AnatomyDiseasesChemicals and DrugsAnalytical, Diagnostic and Therapeutic Techniques and EquipmentHealth Care
CeroidNeuronal Ceroid-LipofuscinosesSerine ProteasesThiolester HydrolasesDipeptidyl-Peptidases and Tripeptidyl-PeptidasesAminopeptidasesLipofuscinLipidosesLysosomal Storage Diseases, Nervous SystemLysosomesPigments, BiologicalMolecular ChaperonesCathepsin FEndopeptidasesDolicholCystaphosCathepsin DDog DiseasesHermanski-Pudlak SyndromeSheep DiseasesSubdural SpaceBrainDisease Models, AnimalTriazinesCongresses as TopicMembrane GlycoproteinsVitamin U
Ceroid: A naturally occurring lipid pigment with histochemical characteristics similar to lipofuscin. It accumulates in various tissues in certain experimental and pathological conditions.Neuronal Ceroid-Lipofuscinoses: A group of severe neurodegenerative diseases characterized by intracellular accumulation of autofluorescent wax-like lipid materials (CEROID; LIPOFUSCIN) in neurons. There are several subtypes based on mutations of the various genes, time of disease onset, and severity of the neurological defects such as progressive DEMENTIA; SEIZURES; and visual failure.Serine Proteases: Peptide hydrolases that contain at the active site a SERINE residue involved in catalysis.Thiolester HydrolasesDipeptidyl-Peptidases and Tripeptidyl-Peptidases: A subclass of exopeptidases that includes enzymes which cleave either two or three AMINO ACIDS from the end of a peptide chain.Aminopeptidases: A subclass of EXOPEPTIDASES that act on the free N terminus end of a polypeptide liberating a single amino acid residue. EC 3.4.11.Lipofuscin: A naturally occurring lipid pigment with histochemical characteristics similar to ceroid. It accumulates in various normal tissues and apparently increases in quantity with age.Lipidoses: Conditions characterized by abnormal lipid deposition due to disturbance in lipid metabolism, such as hereditary diseases involving lysosomal enzymes required for lipid breakdown. They are classified either by the enzyme defect or by the type of lipid involved.Lysosomal Storage Diseases, Nervous System: A group of enzymatic disorders affecting the nervous system and to a variable degree the skeletal system, lymphoreticular system, and other organs. The conditions are marked by an abnormal accumulation of catabolic material within lysosomes.Lysosomes: A class of morphologically heterogeneous cytoplasmic particles in animal and plant tissues characterized by their content of hydrolytic enzymes and the structure-linked latency of these enzymes. The intracellular functions of lysosomes depend on their lytic potential. The single unit membrane of the lysosome acts as a barrier between the enzymes enclosed in the lysosome and the external substrate. The activity of the enzymes contained in lysosomes is limited or nil unless the vesicle in which they are enclosed is ruptured. Such rupture is supposed to be under metabolic (hormonal) control. (From Rieger et al., Glossary of Genetics: Classical and Molecular, 5th ed)Pigments, Biological: Any normal or abnormal coloring matter in PLANTS; ANIMALS or micro-organisms.Molecular Chaperones: A family of cellular proteins that mediate the correct assembly or disassembly of polypeptides and their associated ligands. Although they take part in the assembly process, molecular chaperones are not components of the final structures.Cathepsin F: A lysosomal papain-related cysteine proteinase that is expressed in a broad variety of cell types.Endopeptidases: A subclass of PEPTIDE HYDROLASES that catalyze the internal cleavage of PEPTIDES or PROTEINS.Dolichol: Eicosamethyl octacontanonadecasen-1-o1. Polyprenol found in animal tissues that contains about 20 isoprene residues, the one carrying the alcohol group being saturated.Cystaphos: Proposed as an adjuvant to cancer chemotherapy; may have radiation protective properties.Cathepsin D: An intracellular proteinase found in a variety of tissue. It has specificity similar to but narrower than that of pepsin A. The enzyme is involved in catabolism of cartilage and connective tissue. EC 3.4.23.5. (Formerly EC 3.4.4.23).Dog Diseases: Diseases of the domestic dog (Canis familiaris). This term does not include diseases of wild dogs, WOLVES; FOXES; and other Canidae for which the heading CARNIVORA is used.Hermanski-Pudlak Syndrome: Syndrome characterized by the triad of oculocutaneous albinism (ALBINISM, OCULOCUTANEOUS); PLATELET STORAGE POOL DEFICIENCY; and lysosomal accumulation of ceroid lipofuscin.Sheep Diseases: Diseases of domestic and mountain sheep of the genus Ovis.Subdural Space: Potential cavity which separates the ARACHNOID MATER from the DURA MATER.Brain: The part of CENTRAL NERVOUS SYSTEM that is contained within the skull (CRANIUM). Arising from the NEURAL TUBE, the embryonic brain is comprised of three major parts including PROSENCEPHALON (the forebrain); MESENCEPHALON (the midbrain); and RHOMBENCEPHALON (the hindbrain). The developed brain consists of CEREBRUM; CEREBELLUM; and other structures in the BRAIN STEM.Disease Models, Animal: Naturally occurring or experimentally induced animal diseases with pathological processes sufficiently similar to those of human diseases. They are used as study models for human diseases.Triazines: Heterocyclic rings containing three nitrogen atoms, commonly in 1,2,4 or 1,3,5 or 2,4,6 formats. Some are used as HERBICIDES.Congresses as Topic: Conferences, conventions or formal meetings usually attended by delegates representing a special field of interest.Membrane Glycoproteins: Glycoproteins found on the membrane or surface of cells.Vitamin U: A vitamin found in green vegetables. It is used in the treatment of peptic ulcers, colitis, and gastritis and has an effect on secretory, acid-forming, and enzymatic functions of the intestinal tract.

