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Author Summary The neuronal ceroid lipofuscinosis (NCL) is a neurodegenerative storage diseases characterized by psychomotor retardation, blindness, and premature death. NCL has been reported in several dog breeds. NCL is characterized by progressive brain and retinal atrophy and the intracellular accumulation of autofluorescent lysosomal storage bodies resembling lipofuscin. Tibetan terriers show a late-onset and lethal NCL (age of onset 5-7 years) with an autosomal recessive inheritance. The most frequently described first symptom is blindness in twilight. In the disease progress the affected dogs often appear nervous or anxious and the lack of motor coordination becomes more severe. In the final stages of this disease, mild but also severe seizures have been observed by the owner. There are no treatment options for affected dogs. Through a genome-wide association analysis using the 127K canine Affymetrix SNP chip, we found a 1 Mb candidate genomic region and identified ATP13A2 as the most likely

Endotoxin-associated protein (EP) from Salmonella typhi activated murine resident peritoneal macrophages to produce prostaglandin E2 (PGE2). Cells from both endotoxin nonresponder (C3H/HeJ) and the endotoxin responder (C3H/OuJ) mouse strains were activated by EP. This EP-induced prostaglandin E2 production was blocked by the protein kinase C (PKC) inhibitor H-7 as well as the tyrosine kinase inhibitor genistein, suggesting the involvement of both serine and threonine phosphorylation and tyrosine phosphorylation pathways in the activation of resident peritoneal macrophages by EP. Immunoblot analysis using antiphosphoserine and antiphosphothreonine antibodies showed that EP induced the serine and threonine phosphorylation of a 14-kDa protein (p14). This phosphorylation was not induced by phorbol myristic acid or by lipopolysaccharide endotoxin. Inhibitors of PKC, PKA, and PKG did not block the phosphorylation of p14. However, the tyrosine kinase inhibitor piceatannol blocked p14 serine and ...

Die Neuronale Ceroid Lipofuszinose ist gekennzeichnet durch einen Zerfall von Nervenzellen.Ursache für die Krankheit ist ein fehlerhafte Stoffspeicherung.

We are Hickory Hollow, breeders of purebred Llewellin Setters for upland bird hunters. If you choose a Hickory Hollow Llewellin Setter, you are not only going to get a quality family and hunting companion, you will also be provided with a rare opportunity of getting glimpses into the life of your much anticipated new arrival and you will become a part of the Hickory Hollow family. We also serve as an English Setter and Llewellin Setter Rescue organization. If you have an English or Llewellin Setter than you can no longer keep, please contact us and we will help you find the perfect, loving and forever home for your dog!

Mus musculus; Rattus norvegicus; Callithrix jacchus; Hippocampus; Stratum lacunosum-moleculare; Entorhinal cortex; Piriform cortex; Episodic-like memory; Radial arm maze; GABAergic interneurons; PolyIC; Neuroinflammation; 3xTg-AD mice; SynGAP; GFAP; Dab1; F4/80; Immunohistochemistry; Alzheimers ...

Sporting Group Cocker Spaniel (Black) Am. GChG CH Mar-Ks Sunrise At Midnight, Select Dog (Breeders Michelle Mitchell, Marlene Ness & Mark Ragusa; owners Jeffrey Hanlin Jr., Mark Ragusa & Marlene Ness; handler Marlene Ness). English Setters Can. GCh. & Am. GChB Sevenoaks Lady Penelope, Select Bitch (breeders Hilary Oaks, Sabrina Oaks & William Potts; owners Sandra Nordstrom & Sabrina Oaks; handler Adam Bernardin) and Am. Ch. Lentigos Saltcreek Teal, Award of Merit (breeder Lenora Barber; owners Peter Ziliotto & Barry Barbant; handler Hailey Griffith). Golden Retriever Am. GCh. Verdoros Overcoming Obstacles, Select Bitch (breeder Ann Greenbank; owners Ann Greenbank & Michael Faulkner; handler Ann Greenbank). Gordon Setter Can. GCh. & Am. GChB Bran Linn Amulet, Best of Breed (breeder/owners Nikki Maounis, Candice Bell & Jerold Bell D.V.M.; handler Will Alexander). Irish Red & White Setter Can. GCh. & Am. Ch. Macneall Red And White Shadow Dog, Award of Merit (breeders Robert & Beata Gnyp and ...

