In this task, you're given a paragraph from the research paper and your task is to generate a suitable title for the research paper based on the given paper. Under 100 words is a good title length.
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Question: We report a hereditary leukodystrophy in Standard Schnauzer puppies. Clinical signs occurred shortly after birth or started at an age of under 4 weeks and included apathy, dysphoric vocalization, hypermetric ataxia, intension tremor, head tilt, circling, proprioceptive deficits, seizures and ventral strabismus consistent with a diffuse intracranial lesion. Magnetic resonance imaging revealed a diffuse white matter disease without mass effect. Macroscopically, the cerebral white matter showed a gelatinous texture in the centrum semiovale. A mild hydrocephalus internus was noted. Histopathologically, a severe multifocal reduction of myelin formation and moderate diffuse edema without inflammation was detected leading to the diagnosis of leukodystrophy. Combined linkage analysis and homozygosity mapping in two related families delineated critical intervals of approximately 29 Mb. The comparison of whole genome sequence data of one affected Standard Schnauzer to 221 control genomes revealed a single private homozygous protein changing variant in the critical intervals, TSEN54:c.371G>A or p.(Gly124Asp). TSEN54 encodes the tRNA splicing endonuclease subunit 54. In humans, several variants in TSEN54 were reported to cause different types of pontocerebellar hypoplasia. The genotypes at the c.371G>A variant were perfectly associated with the leukodystrophy phenotype in 12 affected Standard Schnauzers and almost 1000 control dogs from different breeds. These results suggest that TSEN54:c.371G>A causes the leukodystrophy. The identification of a candidate causative variant enables genetic testing so that the unintentional breeding of affected Standard Schnauzers can be avoided in the future. Our findings extend the known genotype-phenotype correlation for TSEN54 variants. Various hereditary diseases of the cerebral white matter occur in humans and dogs. We describe a new leukodystrophy in Standard Schnauzers. Genetic mapping and whole genome sequence analysis identified a likely candidate causative variant in the TSEN54 gene encoding tRNA splicing endonuclease 54. These results provide new information about the role of TSEN54 in cell metabolism and the development of the central nervous system in the late gestational and early post-natal period. The affected dogs potentially represent a translational large animal model for similar leukoencephalopathies in human medicine. The clinical phenotype in Schnauzers included multifocal central nervous system signs. A holistic pathogenically driven understanding of disease initiation and perpetuation requires a solid analysis of the underlying genetics and characterization of the disease phenotype at the clinical and cellular as well as sub-cellular level. In contrast to the canine phenotype with a predominant manifestation in the cerebrum white matter, other TSEN54 variants in humans have been reported to result in a different pathological phenotype characterized by pontocerebellar hypoplasia. The differences between humans and dogs underscore the need for comparative analysis at the clinical, pathological and molecular level to understand species-specific protein mediated pathways, interactions and outcomes.

Answer: TSEN54 missense variant in Standard Schnauzers with leukodystrophy Author summary


Question: Ten years after the severe acute respiratory syndrome epidemic, a second coronavirus, the Middle East respiratory syndrome coronavirus (MERS-CoV), has been identified as the cause of a highly lethal pneumonia in patients in the Middle East and in travelers from this region. Over the past 9 months, since the virus was first isolated, much has been learned about the biology of the virus. It is now clear that MERS-CoV is transmissible from person to person, and its close relationship with several bat coronaviruses suggests that these animals may be the ultimate source of the infection. However, many key issues need to be addressed, including identification of the proximate, presumably zoonotic, source of the infection, the prevalence of the infection in human populations, details regarding clinical and pathological features of the human infection, the establishment of a small rodent model for the infection, and the virological and immune basis for the severe disease observed in most patients. Most importantly, we do not know whether a MERS-CoV epidemic is likely or not. Infection with the virus has so far resulted in only 91 cases and 46 deaths (as of 29 July 2013), but it is nonetheless setting off alarm bells among public health officials, including Margaret Chan, Director-General of the World Health Organization, who called MERS-CoV "a threat to the entire world." This article reviews some of the progress that has been made and discusses some of the questions that need to be answered. Citation Perlman S. 2013. The Middle East respiratory syndrome-how worried should we be? mBio 4(4):e00531-13.

Answer: The Middle East Respiratory Syndrome-How Worried Should We Be? A NOVEL VIRUS IS IDENTIFIED IN SAUDI ARABIA


Question: A 7-month-old unvaccinated domestic shorthair cat was presented with a history of inappetence and hypersalivation. Clinical examination revealed fever, gingivitis and oral ulceration. An initial blood sample revealed a severe leukopenia and neutropenia and a feline panleukopenia virus (FPV) titre of 1:640; a second titre 10 days later was 1:2,560. A feline calicivirus (FCV) titre was 1:320 and the initial clinical signs and laboratory findings were attributed to a coinfection with FPV and FCV. The cat was treated with interferon omega. A blood sample taken 10 days later revealed a severe thrombocytosis of 3,448×10 9 /L (reference range 200-500×10 9 /L) confirmed by blood smear examination. The platelet count on day 35 was 4,990×10 9 /L. No bone marrow examination was carried out. Two differential diagnoses for an extreme thrombocytosis are a reactive thrombocytosis or essential thrombocythemia (ET). ET is a hemic neoplasia that causes a severe and persistent thrombocytosis, and is a diagnosis of exclusion. Reactive thrombocytosis is generally mild to moderate and of shorter duration than described here, but an excessive response could have been caused by infection. The influence of additional factors such as an initial thrombocytopenia, age and interferon treatment are not clear but can be speculated on. Although the exact pathophysiology in this case remains unclear, such high, persistent thrombocyte numbers have not been reported in cats in the absence of a neoplastic process. A 7-month-old, male neutered domestic shorthair cat presented to his veterinarian with a complaint of inappetence and a swaying gait. The cat was allowed to go outside and was unvaccinated. The cat shared a household with a female littermate that had received only the first part of the primary vaccination 3 months previously. Upon examination, the cat was found to be pyrexic (temperature 41°C), and an examination of the oral cavity revealed an ulcerative gingivostomatitis. The gait abnormality was identified by the veterinarian as a hindlimb ataxia. A blood sample was collected and sent to the InVitro Diagnostic Laboratory. Hematology was performed (CELL-DYN. 3500 with Veterinary Package Software, Abbott Laboratories, Abbott Park, IL, USA) and revealed severe leukopenia (WBC 1.0×10 9 /L, reference interval 6.0-15.0×

Answer:
Extreme thrombocytosis in a young cat