Morphological study on pigmented cells in the horse testis. (1/32)

One of the most attractive characteristics of a horse testis is the change of the weight during development. As the testicular weight changes and the number of Leydig cells decreases, pigments appear in interstitial tissues. In the present study, the characteristics of the pigments found in the interstitial tissues were examined histochemically and ultrastructurally. Specific stainings indicated that the pigmented granules showed almost all of the histological and histochemical characteristics of ceroid or ceroid-like pigment. The cells showed positive reaction for acid phosphatase while the pigmented cells contained a lot of lysosomes ultrastructurally. These results suggest that macrophages might phagocytize Leydig cells, and store their digested materials as ceroid-like pigment.  (+info)

Inducible nitric oxide synthase colocalizes with signs of lipid oxidation/peroxidation in human atherosclerotic plaques. (2/32)

OBJECTIVE: Advanced human atherosclerotic plaques are characterized by the abundant presence of the autofluorescent non-soluble lipid pigment ceroid, consisting of oxidized lipoproteins. The aim of the present study was to examine the topographical and cellular distribution of inducible nitric oxide synthase (iNOS or NOS II) within different stages of atherosclerosis and its colocalization with ceroid deposits and nitrotyrosine. METHODS AND RESULTS: Different stages of atherosclerosis were studied by immunohistochemistry on whole-mount longitudinal sections of carotid endarterectomy specimens. In the adaptive intimal thickening the predominant cell type were smooth muscle cells. The fatty streaks contained both smooth muscle cells and macrophages with an extremely low NOS II immunoreactivity. The advanced atherosclerotic plaques however, showed a very dense infiltration by macrophages, of which a subpopulation expressed NOS II as a vesicular immunoreactivity in their cytoplasm. These were mainly present around the necrotic core, in association with ceroid accumulation and nitrotyrosine. Fluorescence quenching microscopy showed the presence of NOS II on autofluorescent ceroid vesicles in the macrophages. Large extracellular ceroid granules were not NOS II immunoreactive. NOS II mRNA was detected by RT-PCR and the protein by Western blot in the plaque tissue but not in mammary arteries used as controls. CONCLUSION: Ceroid, nitrotyrosine and NOS II colocalized in late stages of atherosclerosis and were found around the necrotic core in the plaque. This could suggest that NOS II expression in macrophages is involved in oxidation and peroxidation of lipids, leading to ceroid formation.  (+info)

Proteasome inhibition by lipofuscin/ceroid during postmitotic aging of fibroblasts. (3/32)

We have studied the effects of hyperoxia and of cell loading with artificial lipofuscin or ceroid pigment on the postmitotic aging of human lung fibroblast cell cultures. Normobaric hyperoxia (40% oxygen) caused an irreversible senescence-like growth arrest after about 4 wk and shortened postmitotic life span from 1-1/2 years down to 3 months. During the first 8 wk of hyperoxia-induced 'aging', overall protein degradation (breakdown of [(35)S]methionine metabolically radiolabeled cell proteins) increased somewhat, but by 12 wk and thereafter overall proteolysis was significantly depressed. In contrast, protein synthesis rates were unaffected by 12 wk of hyperoxia. Lysosomal cathepsin-specific activity (using the fluorogenic substrate z-FR-MCA) and cytoplasmic proteasome-specific activity (measured with suc-LLVY-MCA) both declined by 80% or more over 12 wk. Hyperoxia also caused a remarkable increase in lipofuscin/ceroid formation and accumulation over 12 wk, as judged by both fluorescence measurements and FACscan methods. To test whether the association between lipofuscin/ceroid accumulation and decreased proteolysis might be causal, we next exposed cells to lipofuscin/ceroid loading under normoxic conditions. Lipofuscin/ceroid-loaded cells indeed exhibited a gradual decrease in overall protein degradation over 4 wk of treatment, whereas protein synthesis was unaffected. Proteasome specific activity decreased by 25% over this period, which is important since proteasome is normally responsible for degrading oxidized cell proteins. In contrast, an apparent increase in lysosomal cathepsin activity was actually caused by a large increase in the number of lysosomes per cell. To test whether lipofuscin/ceroid could in fact directly inhibit proteasome activity, thus causing oxidized proteins to accumulate, we incubated purified proteasome with lipofuscin/ceroid preparations in vitro. We found that proteasome is directly inhibited by lipofuscin/ceroid. Our results indicate that an accumulation of oxidized proteins (and lipids) such as lipofuscin/ceroid may actually cause further increases in damage accumulation during aging by inhibiting the proteasome.  (+info)

Phagocytosis and macrophage activation associated with hemorrhagic microvessels in human atherosclerosis. (4/32)

OBJECTIVE: Previously, we demonstrated that activated inducible NO synthase (iNOS)-expressing foam cells in human carotid plaques often produce autofluorescent (per)oxidized lipids (ceroid). Here, we investigate whether intraplaque microvessels can provide foam cells with lipids and trigger macrophage activation. METHODS AND RESULTS: Microvessels (von Willebrand factor [vWf] immunoreactivity), activated macrophages (iNOS immunoreactivity), and ceroid were systematically mapped in longitudinal sections of 15 human carotid endarterectomy specimens. An unbiased hierarchical cluster analysis classified vascular regions into 2 categories. One type with normal vWf expression and without inflammatory cells was seen, and another type with cuboidal endothelial cells, perivascular vWf deposits, and iNOS and ceroid-containing foam cells was seen in 4 (27%) of 15 plaques. The perivascular foam cells frequently contained platelets (glycoprotein Ibalpha) and erythrocytes (hemoglobin, iron), pointing to microhemorrhage/thrombosis and subsequent phagocytosis. Similar lipid-containing cells, expressing both ceroid and iNOS, were generated in atherosclerosis-free settings by incubating murine J774 macrophages with platelets or oxidized erythrocytes and also in vivo in organizing thrombi in normocholesterolemic rabbits. CONCLUSIONS: Focal intraplaque microhemorrhages initiate platelet and erythrocyte phagocytosis, leading to iron deposition, macrophage activation, ceroid production, and foam cell formation. Neovascularization, besides supplying plaques with leukocytes and lipoproteins, can thus promote focal plaque expansion when microvessels become thrombotic or rupture prone.  (+info)