As a breeder and trainer of pointing dogs for many years, Im a little biased. Pointing dogs fit my hunting style best, but I have enjoyed hunting with Flushers and Retrievers as well.. Hunting Strengths - Pointing breeds are often somewhat smaller and leaner than retrievers, and capable of covering a lot of ground due to tremendous athleticism. Some breeds have less speed but still exhibit great endurance. They will generally hunt within gun range, and upon finding a bird, they will lock on point and give you time to approach for a comfortable shot. Most pointing breeds will naturally retrieve to hand.. Common Breeds - Brittany, English Setter, German Shorthaired Pointer, German Wirehaired Pointer and Pointer (English).. ...

Hi, Wondered if anyone could give me information regarding this. One of my English Setters Darcey has been very ill for about 6 weeks, he has been...

Country of Origin: The Brittany (also accepted as the American Brittany, Brittany Spaniel, and Epagneul Breton) originated in the Brittany arena of France in the 1800s from crosses of the English Setter and baby Spaniels. It is a accomplished bird hunter with accomplished scenting abilities. The Brittanys pointing and retrieving abilities and top akin of accordance fabricated it accepted a allotment of hunters, decidedly poachers. Brittanys were aboriginal alien to North America in the 1920s and accustomed by the American Kennel Club in 1934 (the registered name was afflicted from Brittany Spaniel to Brittany in 1982, out of acceptance of its added setter-like hunting style), but took a while to bolt on acceptance due to the absence of a continued tail. The Brittany has boring risen in acceptance due to its baby size, amenable personality, and accomplished hunting skills. It is today the a lot of accepted arrow in acreage trials ...

IB4-saporin attenuates acute and eliminates chronic muscle pain in the rat.. Alvarez P, Gear RW, Green PG, Levine JD.. Exp Neurol 233(2):859-865, 2012.. In order to clarify the roles of isolectin B4-positive and IB4-negative nociceptors in inflammatory and ergonomic muscle pain, the authors administered 3.2 µg of IB4-SAP (Cat. #IT-10) into the intrathecal space of rats. Although the baseline mechanical nociceptive threshold was not affected in the lesioned animals, mechanical hyperalgesia had a shorter duration. In the ergonomic models peak hyperalgesia was attenuated, and prolongation of PGE2-induced mechanical hyperalgesia was completely prevented.. Age-related Accumulation of Non-heme Ferric and Ferrous Iron in Mouse Ovarian Stroma Visualized by Sensitive Non-heme Iron Histochemistry.. Asano Y.. J Histochem Cytochem 60(3):229-242, 2012.. The mammalian ovary engages in continuous growth and cellular differentiation as long as the animal is capable of reproduction. During these processes iron ...

Aging is associated with an increasing dysfunction of key brain homeostasis mechanisms and represents the main risk factor across most neurodegenerative disorders. However, the degree of dysregulation and the affectation of specific pathways set apart normal aging from neurodegenerative disorders. In particular, the neuronal metabolism of catecholaminergic neurotransmitters appears to be a specifically sensitive pathway that is affected in different neurodegenerations. In humans, catecholaminergic neurons are characterized by an age-related accumulation of neuromelanin (NM), rendering the soma of the neurons black. This intracellular NM appears to serve as a very efficient quencher for toxic molecules. However, when a neuron degenerates, NM is released together with its load (many undegraded cellular components, transition metals, lipids, xenobiotics) contributing to initiate and worsen an eventual immune response, exacerbating the oxidative stress, ultimately leading to the neurodegenerative ...

