19 (OLL 2016/134); 3 zooids (interior frontal shield), mounted on stub and sputter-coated, Stn 20 (OLL 2016/135); several colonies on &lt;i&gt;C. debilis&lt;/i&gt; skeletons, unbleached, Stn 21 (OLL 2016/136); numerous colonies on limestone, unbleached, Stn 20 (OLL 2016/137).&lt;/p&gt; &lt;p&gt; IRVING SMT: 2 colonies on small rocks, 3 colonies on &lt;i&gt;Flabellum chunii&lt;/i&gt; Marenzeller, 1904, Stn 31 (unregistered MNHN material); 5 colonies on small rocks, Stn 32 (unregistered MNHN material); 12 colonies on small rocks (1 sputter-coated) plus 4 colonies on &lt;i&gt;F. chunii&lt;/i&gt;, Stn 33 (unregistered MNHN material); 1 colony on stylasterid skeleton, Stn 34 (unregistered MNHN material).&lt;/p&gt; &lt;p&gt; HY&Egrave;RES SMT: 3 colonies on &lt;i&gt;F. chunii&lt;/i&gt;, Stn 26 (unregistered MNHN material); 1 small colony on old shell, Stn 27 (unregistered MNHN material); 1 colony on &lt;i&gt;F. chunii&lt;/i&gt; and 5 small colonies on shell fragments (one sputter-coated), Stn 28 (unregistered MNHN material); 1 colony on &lt;i&gt;F. chunii&lt;/i&gt;, Stn 29 (unregistered MNHN material); 3 colonies on &lt;i&gt;F. chunii&lt;/i&gt;, Stn 30 (unregistered MNHN material); 1 colony on rock fragment, Stn 25 (unregistered MNHN material); 1 colony on bioclast, mounted on stub and sputter-coated, Stn 28 (OLL 2016/138); 1 colony on bioclast, mounted on stub and sputter-coated, Stn 28 (OLL 2016/139).&lt;/p&gt; Description &lt;p&gt;Colony encrusting, unilaminar, forming small patches and bi- to triserial ribbons (Fig. 3A). Zooecia oval to polygonal, with tapering proximal end wedged in between proximal zooecia, separated by deep grooves (Fig. 3B). Frontal shield matted vitreous, convex, surface densely covered with relatively small irregular and flattened nodules, imperforate except for some six to eight very small marginal pores, invisible in frontal view or in older zooecia; lateral walls particularly well developed, septular pores in&lt;/p&gt; &lt;p&gt;lateral walls very large and transversely oval, surrounded by a broad area of nodular cryptocyst, distal pore suborbicular, slightly raised relative to lateral ones (Fig. 1B).&lt;/p&gt; &lt;p&gt;Orifice almost as wide as long, broadest in distal third, proximal and lateral margins fairly straight, proximal third delimited by a pair of very short and thick, blunt condyles oriented proximomedially (Fig. 3C); lateral and distolateral margins with eight (very rarely nine) closely-spaced, slender, tapering and slightly curved spines with thick cylindrical bases (Fig. 3C, F), arranged in two groups of four with a distinct distal gap (in case there are nine spines, one group consists of five); all eight spines present in ovicellate zooids, with the distalmost pair thinner and tightly pressed against the ooecial peristome (Fig. 3D).&lt;/p&gt; &lt;p&gt;Ovicell hyperstomial, ooecium barely resting on distal zooid&rsquo;s frontal shield (Fig. 3B, D), globular, about as long as wide, with a short tubular peristome wedged in between distalmost pair of spines and terminating at distal orifice margin; ectooecium smooth, encompassing approximately lower half of ooecium; exposed endooecium relatively large, hemispherical, surface covered by flattened irregular nodules similar to frontal shield (Fig. 3D); ooecial aperture suborbicular, about as tall as wide, acleithral.&lt;/p&gt; &lt;p&gt;Ancestrula oval (ca 280 &micro;m long, 210 &micro;m wide), tatiform, gymnocyst well-developed and gently sloping proximally, becoming steeper and narrower distally; cryptocyst practically absent; opesia oval (ca 185 &micro;m long, 140 &micro;m wide), slightly constricted in distal third; presumably nine mural spines, with five proximal ones widely spaced and four distal ones situated closer together; a single first-generation autozooid budded distally or distolaterally (Fig. 3E).