A clinical variant of familial Hermansky-Pudlak syndrome. (5/32)

Hermansky-Pudlak syndrome (HPS) is an autosomal recessive inherited disease consisting of (1) partial oculocutaneous albinism (with nystagmus, strabism, and visual acuity loss), (2) platelet storage pool deficiency (with bleeding diathesis), and (3) disorder of "ceroid" metabolism with a multisystem tissue lysosomal ceroid deposition. HPS is less uncommon in Puerto Rico, where the most important studies have been performed, but is a very rare disease in Europe. HPS basic defect remains unknown, even if an HPS-causing gene was identified in chromosome segment 10q23-q23.3, and several mutations have been reported. The aim of this article is to discuss, on the basis of a review of relevant literature, a new familial HPS clinical variant observed in 2 young sisters (aged 16 and 23 years old, respectively), characterized by the typical symptoms of this syndrome. Our patients also suffered from diffuse interstitial pulmonary disease and an unexpectedly increased platelet aggregation and were prone to bacterial infections. Interestingly, we observed urinary tract abnormality in the younger HPS sister and a porencephalic cyst in the older HPS sister; both of these developmental defects have been reported in the Cross syndrome (or oculocerebral hypopigmentation syndrome). It seems that in our patients, an overlapping of the phenotypic manifestations of different rare syndromes may be present. The presence of ceroid-like autofluorescent material in urinary sediment together with the histologic aspects and the autofluorescence of oral mucosa biopsy are consistent with a ceroid-like lipofuscin storage. HPS should be carefully tested for in suspected cases to prevent the severe visual impairment, rapidly progressive pulmonary fibrosis, and other complications associated with this disorder.  (+info)

Ascorbic acid oxidation: a potential cause of the elevated severity of atherosclerosis in diabetes mellitus? (6/32)

The exposure of mouse peritoneal macrophages to cholesterol linoleate-containing artificial lipoproteins can lead to intracellular ceroid accumulation. This can be used as a model to study the role of oxidation in macrophage uptake of lipoproteins containing unsaturated fatty acids, considered by many as a primary event in atherosclerotic plaque formation. Our studies show that ascorbic acid can both inhibit and promote the formation of ceroid in such a model system. The transition metal copper (Cu(II)) further elevates ceroid accumulation and EDTA, a metal chelator, inhibits it. When trace levels of transition metals are present, low concentrations of ascorbic acid can elevate ceroid formation. This pro- and antioxidant characteristic of ascorbic acid was confirmed by monitoring the generation of oxidants by various concentrations of ascorbic acid, assessed by benzoic acid hydroxylation or the fragmentation of BSA. We discuss these observations in the context of an apparent increase in ascorbic acid oxidation and elevated severity of atherosclerosis in diabetes mellitus.  (+info)

Diagnostic usefulness of bronchoalveolar lavage in Hermansky-Pudlak syndrome: a case with double lung cancers. (7/32)

A 65-year-old man was admitted to our hospital because of dyspnea on exertion. He had oculocutaneous albinism innately and his parents were consanguineous. His chest roentgenogram on admission showed reticulo-nodular infiltrates and cystic changes throughout both lung fields, and 7 cm mass in the left middle field. Cytology of bronchoalveolar lavage fluid (BALF) revealed macrophages containing ceroid. The diagnosis of HPS was made clinically and the tumor was diagnosed as poorly differentiated adenocarcinoma of the lung. He died of respiratory failure. By autopsy, additional well-differentiated adenocarcinoma was detected. Cytology of BALF was useful to confirm ceroid accumulation in the lung.  (+info)

Retinal pathology in a canine model of late infantile neuronal ceroid lipofuscinosis. (8/32)

 (+info)