To accelerate research into rare disorders by connecting individuals who are interested in research and who have been diagnosed with a rare disorder (or a disorder of unknown prevalence, or who are undiagnosed) with researchers who study rare diseases ...

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Some people argue not to have getter/setters..I have a simple requirement...user should be able to creat transport packages..which application saves into database..if user wants to change some attributes of a package..app displays that package..user makes changes and then the app saves the updated package..now,without getter how will i display to user..maybe ,use a toString method which returns a string..which then I will have to be split to show in different UI elements..and to change state of an object i will need setters..or..make object immutable..so when user makes changes create a new object with same identity(database)..and then save into database ...

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a class to periodically clean old log records from the database public class LogCleaner { List _logTables = new LinkedList(); // the createXXX method is optional, and allows use something other than // the default constructor for a sub-bean public LogTable createTable() { return new LogTable(); } // you could also use setTable(LogTable logTable) public void addTable(LogTable logTable) { _logTables.add(logTable); } public class LogTable { String _name; String _timestampField; public void setName(String name) { _name = name; } public void setTimestampField(String timestampField) { _timestampField = timestampField; } @PostConstruct public void init() throws Exception { if (_name == null) throw new Exception(name is required); if (_timestampField == null) throw new Exception(timestamp-field is required); } public void cleanTable(DataSource pool) { Connection conn = null; try { conn = pool.getConnection(); ... } catch (SQLException e) { throw new ServletException(e); } finally { try { if ...

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Constructs a person construct using an object parameter whose property names match the setter method to use for each property. Parameters: ...

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Neuronal ceroid lipofuscinosis (NCL) is the general name for a family of at least eight genetically separate neurodegenerative disorders that result from excessive accumulation of lipopigments (lipofuscin) in the bodys tissues. These lipopigments are made up of fats and proteins. Their name comes from the word stem lipo-, which is a variation on lipid or fat, and from the term pigment, used because the substances take on a greenish-yellow color when viewed under an ultraviolet light microscope. These lipofuscin materials build up in neuronal cells and many organs, including the liver, spleen, myocardium, and kidneys. The classic characterization of the group of neurodegenerative, lysosomal storage disorders called the neuronal ceroid lipofuscinoses (NCLs) is through the progressive, permanent loss of motor and psychological ability with a severe intracellular accumulation of lipofuscins, with the United States and northern European populations having slightly higher frequency with an ...

The neuronal ceroid lipofuscinoses (NCLs) are inherited neurodegenerative lysosomal storage disorders caused by the accumulation of ceroid and lipofuscin in various cell types, mainly cells of the cerebral cortex, cerebellar cortex, and retina (Dyken et al. 1988; Williams and Mole 2012). Characteristic features at onset include clumsiness; deterioration of vision and psychomotor functions; seizures; and behavioral changes. Progression of clinical features results ultimately in total disability, blindness and premature death. Although NCL affects primarily children, age of onset of symptoms varies from infancy to adulthood. The incidence of NCL is variable and ranges from 1.3 to 7 per 100,000 (Mole and Williams 2013). However, it is more common in northern European populations, particularly Finland where the incidence may reach 1 in 12,500 individuals and a carrier frequency of 1 in 70 (Rider and Rider 1988). NCLs are clinically and genetically heterogeneous. A nomenclature and classification ...

Learn about the signs, symptoms, and treatment of Neuronal Ceroid Lipofuscinosis 2, NCL 2, and what to do if your dog has this health condition.

Neuronal Ceroid Lipofuscinosis 5 (NCL5) is an autosomal-recessive neurological disease with progressive neurodegeneration in Golden Retriever.