&lt;/p&gt; Remarks &lt;p&gt; &lt;i&gt;Atlantisina meteor&lt;/i&gt; gen. et sp. nov. is the only species in the genus with eight or occasionally even nine spines surrounding the orifice (all other species have six), and in which the number of spines may occasionally vary. The additional spine is usually thinner and wedged in between the four others on one side of the orifice. Besides this difference in spine number, &lt;i&gt;A. meteor&lt;/i&gt; gen. et sp. nov. is very similar to &lt;i&gt;A. atlantis&lt;/i&gt; gen. et sp. nov., which occurs on the relatively closely located Atlantis Smt (ca 150 km north of the Great Meteor Bank-Hy&egrave;res-Irving seamount complex), and also to &lt;i&gt;A. inarmata&lt;/i&gt; gen. et sp. nov. from the Canary Islands (see below). However, the nodules on the frontal shield in &lt;i&gt;A. meteor&lt;/i&gt; gen. et sp. nov. are slightly smaller and more irregular in outline. Moreover, the surface structure of the endooecium is similar to that of the frontal shield, while it is more indistinctly and variably sculptured in &lt;i&gt;A. atlantis&lt;/i&gt; gen. et sp. nov., and deeply pitted in &lt;i&gt;A. inarmata&lt;/i&gt; gen. et sp. nov.&lt;/p&gt; &lt;p&gt; Besides the Great Meteor Bank, &lt;i&gt;A. meteor&lt;/i&gt; gen. et sp. nov. has also been recorded from the relatively closely located Irving and Hy&egrave;res seamounts. These three discrete populations differ slightly in the size of their frontal shield nodules, and also in the nodular pattern on the endooecial surface, which may be variably pronounced. However, these differences are very subtle and may also occur within colonies. We thus regard these differences as representing intraspecific variability until genetic analyses can be carried out.&lt;/p&gt; Ecology &lt;p&gt;The bi- to triserial colonies of this species encrust coral and stylasterid skeletons, shells and pebbles at depths between 270 and 750 m.&lt;/p&gt; Distribution &lt;p&gt; &lt;i&gt;Atlantisina meteor&lt;/i&gt; gen. et sp. nov. occurs on the central Atlantic Great Meteor Bank and probably also on Irving Smt and Hy&egrave;res Smt.&lt;/p&gt;Published as part of &lt;i&gt;Berning, Björn, Harmelin, Jean-Georges &amp; Bader, Beate, 2017, New Cheilostomata (Bryozoa) from NE Atlantic seamounts, islands, and the continental slope: evidence for deep-sea endemism, pp. 1-51 in European Journal of Taxonomy 347&lt;/i&gt; on pages 11-13, DOI: 10.5852/ejt.2017.347, &lt;a href="http://zenodo.org/record/3832630"&gt;http://zenodo.org/record/3832630&lt;/a&gt

Atlantisinidae Berning, Harmelin & Bader 2017

&lt;p&gt; Family &lt;b&gt;Atlantisinidae&lt;/b&gt; fam. nov.&lt;/p&gt; &lt;p&gt;urn:lsid:zoobank.org:act: 17F29C69-ECAC-4D27-A3B6-DE8C96009930&lt;/p&gt; Type genus &lt;p&gt; &lt;i&gt;Atlantisina&lt;/i&gt; gen. nov.&lt;/p&gt; Diagnosis &lt;p&gt;Colonies encrusting; autozooidal frontal shield umbonuloid, imperforate or pseudoporous. Orifice with condyles, oral spines present. Adventitious and/or interzooidal avicularia present in some taxa. Lateral walls with basal pore-chambers, usually well developed, interzooidal commmunication via a single or few septular pores per neighbouring zooid, budding intrazooidal. Ovicell hyperstomial, ooecium either produced by the zooid distal to the maternal one or of kenozooidal origin, not closed by the operculum, the distal pair of oral spines closely appressed to the sides of the short, tubular ooecial opening; the kenozooidal ooecium may be entirely independent of the substratum and distal zooid, and may be budded from a distinct pore in the maternal zooid&rsquo;s distal wall; endooecium fully calcified, ectooecium partially calcified. Ancestrula tatiform with extensive opesia; cryptocyst absent.