English Setter | Breeds | DogZone.comEnglish Setter | Breeds | DogZone.com
English Setters are sweet and gentle. They are said to be the nicest breed from the Setter family, as they are very fond of people and get along well with children and pets. They are also friendly towards strangers, which does not make them a very good guard dog. They are, however, very alert and will bark to announce guests - just to happily greet them right after.. English Setters are extremely active dogs and are definitely suited for the active person, who will take them along for a run, a hike, a bike ride, or a hunt, as this was the main activity for which they were bred. They are demanding in terms of exercise, as they love running and playing outside, but when their energy is well-spent, they also know how to behave indoors. They are quiet and calm, as long as their energy needs have been met. Due to its high stamina and drive, the English Setter may become destructive inside the house if not properly exercised. They also tend to bark, dig and jump, which is why fenced yards are a must. ...
Mountain View Llewellins Setter Kennel, LLC. - Kentucky English Setter, Llewellin Setter Breeders - Gun Dog Breeders...Mountain View Llewellin's Setter Kennel, LLC. - Kentucky English Setter, Llewellin Setter Breeders - Gun Dog Breeders...
Mountain View Llewellins Setter Kennel, LLC. - Kentucky English Setter, Llewellin Setter Breeders Hunting dog classified ads for selling bird dog puppies, started and finished gun dogs for sale.
English Setter | CynologistEnglish Setter | Cynologist
In the wild a dog can roam free and exercise himself. In the city you have to make sure he gets enough exercise by walking him and Playing with him. Professional dog walkers are fine, but if you walk your dog yourself, the experience will be far more rewarding. A six-foot leash is fine and its purpose is not only to keep your dog from running away, but also to teach him that he is to stay by your side. Every client is taught how to walk his dog properly. The dog should walk and not pull you down the street. Ideally, your dog should now know enough to sit and stay when hes told to. But whats to keep him at your side when youre actually walking him? If you cant answer that one, you are in for some unpleasant experiences. One womans two great Danes pulled her down the street three times a day until the dogs saw a cat on the other side of the street. A strikingly beautiful girl, who never owned even one dog bought two English setters to promenade in the park. One day both dogs headed for the ...
Gilmans White Lighting - English Setter Pedigrees - Bird Dog PedigreesGilman's White Lighting - English Setter Pedigrees - Bird Dog Pedigrees
Connecting buyers and breeders with hunting dog classified ads for selling bird dog puppies, started and finished gun dogs for sale.
Breed Standard | English Setter Club of CanadaBreed Standard | English Setter Club of Canada
The entire head should be in proportion to the body. It should be long and lean with a well-defined stop. The skull, when viewed from above, should be oval. The skull should be of medium width, without coarseness, and should be only slightly wider at the base than at the brows. The widest part of the oval should be at the ear set. There should be a moderately defined occipital protuberance. The length of the skull from the occiput to the stop should be equal in length to the muzzle. Muzzle: brick-shaped, and the width to be in harmony with the skull. It should be level from the eyes to the top of the nose. When viewed from the side, the line of the top of the muzzle should be parallel to the line of the top of the skull. A dish or a Roman nose is objectionable. The flews should be square and pendant. The nose to be black or dark brown in colour except in white, orange and white, lemon and white or liver and white where it may be lighter. The nostrils should be wide apart and large in the ...
Dietary supplementation with (R)-alpha-lipoic acid reverses the age-related accumulation of iron and depletion of antioxidants...Dietary supplementation with (R)-alpha-lipoic acid reverses the age-related accumulation of iron and depletion of antioxidants...
Legal Disclaimer: Chelation and Hyperbaric Therapy, Stem Cell Therapy, and other treatments and modalities mentioned or referred to in this web site are medical techniques that may or may not be considered "mainstream". As with any medical treatment, results will vary among individuals, and there is no implication or guarantee that you will heal or achieve the same outcome as patients herein. As with any procedure, there could be pain or other substantial risks involved. These concerns should be discussed with your health care provider prior to any treatment so that you have proper informed consent and understand that there are no guarantees to healing. THE INFORMATION IN THIS WEBSITE IS OFFERED FOR GENERAL EDUCATIONAL PURPOSES ONLY AND DOES NOT IMPLY OR GIVE MEDICAL ADVICE. No Doctor/Patient relationship shall be deemed to have arisen simply by reading the information contained on these pages, and you should consult with your personal physician/care giver regarding your medical treatment before ...
Macrophage Stimulating Protein (MSP) Evokes Superoxide Anion Production by Human Macrophages of Different OriginMacrophage Stimulating Protein (MSP) Evokes Superoxide Anion Production by Human Macrophages of Different Origin
1. Macrophage Stimulating Protein (MSP), a serum factor related to Hepatocyte Growth Factor, was originally discovered to stimulate chemotaxis of murine resident peritoneal macrophages. MSP is the ligand for Ron, a member of the Met subfamily of tyrosine kinase receptors. The effects of MSP on human …