Ceroid lipofuscinosis neuronal type 8 (CLN8) Test Cost INR 30000.00 Surat Pune Jaipur Lucknow Kanpur Nagpur Visakhapatnam Indore Thane Bhopal Patna Vadodara Ghaziabad Ludhiana Coimbatore Madurai Meerut Ranchi Allahabad Trivandrum Pondicherry Mysore Aligarh best offer discount price

The neuronal ceroid lipofuscinoses (NCL; CLN) are a clinically and genetically heterogeneous group of neurodegenerative disorders characterized by the intracellular accumulation of autofluorescent lipopigment storage material in different patterns ultrastructurally. The lipopigment patterns observed most often in CLN8 comprise mixed combinations of granular, curvilinear, and fingerprint profiles (Mole et al., 2005). For a general phenotypic description and a discussion of genetic heterogeneity of CLN, see CLN1 (256730).

Ceroid lipofuscinosis, neuronal, 2 (CLN2) [MIM:204500]: A form of neuronal ceroid lipofuscinosis. Neuronal ceroid lipofuscinoses are progressive neurodegenerative, lysosomal storage diseases characterized by intracellular accumulation of autofluorescent liposomal material, and clinically by seizures, dementia, visual loss, and/or cerebral atrophy. The lipopigment pattern seen most often in CLN2 consists of curvilinear profiles. {ECO:0000269,PubMed:10330339, ECO:0000269,PubMed:10665500, ECO:0000269,PubMed:11241479, ECO:0000269,PubMed:11339651, ECO:0000269,PubMed:11589012, ECO:0000269,PubMed:12376936, ECO:0000269,PubMed:12414822, ECO:0000269,PubMed:12698559, ECO:0000269,PubMed:14736728, ECO:0000269,PubMed:19201763, ECO:0000269,PubMed:20340139, ECO:0000269,PubMed:21990111, ECO:0000269,PubMed:22612257, ECO:0000269,PubMed:9295267}. Note=The disease is caused by mutations affecting the gene represented in this entry ...

Diagnosis Code E75.4 information, including descriptions, synonyms, code edits, diagnostic related groups, ICD-9 conversion and references to the diseases index.

I grew up with pedigree dogs - English Setters, Great Danes, Labradors and, most recently, Flatcoated Retrievers. Today, I share my home with an assortment of dogs, purebred and mutts. In 2008, I directed Pedigree Dogs Exposed, a BBC documentary which uncovered the extent of health and welfare problems in pedigree dogs. The film has now been shown in more than 20 countries. Campaigning for improved purebred dog health is now a great passion - one fuelled by the fear that those who currently view themselves as the guardians of pedigree dogs are, often unwittingly, the agents of their demise. My mission, then, is to continue to highlight where things have gone wrong and to encourage breeders and Kennel Clubs to embrace reform - particularly when it comes to harmful phenotypes and inbreeding ...

Aging is one of the biggest risk factors for the major prevalent diseases such as cardiovascular diseases, neurodegeneration and cancer, but due to the complex and multifactorial nature of the aging process, the molecular mechanisms underlying age-related diseases are not yet fully understood. glycation of proteins occurs under physiological conditions and represents a type of post-translational modification taking place slowly but continuously throughout the life span, promoting AGE accumulation during aging. Thus, to some extent the accumulation of AGEs is inevitably linked to aging and age-related accumulation of AGEs was shown to exist in human cartilage, skin collagen and pericardial fluid [13-15]. Increased protein glycation is also associated with the pathogenesis of several age-related and chronic inflammatory diseases such as cardiovascular diseases [13], Alzheimers disease [16], stroke [17], as well as the general decline in health associated with old age. Under hyperglycemic ...

Purpose: : Reactions that result in the age-related accumulation of increasingly insoluble, undigestible collagen in human Bruchs membrane (BM) are only partially known. Non-enzymatic glycation is one such process and has been linked to diabetic-related complications and aging. A novel mechanism particularly relevant to smoking- and inflammation-related processes is non-enzymatic nitration (NEN). BM contains meshwork collagen IV in RPE and choriodal basement membranes as well as fibrillar collagen I in the inner and outer collagen layers. We have recently shown that NEN of basement membrane proteins can impart deleterious effects on overlying RPE cells. The present study was undertaken in order to examine the effects of NEN of fibrillar collagen I on cell-mediated remodeling and biomechanical properties of tissues. Methods: : We used an engineered tissue analog (previously developed by our group) for studying the remodeling and mechanics of collagenous tissues. Adult rat cardiac fibroblasts ...