&lt;/p&gt; Remarks &lt;p&gt; The taxa that are here combined in the Atlantisinidae fam. nov. are morphologcially somewhat similar to, and share some characters with, the families Escharellidae Levinsen, 1909, Exochellidae Bassler, 1935 and Romancheinidae Jullien, 1888, while the latter is occasionally considered to include the former two (D.P. Gordon, pers. comm. 2014). However, the new taxa described here share distinct traits, which can be regarded as relatively conservative in an evolutionary sense, that are not present in any of the existing families. Assigning the new genera to the Romancheinidae &lt;i&gt;sensu lato&lt;/i&gt; would add yet another set of new characters to this large taxon, and would render the family difficult to define precisely, as well as to delimit it from other lepralielloid families, e.g., the Bryocryptellidae Vigneaux, 1949.&lt;/p&gt; &lt;p&gt;Characters common to the species in all three genera presented here comprise the partial calcification of the ectooecium and the position of the distalmost pair of spines in maternal zooids, which are so closely appressed to the peristomial aperture of the ooecium as to leave a furrow on each side when the spine is missing. The species usually have extensive lateral walls composed of smooth gymnocyst, and interzooidal communication takes place via one (occasionally two) large septular pores. Moreover, all species share the same ancestrula-type (tatiform with a large opesia that is somewhat constricted in the distal oral part, while the cryptocyst is practically absent).&lt;/p&gt; &lt;p&gt; In contrast, the representative taxa in the Romancheinidae &lt;i&gt;sensu lato&lt;/i&gt; are characterised by ancestrulae with a reduced oval or otherwise shaped opesia that is often bounded by an extensive cryptocyst. The ooecium is also structurally different in that the ectooecium is membranous, and the endooecium fuses with the frontal shield of the distal auto- or kenozooid (Ostrovsky 2013). Both the relatively primitive structure of the ancestrula as well as the morphology of the ooecium in the new taxa may thus suggest that the new family forms a clade basal to the Romancheinidae &lt;i&gt;sensu lato&lt;/i&gt; (further discussed below). Genetic studies will be needed to ultimately shed light on the phylogenetic relationships between the new taxa and the Romancheinidae, as well as among the taxa currently placed within the Romancheinidae.&lt;/p&gt;Published as part of &lt;i&gt;Berning, Björn, Harmelin, Jean-Georges &amp; Bader, Beate, 2017, New Cheilostomata (Bryozoa) from NE Atlantic seamounts, islands, and the continental slope: evidence for deep-sea endemism, pp. 1-51 in European Journal of Taxonomy 347&lt;/i&gt; on pages 3-5, DOI: 10.5852/ejt.2017.347, &lt;a href="http://zenodo.org/record/3832630"&gt;http://zenodo.org/record/3832630&lt;/a&gt

Atlantisina seinensis Berning & Harmelin & Bader & Cibio 2017, gen. et sp. nov.

&lt;i&gt;Atlantisina seinensis&lt;/i&gt; gen. et sp. nov. &lt;p&gt;urn:lsid:zoobank.org:act: E78F78F3-3E20-4F34-98E9-20441E929E45&lt;/p&gt; &lt;p&gt;Fig. 5 A&ndash;E, Table 5&lt;/p&gt; Diagnosis &lt;p&gt;Frontal shield densely covered by relatively large, flattened nodules; lateral walls well developed, septular pores large, round to elongated oval, surrounded by a broad area of cryptocystal calcification; orifice margin with six oral spines; a single, large, pointed mucro with a broad base along the proximal orifice margin is positioned suborally. Ooecium longer than wide; ectooecium covering approximately the lower half of ooecium; exposed endooecium oval, convex, surface densely covered by numerous deep pits bounded by thickened ridges.&lt;/p&gt; Etymology &lt;p&gt;Named after its type locality, Seine Smt.&lt;/p&gt; Material examined &lt;p&gt; &lt;b&gt;Holotype&lt;/b&gt;&lt;/p&gt; &lt;p&gt;SEINE SMT: 1 coated colony on biogenic concretion, Stn 42 (MNHN-IB-2014-57).&lt;/p&gt; &lt;p&gt; &lt;b&gt;Paratypes&lt;/b&gt;&lt;/p&gt; &lt;p&gt;SEINE SMT: 1 colony on small rock, Stn 41 (MNHN-IB- 2014-58); 3 small colonies (2 ovicellate, 1 immature) on small rock, Stn 42 (MNHN-IB- 2014-59); 1 colony on limestone, Stn 41 (OLL 2016/144).