Columnists | www.massapequapost.com | Massapequa PostColumnists | www.massapequapost.com | Massapequa Post
Angel got her splint on October 6 when she was about five-weeks-old, and since she would need anesthesia to manipulate her joint back in position, Eileen officially adopted her. She wanted Angel to "belong" to someone during this critical point in her young life. Afterwards Eileen had to cut the bandages twice to redo them because tiny Angel was growing. She was supposed to wear the splint for two weeks, but the specialist took it off a few days early and instead restricted her exercise for two more weeks. This means her siblings are staying with Josey for now. In the meantime, Angel (all seven pounds of her) continues to recover, entertaining herself by arranging her toys in her Great Dane size crate and wearing her leopard Doggles, while nibbling on her "Miss Porkie" snout in the sunshine. Soon the albino trio will be reunited for playtime.. Above & Beyonds mission is to put English Setters welfare first and foremost. Quinn became the canine guardian angel, perhaps a friendly ghost, to those ...
Dog days? In Italy, count them like family sick days | National PostDog days? In Italy, count them like family sick days | National Post
ROME — An Italian librarian who says her English setter is her family has won the right from her employer to use family sick leave to care for her ailing pet instead of having to use vacation days.
A-1 Special reproduction 12ga for saleA-1 Special reproduction 12ga for sale
2 barrel set, 26, imp. cly.& mod.and 28 mod. and full.Custom engraved with English setter,ducks,and grouse,all wonderfully and pe for sale by lawrence schaedel on GunsAmerica - 976929498
Search - NeL.eduSearch - NeL.edu
CONCLUSIONS: HA can react with MDA to form stable products, a non-fluorescent enamine (product 1) and a fluorescent 1,4-dihydropyridine (product 2) which are ceroid/lipofuscin-related adducts. The reaction of HA with MDA may reveal toxic effect of unsaturated carbonyls in the brain and may reflect a novel de-carbonylation function of histamine under various pathological conditions..... Read abstract Full text PDF ...
how to specify setter injection for a static class using StructureMap? - Stack Overflowhow to specify setter injection for a static class using StructureMap? - Stack Overflow
PluginType Name Description ------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------ Func`1,TResult, (Func`1,TResult,) Scoped as: Transient 311731d7-60c7-46de-9ef4-24608f21df04 ------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------ IDomainEventHandlerFactory (DE.Infrastructure.Domain.Events.IDomainEventHandlerFactory) ...
PATCH 01/14] mshtml/tests: Added body event setters tests.PATCH 01/14] mshtml/tests: Added body event setters tests.
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PATCH 01/12] mshtml/tests: Added body event setters tests.PATCH 01/12] mshtml/tests: Added body event setters tests.
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Amyotrofisk lateralsklerose (ALS) | Blausen MedicalAmyotrofisk lateralsklerose (ALS) | Blausen Medical
Alle viljestyrte bevegelser i kroppen kontrolleres av hjernen. Nerveceller i hjernen kalt øvre motornevroner, setter i gang bevegelser gjennom å frigj...
Abatement - definition of abatement by The Free DictionaryAbatement - definition of abatement by The Free Dictionary
Define abatement. abatement synonyms, abatement pronunciation, abatement translation, English dictionary definition of abatement. n. 1. Reduction in amount, degree, or intensity; diminution. 2. The amount lowered; a reduction. 3. Law The act of reducing something, such as a tax, for...
15 English Setter Springer spaniel German pointer Czechoslovakia from morninglineantiques on Ruby Lane15" English Setter Springer spaniel German pointer Czechoslovakia from morninglineantiques on Ruby Lane
RARE 15" heavy high quality porcelain hunting dog from Schleggenwald Czechoslovakia....English setter/Springer spaniel/German pointer or other type bird dog....early first casting detail shows hand painted piercing tri color inset eyes, eye lids, eye brows, black nose w nostrils, mouth, jowls, hips, shoulders, great floppy ears, toes w toenails, paw pads...hand painted black collar...... defined fur with hand painted accents.... 15"long 7"tall.....no chips, cracks, crazing...clear clean OLD marks...check out my other hunting dogs ...
Dog Ceroid Lipofuscinosis Philadelphia PA - Dog Ceroid Lipofuscinosis, Philadelphia PA dog adoption, Philadelphia PA dog care...Dog Ceroid Lipofuscinosis Philadelphia PA - Dog Ceroid Lipofuscinosis, Philadelphia PA dog adoption, Philadelphia PA dog care...
Looking for information on Dog Ceroid Lipofuscinosis in Philadelphia? We have compiled a list of businesses and services around Philadelphia that should help you with your search. We hope this page helps you find information on Dog Ceroid Lipofuscinosis in Philadelphia.
PLOS Genetics: A One Base Pair Deletion in the Canine ATP13A2 Gene Causes Exon Skipping and Late-Onset Neuronal Ceroid...PLOS Genetics: A One Base Pair Deletion in the Canine ATP13A2 Gene Causes Exon Skipping and Late-Onset Neuronal Ceroid...
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
Induction of serine and threonine protein phosphorylation by endotoxin-associated protein in murine resident peritoneal...Induction of serine and threonine protein phosphorylation by endotoxin-associated protein in murine resident peritoneal...
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 ...
Hickory Hollow Llewellin Setters - About UsHickory Hollow Llewellin Setters - About Us
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!
Age-related accumulation of Reelin in amyloid-like deposits - Research CollectionAge-related accumulation of Reelin in amyloid-like deposits - Research Collection
