ceroid


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ce·roid

(sē'royd),
A waxlike, golden, or yellow-brown pigment first found in fibrotic livers of choline-deficient rats, and also known to be present in some cirrhotic livers (and certain other tissues) of human beings. Ceroid is acid fast, insoluble in fat solvents, and probably a type of lipofuscin although differing from true lipofuscins by failing to stain with Schmorl ferric-ferricyanide reduction stain; it also exhibits autofluorescence. Accumulates in Hermansky-Pudlak syndrome.
[L. cera, wax, + G. eidos, appearance]

ceroid

A complex of alcohol-insoluble, oxidised polyunsaturated lipid pigment(s) resulting from the peroxidation of unsaturated lipids, which are similar or identical to lipofuscin. Ceroid accumulates in macrophages of the heart, liver, gastrointestinal tract and brain in the elderly, and is thus termed a “wear and tear” pigment; it has been inculpated in age-related organ dysfunction hypovitaminosis E, cathartic colon, and hereditary conditions (e.g., Batten’s disease and sea-blue histiocytosis).

ceroid

A complex of alcohol-insoluble, oxidized polyunsaturated lipid pigment(s) resulting from the peroxidation of unsaturated lipids that are similar or identical to lipofuscin; ceroid accumulates in macrophages of the heart, liver, GI tract, and brain in the elderly and is thus termed 'wear and tear' pigment; it has been inculpated in age-related organ dysfunction–see 'garbage can' hypothesis, hypovitaminosis E, cathartic colon, and hereditary conditions–eg, Batten's disease, sea-blue histiocytosis.
References in periodicals archive ?
###CLN6###Neuronal Ceroid Lipofuscinosis-6###PYCR2###Hypomyelinating Leukodystrophy, 10
The company said that RGX-181 is a one-time treatment candidate for late-infantile neuronal ceroid lipofuscinosis type 2 (CLN2) disease, one of the most common forms of Batten disease caused by mutations in the tripeptidyl peptidase 1 (TPP1) gene.
The panel was prepared using a NimbleGen SeqCap EZ Choice kit (Roche, Basel, Switzerland), which included 927 disease-causative genes of the neurogenetic disease, containing progressive myoclonic epilepsy, hereditary ataxia, mitochondrial diseases, neuronal ceroid lipofuscinosis and other neurogenetic diseases.
Infantile neuronal ceroid lipofuscinosis (INCL) is a severe lysosomal disease caused by mutations in the CLN1 gene, which encodes the soluble lysosomal enzyme Palmitoyl- Protein-Thioesterase-1 (PPT1) and result in osmiophilic granules accumulating in lysosomes and leading to neuroinflammation, neurodegeneration and death.
Interestingly, there was one patient who was diagnosed with neuronal ceroid lipofuscinosis (NCL), which is generally refractory to other AEDs; this patient showed significant improvement in seizure frequency as described below.
Periodic acid-Schiff (PAS) stain with diastase pretreatment drew attention to the ceroid pigment-laden Kupffer cells indicative of the significant hepatocyte death that must have preceded this biopsy (Figure 2(c)).
Batten disease is commonly used to make reference to all forms of  Neuronal Ceroid Lipofuscinoses (NCL).
Originally described by Hermansky and Pudlak in 1959, (77) Hermansky-Pudlak Syndrome (HPS) is caused by a related group of autosomal recessive disorders that are characterized by the triad of oculocutaneous albinism, bleeding diathesis due to a platelet storage pool deficiency, and lysosomal accumulation of ceroid lipofuscin.
Brineura is the first FDA-approved treatment to slow loss of walking ability in symptomatic pediatric patients 3 years of age and older with late infantile neuronal ceroid lipofuscinosis type 2 (CLN2), also known as tripeptidyl peptidase-1 (TPP1) deficiency.
TPP1 deficiency results in classic late infantile (type 2) neuronal ceroid lipofuscinosis (LINCL, also called Jansky-Bielschowsky disease) (9).
Serum antibodies to oxidized, low-density lipoprotein and ceroid in chronic periaortitis.
Kwon, "Classification and natural history of the neuronal ceroid lipofuscinoses," Journal of Child Neurology, vol.