To accelerate research into rare disorders by connecting individuals who are interested in research and who have been diagnosed with a rare disorder (or a disorder of unknown prevalence, or who are undiagnosed) with researchers who study rare diseases ...

Er det lenge siden du hørte et album fra begynnelse til slutt? På Deichman setter vi stiften på en utvalgt norsk klassiker og gir den sin fulle og fortjente oppmerksomhet.... ...

for low-Na foods Potassium acetate (CH3COOK) is a chemical compound. CH 3 CO 2 K is a potassium salt of acetic acid with chemical name Potassium acetate. [8] For example, Lenins mummy was soaked in a bath containing potassium acetate. It is a substitute for chloride salts such as calcium chloride or magnesium chloride in deicing applications. Formula Weight. Hawkins carries Potassium Acetate in bulk and in multiple packaging options. Potassium Acetate, USP is chemically designated CH 3 COOK, and is comprised of colorless crystals or a white crystalline powder that is very soluble in water. Potassium Acetate Injection, USP. Potassium Acetate 60% - Food Grade. Ph. Molecular Weight 98.14 . Predict the chemical formula of the compound that element X makes with oxygen. 1.0 2010-04-08 22:11:50 UTC 2018-05-29 01:18:49 UTC FDB015417 Potassium acetate Flavouring ingredient. chem. Question: Is KC2H3O2 ( potassium acetate ) Soluble or Insoluble in water ? CAS Number. It is widely used as a deicer to ...

Mouse models of neuronal ceroid lipofuscinosis (NCL) exhibit many features of the human disorder, with widespread regional atrophy and significant loss of GABAergic interneurons in the hippocampus and neocortex. Reactive gliosis is a characteristic of all forms of NCL, but it is unclear whether glial activation precedes or is triggered by neuronal loss. To explore this issue we undertook detailed morphological characterization of the Cln3 null mutant (Cln3(-/-)) mouse model of juvenile NCL (JNCL) that revealed a delayed onset neurodegenerative phenotype with no significant regional atrophy, but with widespread loss of hippocampal interneurons that was first evident at 14 months of age. Quantitative image analysis demonstrated upregulation of markers of astrocytic and microglial activation in presymptomatic Cln3(-/-) mice at 5 months of age, many months before significant neuronal loss occurs. These data provide evidence for subtle glial responses early in JNCL pathogenesis.. ...

DIAGNOSIS: Kufs Disease (Adult Neuronal Ceroid Lipofuscinosis). Further inquiry revealed that the deceased had an older sibling (brother) with similar symptoms who had died some years ago with a similar neurodegenerative condition. An autopsy was not performed. DISCUSSION:. Neuronal ceroid lipofuscinosis (NCL) is a heterogenous group of neurodegenerative disorders characterized by accumulation of ceroid-lipopigment inclusions in neurones and most other cells (3,14). The typical clinical features include progressive cognitive and motor deterioration and seizures. The early classification of this group of metabolic diseases was based on age of onset and ultrastructural morphology of the lysosomes, and was divided into Infantile, Late-infantile, Juvenile and Adult forms of the disease. The initial separation of subtypes by age of onset is inaccurate as the clinical onset was found to be highly variable (3,12,14). Subsequent knowledge of some of the genetic and enzyme defect for these diseases has ...

Breast cancer is the most common cancer in women worldwide. Elucidation of underlying biology and molecular pathways is necessary for improving therapeutic options and clinical outcomes. CLN3 protein (CLN3p), deficient in neurodegenerative CLN3 disease is anti-apoptotic, and defects in the CLN3 gene cause accelerated apoptosis of neurons in CLN3 disease and up-regulation of ceramide. Dysregulated apoptotic pathways are often implicated in the development of the oncogenic phenotype. Predictably, CLN3 mRNA expression and CLN3 protein were up-regulated in a number of human and murine breast cancer-cell lines. Here, we determine CLN3 expression in non-tumor vs. tumor samples from fresh and formalin-fixed/paraffin-embedded (FFPE) breast tissue and analyze the association between CLN3 overexpression and different clinicopathological characteristics of breast cancer patients. Additionally, gene expression of 28 enzymes involved in sphingolipid metabolism was determined. CLN3 mRNA is overexpressed in tumor vs.