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 ...
Brittany Puppies Pictures Images | Puppies Dog Breed Information Image PicturesBrittany Puppies Pictures Images | Puppies Dog Breed Information Image Pictures
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 ...
Info. needed regarding Teeth extraction in dogs - PurseForumInfo. needed regarding Teeth extraction in dogs - PurseForum
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...
Targeting Topics 12q2Targeting Topics 12q2
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 ...
Search of: congenital neuronal ceroid lipofuscinosis OR Neuronal Ceroid-Lipofuscinoses - List Results - ClinicalTrials.govSearch of: 'congenital neuronal ceroid lipofuscinosis' OR 'Neuronal Ceroid-Lipofuscinoses' - List Results - ClinicalTrials.gov
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 ...
Setters Barn (Reading) - B&B Reviews, Photos & Price Comparison - TripAdvisorSetters Barn (Reading) - B&B Reviews, Photos & Price Comparison - TripAdvisor
Book Setters Barn, Reading on TripAdvisor: See 66 traveller reviews, 11 candid photos, and great deals for Setters Barn, ranked #1 of 17 B&Bs / inns in Reading and rated 5 of 5 at TripAdvisor.
The Design of Software (CLOSED) - getters/setters confusion...immutable objectsThe Design of Software (CLOSED) - getters/setters confusion...immutable objects
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 ...
ch breed started setter - Ad #39428ch breed started setter - Ad #39428
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I have a red setter 6 months old has been vomiting and diarrheaI have a red setter 6 months old has been vomiting and diarrhea
Question - I have a red setter 6 months old has been vomiting and diarrhea - 2D. Find the answer to this and other Vet questions on JustAnswer
Setter Injection Bean PatternsSetter Injection Bean Patterns
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 ...
Download Non State Actors As Standard SettersDownload Non State Actors As Standard Setters
download to find the story-telling. The exclusive transformation sent not Translated on this trauma. Please speaker-independent the visit for themes and contact however.
google.gdata.Persongoogle.gdata.Person
Constructs a person construct using an object parameter whose property names match the setter method to use for each property. Parameters: ...
Element IndexElement Index
Setter for the hash method. Supports md5() and sha1() functions, and if available the hashing algorithms supported by the hash() PHP5 function or the mhash extension ...
EnglishEnglish
Download Hamster Life 4.3.8 for Android. Feed him, play with him and keep it clean. What would it feel like to be a Hamster?. Hamster Life is a management game where you
Dog Ceroid Lipofuscinosis Chicago IL - Dog Ceroid Lipofuscinosis, Chicago IL dog adoption, Chicago IL dog care, Chicago IL ...Dog Ceroid Lipofuscinosis Chicago IL - Dog Ceroid Lipofuscinosis, Chicago IL dog adoption, Chicago IL dog care, Chicago IL ...
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Neuronal ceroid lipofuscinosis - WikipediaNeuronal ceroid lipofuscinosis - Wikipedia
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 ...
Neuronal Ceroid Lipofuscinosis 13 via the CTSF Gene - PreventionGeneticsNeuronal Ceroid Lipofuscinosis 13 via the CTSF Gene - PreventionGenetics
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 ...
Neuronal Ceroid Lipofuscinosis 5 (NCL5) - CAG - Center for Animal GeneticsNeuronal Ceroid Lipofuscinosis 5 (NCL5) - CAG - Center for Animal Genetics
Neuronal Ceroid Lipofuscinosis 5 (NCL5) is an autosomal-recessive neurological disease with progressive neurodegeneration in Golden Retriever.
Ceroid lipofuscinosis neuronal type 8 (CLN8) DNA Test Lab Cost INR 30000.00 best genetic offer discount price Delhi Mumbai...Ceroid lipofuscinosis neuronal type 8 (CLN8) DNA Test Lab Cost INR 30000.00 best genetic offer discount price Delhi Mumbai...
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
Ceroid lipofuscinosis, neuronal, 8, northern epilepsy variant (Concept Id: C1864923) - MedGen - NCBICeroid lipofuscinosis, neuronal, 8, northern epilepsy variant (Concept Id: C1864923) - MedGen - NCBI
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).
UniProtKB/SwissProt variant VAR 016800UniProtKB/SwissProt variant VAR 016800
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 ...
ICD-10 Diagnosis Code E75.4 Neuronal ceroid lipofuscinosisICD-10 Diagnosis Code E75.4 Neuronal ceroid lipofuscinosis
Diagnosis Code E75.4 information, including descriptions, synonyms, code edits, diagnostic related groups, ICD-9 conversion and references to the diseases index.
Pedigree Dogs Exposed - The BlogPedigree Dogs Exposed - The Blog
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...Aging is one of the biggest risk factors for the major prevalent diseases such as cardiovascular diseases, neurodegeneration...
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 ...
Non-Enzymatic Nitration Alters Remodeling and Mechanical Properties of Collagenous Engineered Tissues | IOVS | ARVO JournalsNon-Enzymatic Nitration Alters Remodeling and Mechanical Properties of Collagenous Engineered Tissues | IOVS | ARVO Journals
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 ...
Search of: late-infantile neuronal ceroid lipofuscinosis OR Jansky-Bielschowsky disease OR Neuronal Ceroid-Lipofuscinoses...Search of: 'late-infantile neuronal ceroid lipofuscinosis' OR 'Jansky-Bielschowsky disease' OR 'Neuronal Ceroid-Lipofuscinoses'...
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 ...
Musikk og konsert | DeichmanMusikk og konsert | Deichman
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.... ...