&lt;/p&gt; Description &lt;p&gt;Colony encrusting, unilaminar, forming small patches and/or bi- to triserial ribbons (Fig. 5A). Zooecia oval to polygonal, with tapering proximal end(s) wedged in between proximal zooecia, separated by deep grooves (Fig. 5A). Frontal shield convex, surface densely covered by relatively large, round to polygonal, flattened nodules (Fig. 5 B&ndash;C), imperforate except for a few very small marginal pores, invisible in frontal view or in older zooecia; suboral frontal shield steeply raised to form a massive mucro with a pointed tip (Fig. 5 B&ndash;C), lateral and distal part made of gymnocystal calcification, proximal face a continuation of nodular cryptocystal-type calcification of frontal shield, broad base of mucro framing proximal orifice margin and levelling towards proximal pair of spines; lateral walls well developed, septular pores relatively large, round to transversely oval, each pore surrounded by a broad cryptocystal area with an irregular surface (Fig. 5B, D&ndash;E).&lt;/p&gt; &lt;p&gt;Orifice suborbicular to oval, slightly longer than wide, broadest at about mid-distance, proximal orifice margin slightly concave, poster comprising about one-third of entire orifice length, delimited from anter by a pair of very short and thick, blunt condyles directing proximomedially (Fig. 5C); lateral and distolateral margins with six closely-spaced spines with thick bases, arranged in two groups of three with a distinct distal gap (Fig. 5 B&ndash;C); all six spines present in ovicellate zooids with distal pair a little thinner and resting firmly against proximolateral sides of ooecial peristome (Fig. 5D).&lt;/p&gt; &lt;p&gt;Ovicell hyperstomial, ooecium barely resting on frontal shield of distal zooid or free at colony margin, globular, distinctly longer than wide and with a short tubular peristome opening at distal orifice margin, ooecial aperture suborbicular, acleithral; a broad band of smooth ectooecial cover encompassing slightly more than the lower half of ooecium; endooecial surface densely covered by numerous, irregularly shaped, deep pits bounded by thick ridges, giving false appearance of a pseudoporous endooecium (Fig. 5 D&ndash;E).&lt;/p&gt; &lt;p&gt;An ancestrula was not observed.&lt;/p&gt; Remarks &lt;p&gt; &lt;i&gt;Atlantisina seinensis&lt;/i&gt; gen. et sp. nov. is easily distinguished from all other species of &lt;i&gt;Atlantisina&lt;/i&gt; gen. nov. owing to its single tall, triangular suboral mucro. Concerning frontal shield morphology, this species takes an intermediate position: whereas &lt;i&gt;A. atlantis&lt;/i&gt; gen. et sp. nov., &lt;i&gt;A. meteor&lt;/i&gt; gen. et sp. nov. and &lt;i&gt;A. inarmata&lt;/i&gt; gen. et sp. nov. have the same type of nodular frontal calcification but lack a suboral mucro, all remaining &lt;i&gt;Atlantisina&lt;/i&gt; gen. nov. species (see below) have complex mucrones but frontal shields with a honeycomb structure. The densely and deeply pitted endooecium in &lt;i&gt;A. seinensis&lt;/i&gt; gen. et sp. nov. is, again, shared with &lt;i&gt;A. inarmata&lt;/i&gt; gen. et sp. nov. from the Canary Islands.&lt;/p&gt; Ecology &lt;p&gt; As in the other &lt;i&gt;Atlantisina&lt;/i&gt; gen. nov. species, colonies of &lt;i&gt;A. seinensis&lt;/i&gt; gen. et sp. nov. are small, combining spot- and runner-type characters (cf. Bishop 1989), and forming small patches with bi- to triserial ribbons. The colonies were found encrusting rocks at depths of 235&ndash; 260 m.&lt;/p&gt; Distribution &lt;p&gt;This species is known only from Seine Smt.&lt;/p&gt;Published as part of &lt;i&gt;Berning, Björn, Harmelin, Jean-Georges &amp; Bader, Beate, 2017, New Cheilostomata (Bryozoa) from NE Atlantic seamounts, islands, and the continental slope: evidence for deep-sea endemism, pp. 1-51 in European Journal of Taxonomy 347&lt;/i&gt; on pages 16-18, DOI: 10.5852/ejt.2017.347, &lt;a href="http://zenodo.org/record/3832630"&gt;http://zenodo.org/record/3832630&lt;/a&gt