ANSWER: There are three main types of gun dogs: retrievers, setters and flushing dogs. Some gun dog breeds are: Labradors. Golden Retrievers. Springer Spaniels. Cocker Spaniels. Irish and English Setters. Weimaraners. Hungarian Vizslas. Italian Spinones.

I grew up with pedigree dogs - English Setters, Great Danes, Labradors and, most recently, Flatcoated Retrievers. Today, I share my home with an assortment of purebreds and mutts. I am an award-winning television producer of science-led documentaries, primarily for the BBC - including Science Documentary of the Year in the 2019 British Documentary Awards. In 2008, I directed Pedigree Dogs Exposed, a BBC documentary which uncovered the extent of health and welfare problems in pedigree dogs. The film has now been shown in more than 20 countries. Campaigning for improved purebred dog health has become a great passion - one fuelled by the fear that those who currently view themselves as the guardians of pedigree dogs are, often unwittingly, the agents of their demise. My mission, then, is to continue to highlight where things have gone wrong and to encourage breeders and Kennel Clubs to embrace reform - particularly when it comes to harmful phenotypes and inbreeding. When not campaigning, I am busy ...

I grew up with pedigree dogs - English Setters, Great Danes, Labradors and, most recently, Flatcoated Retrievers. Today, I share my home with an assortment of purebreds and mutts. I am an award-winning television producer of science-led documentaries, primarily for the BBC - including Science Documentary of the Year in the 2019 British Documentary Awards. In 2008, I directed Pedigree Dogs Exposed, a BBC documentary which uncovered the extent of health and welfare problems in pedigree dogs. The film has now been shown in more than 20 countries. Campaigning for improved purebred dog health has become a great passion - one fuelled by the fear that those who currently view themselves as the guardians of pedigree dogs are, often unwittingly, the agents of their demise. My mission, then, is to continue to highlight where things have gone wrong and to encourage breeders and Kennel Clubs to embrace reform - particularly when it comes to harmful phenotypes and inbreeding. When not campaigning, I am busy ...

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The Irish Red and White Setter is a friendly dog with a good temperament. They make good family pets, as they will seek out affection and attention from their owners. Irish Red and White Setters are an active breed so will settle best with owners who will fulfill their high exercise requirements. Many Irish Red and White Setters are still working dogs and will behave as such on walks. They love to roam around their surroundings looking for scents, ideally in woodland. Although they could adapt to an urban environment, they will need an outlet for scenting behaviour and plenty of activities to keep them occupied.. Irish Red and White Setters are calm dogs, and while they like to be around their owners, they can be left home alone so long as they have other dogs for company. They are known for being gentle dogs who enjoy playing with either children or other canine companions. They can even get on with other pets if theyre brought up with them, but be careful introducing a new cat to the family, ...

Evidence-based recommendations on cerliponase alfa (Brineura) for treating neuronal ceroid lipofuscinosis type 2 in children ...

PPT1, codes Palmitoyl-protein thioesterase, a small glycoprotein that removes palmitate groups from cysteine residues in lipid-modified proteins. Mutations cause neuronal Ceroid lipofuscinosis type 1. There are at numerous disease causing SNPs identified. ...

An age spot is an omega 3 barcode. Its the Mark of the Fish and/or the Mark of the Flax. Its the Mark of Undegradable Lipofuscin - or even worse, the Mark of Ceroid. Its the three Super-Villains - DHA, EPA, and ALA - General Zod, Non, and Ursa. General Zod, Non, and Ursa are t... Read More ...

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