Late onset neurodegeneration in the Cln3-/- mouse model of juvenile neuronal ceroid lipofuscinosis is preceded by low level...Late onset neurodegeneration in the Cln3-/- mouse model of juvenile neuronal ceroid lipofuscinosis is preceded by low level...
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.. ...
Final Diagnosis -- Case 536Final Diagnosis -- Case 536
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 ...
Question: Which dogs are gun dogs? (2020)Question: Which dogs are gun dogs? (2020)
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.
Irish Red and White Setter Breed Information, Characteristics & Heath Problems | DogZone.comIrish Red and White Setter Breed Information, Characteristics & Heath Problems | DogZone.com
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, ...
PPT1 - SNPediaPPT1 - SNPedia
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. ...
SunSync Blog | SunSync Nutrition | Page 5SunSync Blog | SunSync Nutrition | Page 5
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 ...
Tooth Setter | Norwood SawmillsTooth Setter | Norwood Sawmills
Restore blade tooth set for smooth milling and perfect lumber with Norwoods Tooth Setter. Reset full blade in under 15 minutes.
Careers.org | Cutting and Slicing Machine Setters, Operators, and Tenders - Occupational Profile andCareers.org | Cutting and Slicing Machine Setters, Operators, and Tenders - Occupational Profile and
Cutting and Slicing Machine Setters, Operators, and Tenders Career Overview Profile with short video, earnings, outlook, knowledge, skills, abilities, typical tasks and activities, training requirements, nature of the work, occupations and academic
How can I become a better fitness goal setter?How can I become a better fitness goal setter?
When it comes to working out, you can simply assume that youre motivated, goal-directed and mentally ready to achieve OR you can distinguish yourself as a flawless fitness goal setter. Not both. For improved motivation and long-term adherence to physical activity, incorporate these three methods into your fitness plan to maximize your results.
Setter Water Bottles - CafePressSetter Water Bottles - CafePress
Shop CafePress for Setter Water Bottles. Find great designs on high quality durable Stainless Steel Water Bottles and Sport Bottles. ✓Free Returns ✓100% Satisfaction Guarantee ✓Fast Shipping
Muziro Spring Reflex S4R Sneakers- UmensionMuziro 'Spring Reflex' S4R Sneakers- Umension
Its impossible to ignore trend in Street-wear fashion. Muziro Spring Reflex S4R Sneakers are the new trend setters and they are unchallenged in comfort and versatility. These sneakers offer wide variety of options on how to clad them. They feature breathable mesh and shock absorbing outsole. Shock absorbing outsole L
google.gdata.Recurrencegoogle.gdata.Recurrence
Constructs a recurrence rule using an object parameter whose property names match the setter method to use for each property. Parameters: ...
Jansky-Bielschowsky diseaseJansky-Bielschowsky disease
Myoclonic epilepsyMyoclonic epilepsy
Kufs diseaseKufs disease
List of OMIM disorder codesList of OMIM disorder codes
Frederick BattenFrederick Batten
CLN5CLN5
Tripeptidyl peptidase ITripeptidyl peptidase I
Batten diseaseBatten disease
Northern epilepsy syndromeNorthern epilepsy syndrome
CLN6CLN6
DNAJC5DNAJC5
Night-blooming cereusNight-blooming cereus
Major facilitator superfamilyMajor facilitator superfamily
Model organism databasesModel organism databases
PPT1PPT1
MFSD8MFSD8
CLN8CLN8
Tibetan TerrierTibetan Terrier
CLN3CLN3
Torsten SjögrenTorsten Sjögren
Glial fibrillary acidic proteinGlial fibrillary acidic protein
Palmitoyl protein thioesterasePalmitoyl protein thioesterase
PPT2PPT2
LipofuscinLipofuscin
Sweat glandSweat gland
Cathepsin DCathepsin D
KCTD7KCTD7
Periodic acid-Schiff stainPeriodic acid-Schiff stain
AnatomyDiseasesChemicals and DrugsAnalytical, Diagnostic and Therapeutic Techniques and EquipmentHealth Care
CeroidNeuronal Ceroid-LipofuscinosesSerine ProteasesThiolester HydrolasesDipeptidyl-Peptidases and Tripeptidyl-PeptidasesAminopeptidasesLipofuscinLipidosesLysosomal Storage Diseases, Nervous SystemLysosomesPigments, BiologicalMolecular ChaperonesCathepsin FEndopeptidasesDolicholCystaphosCathepsin DDog DiseasesHermanski-Pudlak SyndromeSheep DiseasesSubdural SpaceBrainDisease Models, AnimalTriazinesCongresses as TopicMembrane GlycoproteinsVitamin U
Search - NeL.eduSearch - NeL.edu
Ceroid cactus - WikipediaCeroid cactus - Wikipedia
Neuronal ceroid lipofuscinosis - WikipediaNeuronal ceroid lipofuscinosis - Wikipedia
Neuronal ceroid lipofuscinoses (NCL): MedlinePlus Medical EncyclopediaNeuronal ceroid lipofuscinoses (NCL): MedlinePlus Medical Encyclopedia
Neuronal Ceroid-Lipofuscinoses - MeSH - NCBINeuronal Ceroid-Lipofuscinoses - MeSH - NCBI
Ultrastructure of the retina in adult neuronal ceroid lipofuscinosis. - PubMed - NCBIUltrastructure of the retina in adult neuronal ceroid lipofuscinosis. - PubMed - NCBI
Orphanet: Neuronal ceroid lipofuscinosisOrphanet: Neuronal ceroid lipofuscinosis
Molecular Genetics of the Neuronal Ceroid LipofuscinosesMolecular Genetics of the Neuronal Ceroid Lipofuscinoses
Cystagon to Treat Infantile Neuronal Ceroid Lipofuscinosis - Full Text View - ClinicalTrials.govCystagon to Treat Infantile Neuronal Ceroid Lipofuscinosis - Full Text View - ClinicalTrials.gov
CiNii 論文 - Lamotrigine Therapy in Juvenile Neuronal Ceroid Lipofuscinosis ...CiNii 論文 - Lamotrigine Therapy in Juvenile Neuronal Ceroid Lipofuscinosis ...
Cellcept for Treatment of Juvenile Neuronal Ceroid Lipofuscinosis - Full Text View - ClinicalTrials.govCellcept for Treatment of Juvenile Neuronal Ceroid Lipofuscinosis - Full Text View - ClinicalTrials.gov
Neuronal Ceroid Lipofuscinosis - Signs And PrognosisNeuronal Ceroid Lipofuscinosis - Signs And Prognosis
neuronal ceroid lipofuscinosis 8 northern epilepsy variant Disease Ontology Browser - DOID:0110724neuronal ceroid lipofuscinosis 8 northern epilepsy variant Disease Ontology Browser - DOID:0110724
NCL-E - Neuronal Ceroid Lipofuscinosis/American Bully | AntageneNCL-E - Neuronal Ceroid Lipofuscinosis/American Bully | Antagene
Short-Term Administration of Mycophenolate Is Well-Tolerated in CLN3 Disease (Juvenile Neuronal Ceroid Lipofuscinosis) |...Short-Term Administration of Mycophenolate Is Well-Tolerated in CLN3 Disease (Juvenile Neuronal Ceroid Lipofuscinosis) |...
Neuronal Ceroid Lipofuscinosis in Border Collie Dogs in Japan: Clinical and Molecular Epidemiological Study (2000-2011)Neuronal Ceroid Lipofuscinosis in Border Collie Dogs in Japan: Clinical and Molecular Epidemiological Study (2000-2011)
AAVRh.10 Administered to Children With Late Infantile Neuronal Ceroid Lipofuscinosis - Full Text View - ClinicalTrials.govAAVRh.10 Administered to Children With Late Infantile Neuronal Ceroid Lipofuscinosis - Full Text View - ClinicalTrials.gov
Expanding the Neuroimaging Phenotype of Neuronal Ceroid Lipofuscinoses | American Journal of NeuroradiologyExpanding the Neuroimaging Phenotype of Neuronal Ceroid Lipofuscinoses | American Journal of Neuroradiology
The neuronal ceroid lipofuscinoses: the same, but different? | Biochemical Society Transactions | Portland PressThe neuronal ceroid lipofuscinoses: the same, but different? | Biochemical Society Transactions | Portland Press
Athena Diagnostics - Epilepsy Advanced Sequencing and CNV Evaluation - Neuronal Ceroid LipofuscinosisAthena Diagnostics - Epilepsy Advanced Sequencing and CNV Evaluation - Neuronal Ceroid Lipofuscinosis
could lipofuscin/ceroid be the main driver of aging?could lipofuscin/ceroid be the main driver of aging?
Neuronal ceroid lipofuscinosis | Genetic and Rare Diseases Information Center (GARD) - an NCATS ProgramNeuronal ceroid lipofuscinosis | Genetic and Rare Diseases Information Center (GARD) - an NCATS Program
Further delineation of eye manifestations in homozygous 15q13.3 microdeletions including TRPM1: a differential diagnosis of...Further delineation of eye manifestations in homozygous 15q13.3 microdeletions including TRPM1: a differential diagnosis of...
MR imaging and localized proton MR spectroscopy in late infantile neuronal ceroid lipofuscinosis. | American Journal of...MR imaging and localized proton MR spectroscopy in late infantile neuronal ceroid lipofuscinosis. | American Journal of...
Astrocytosis in infantile neuronal ceroid lipofuscinosis: friend or foe? | Biochemical Society TransactionsAstrocytosis in infantile neuronal ceroid lipofuscinosis: friend or foe? | Biochemical Society Transactions
PLOS Genetics: A One Base Pair Deletion in the Canine ATP13A2 Gene Causes Exon Skipping and Late-Onset Neuronal Ceroid...PLOS Genetics: A One Base Pair Deletion in the Canine ATP13A2 Gene Causes Exon Skipping and Late-Onset Neuronal Ceroid...
Cardiac pathology in neuronal ceroid lipofuscinoses (NCL): More than a mere co-morbidity - Open Research OnlineCardiac pathology in neuronal ceroid lipofuscinoses (NCL): More than a mere co-morbidity - Open Research Online
GMS | 24th Annual Meeting of the German Retina Society | Variant retinal phenotyps in murine models of neuronal ceroid...GMS | 24th Annual Meeting of the German Retina Society | Variant retinal phenotyps in murine models of neuronal ceroid...
Disease characteristics and progression in patients with late-infantile neuronal ceroid lipofuscinosis type 2 (CLN2) disease:...Disease characteristics and progression in patients with late-infantile neuronal ceroid lipofuscinosis type 2 (CLN2) disease:...
Ceroid Lipofuscinosis, Neuronal, 10 disease: Malacards - Research Articles, Drugs, Genes, Clinical TrialsCeroid Lipofuscinosis, Neuronal, 10 disease: Malacards - Research Articles, Drugs, Genes, Clinical Trials
Batten sj kd mur (Juvenile Neuronal Ceroid Lipofuscinosis) | GreiningBatten sj kd mur (Juvenile Neuronal Ceroid Lipofuscinosis) | Greining
AnatomyDiseasesChemicals and DrugsAnalytical, Diagnostic and Therapeutic Techniques and EquipmentHealth Care
CeroidNeuronal Ceroid-LipofuscinosesSerine ProteasesThiolester HydrolasesDipeptidyl-Peptidases and Tripeptidyl-PeptidasesAminopeptidasesLipofuscinLipidosesLysosomal Storage Diseases, Nervous SystemLysosomesPigments, BiologicalMolecular ChaperonesCathepsin FEndopeptidasesDolicholCystaphosCathepsin DDog DiseasesHermanski-Pudlak SyndromeSheep DiseasesSubdural SpaceBrainDisease Models, AnimalTriazinesCongresses as TopicMembrane GlycoproteinsVitamin U
NCLsInfantileLIPOFUSCINJNCLLipofuscinosis NeuronalKnown as Batten diseaseDisordersBatten DiseaseNeuronsCLN2CLN6DiseaseModels of neuronalRecessiveGeneticGeneGenesTibetan TerrierDiseasesAdult neuronalMutationNervous SystemMutationsProteinsINCLOnsetDiagnosisNeurologicalPPT1HomozygousType
NCLs26
Infantile13
LIPOFUSCIN17
JNCL8
Lipofuscinosis Neuronal9
Known as Batten disease1
Disorders6
Batten Disease3
Neurons2
CLN24
CLN63
Disease8
Models of neuronal1
Recessive2
Genetic3
Gene7
Genes2
Tibetan Terrier1
Diseases1
Adult neuronal2
Mutation1
Nervous System2
Mutations1
Proteins1
INCL1
  • In laboratory experiments, Cystagon has helped remove ceroid from cells of patients with INCL. (clinicaltrials.gov)
Onset1
Diagnosis3
Neurological1
  • We report the electroencephalographic (EEG) features of 22 patients with neuronal ceroid lipofuscinoses (NCL) who were referred to the Neurological Institute of Milan between 1984 and 1998. (elsevier.com)
PPT11
  • NCL safnast upp kve nar sameindir fitu og pr teina ( ceroid og lipufuscin ) og er ekkt a truflun remur hv tum geta valdi sj kd mnum ( PPT1,TPP-1 og CathepsinD/CTSD ). (greining.is)
Homozygous1
Type2